Term
| The primary receptors associated with temperature detection are what? |
|
Definition
| Transient receptor potential channels (TRP’s) |
|
|
Term
| Most peripheral receptors are sensitive to what? |
|
Definition
|
|
Term
| The perceived sensations of heat and cold are carried via what system and tracts? |
|
Definition
| The anterolateral system for the body and trigeminothalamic tracts for the face |
|
|
Term
| Conscious perception of temperature is achieved through the anterolateral system and trigemonothalamic tracts projecting to what part of the CNS? |
|
Definition
Through the thalamus to the insular cortex
Many of these pathways have numerous collaterals to the reticular formation |
|
|
Term
| Where are the major control centers for thermoregulation located? |
|
Definition
|
|
Term
| The hypothalamus receives thermal receptor information from the anterolateral system indirectly through what? |
|
Definition
|
|
Term
| What part of the hypothalamus contains the detector neurons (mostly heat sensitive)? |
|
Definition
| Anterior hypothalamus, primarily in the preoptic nuclei |
|
|
Term
| What part of the hypothalamus contains the regulatory balance point? |
|
Definition
| The posterior hypothalamus |
|
|
Term
| Regulation of body temperature by the hypothalamus is accomplished via what mechanisms? |
|
Definition
Vasomotor control
Perspiration
Shivering and resting muscle tone
Regulation of basal metabolic rate through humoral mechanisms (TRH, TSH)
Brown fat metabolism
Cardiopulmonary control (heart rate, breathing)
More complex behavioral responses |
|
|
Term
| What is the function of the caudolateral center of the hypothalamus? |
|
Definition
Houses sympathetic effectors
Energy/heat expending/generating mechanisms, heat conservation, controls the "balance" point for body temp
Activation increases body temp via vasoconstriction
Regulates shivering |
|
|
Term
| What would be the consequences of a lesion through the caudolateral hypothalamus? |
|
Definition
| A decline in sympathetics leading to poikilothermia |
|
|
Term
| What regulates shivering and the activation of brown adipose tissue? |
|
Definition
| The caudolateral hypothalamus via the reticulospinal pathway |
|
|
Term
| What is the function of the rostromedial center of the hypothalamus? |
|
Definition
| Houses parasympathetics for energy conservation, heat dissipation, decreasing body temp via vasodilation and sweating |
|
|
Term
| A lesion through the rostromedial center of the hypothalamus would produce what effect? |
|
Definition
|
|
Term
| What structure allows for humoral thermoregulation by the hypothalamus? |
|
Definition
| The tuberoinfundibular pathways |
|
|
Term
| What stimulates the release of TRH? |
|
Definition
| Anterior hypothalamic nucleus |
|
|
Term
| What structure releases TRH following stimulation by the anterior hypothalamic nucleus? |
|
Definition
| Parvocellular area of the paraventricular nucleus |
|
|
Term
| What are the effects of TRH? |
|
Definition
| Induces TSH release from the anterior pituitary to increase the basal metabolic rate |
|
|
Term
| How will changes in blood temp affect TRH levels? |
|
Definition
Low blood temp increases TRH release
High blood temp causes reduced TRH release |
|
|
Term
| How is thermoregulation controlled neurally? |
|
Definition
Activates breakdown of brown adipose tissue and shivering/muscle tone
Both of these responses originate in the hypothalamus and are modulated through the reticular formation of the brainstem |
|
|
Term
|
Definition
| Gamma motor neurons respond to signals from the caudolateral hypothalamus via the reticulospinal tract |
|
|
Term
| What is normal body temp when taken orally? |
|
Definition
|
|
Term
| What is normal body temp when taken axillary? |
|
Definition
|
|
Term
| What is normal body temp when taken rectally? |
|
Definition
|
|
Term
| What variables can affect an accurate temperature measurement? |
|
Definition
| Time of day (0.5 C variance), age, sex, menstrual cycle, activity, drinks, temp changes can be more dramatic in children, etc. |
|
|
Term
|
Definition
| Core body temp <35 C or 95 F |
|
|
Term
| What is the most common cause of hypothermia? |
|
Definition
|
|
Term
| How does alcohol cause hypothermia? |
|
Definition
| Promotes vasodilation and reduces peripheral vasoconstriction |
|
|
Term
| Other than alcohol, what are other common causes of hypothermia? |
|
Definition
Neuroleptic drugs
Shivering response decreases with age
Hypothyroidism |
|
|
Term
| How do you know a patient has died from hypothermia? |
|
Definition
|
|
Term
| What are the consequences of hypothermia? |
|
Definition
Most symptoms and neurological deficits are fully reversible with rewarming unless hypothermia is severe and long lasting
Complications: pneumonia, pulmonary edema, cardiac arrhythmias, myoglobinuria, disseminated intravascular thrombosis, seizures |
|
|
Term
| How is mild hypothermia treated? |
|
Definition
| Passive (blankets, warm environment) or active external warming (heating lamps, hot bath) |
|
|
Term
| How do you treat moderate to severe hypothermia? |
|
Definition
Resuscitate as necessary. Avoid rapid peripheral warming –peripheral blood may be cold and produce a core temperature drop leading to cardiac atrial/ventricular fibrillation or arrest. Blood from peripheral ischemic areas can also disrupt blood chemistry and produce shock or vascular collapse
Try to warm the core temp first: warmed humidified oxyge, warm fluid lavage of body cavities, warm IV fluids
esophageal warming tube, cariopulmonary bypass, etc. |
|
|
Term
|
Definition
A core body temp >41C or 105.8F, though temps up to 42 C are usually tolerated
Past 42 C, cerebral oxygen consumption falls, EEG slows |
|
|
Term
| What are the most common causes of hyperthermia? |
|
Definition
Environmental heat exposure with or without exertion
Complications of anesthesia (malignant hyperthermia) in pts with inherited defects in skeletal muscle Ca++ transport
Neuroleptics that involve blockade of central dopamine receptors, causing neuroleptic malignant syndrome |
|
|
Term
| Hyperthermia is most common among what demographics? |
|
Definition
Young who overexert themselves in hot conditions
Elderly who cannot dissipate heat well at rest
Pts on anticholinergic drugs: TCAs, Antihistamines, neuroleptics, etc.
The very young |
|
|
Term
|
Definition
A form of hyperthermia where core temps may rise slowly and symptoms can develop over days
Can be water depleted, Na+ depleted or both |
|
|
Term
| What are the characteristics of water depleted heat exhaustion? |
|
Definition
| Most common in elderly or persons with pre-existing conditions or medications that predispose them to dehydration during hot weather. Symptoms – thirst, fatigue, dry mouth, decreased urine |
|
|
Term
| What are the characteristics of Na+ depleted heat exhaustion? |
|
Definition
| Common in unacclimated persons who maintain volume status with water, but don’t replace sodium. May be hyperhydrated- Symptoms-weakness, fatigue, headache may appear acutely, but onset is typically over several days |
|
|
Term
|
Definition
| A type of hyperthermia that may be associated with or precede heat exhaustion, likely secondary to sodium depletion or sodium osmotic imbalance |
|
|
Term
| How are heat cramps treated |
|
Definition
| Increase fluids containing electrolytes and to passively stretch muscles or massage |
|
|
Term
|
Definition
A type of hyperthermia where core temps rise very rapidly to 41C or 105F or greater
Considered a medical emergency, can be either exertional or non-exertional |
|
|
Term
| What demographics most commonly experience exertional heat stroke (EHS)? |
|
Definition
| Young people doing strenuous exercise over long periods in a hot environment. Second most common cause of death among high school athletes |
|
|
Term
| What demographics most commonly experience non-exertional heat stroke (NEHS)? |
|
Definition
| Elderly sedentary, chronically ill individuals and the very young |
|
|
Term
| How is hyperthermia treated? |
|
Definition
Rapid reduction of body temp
Evaporative cooling if between 38-38.8C
Ice baths or cool water immersions, careful not to induce shivering or hypothermia
Antipyretics such as dantrolene are ineffective |
|
|
Term
| What is malignant hyperthermia? |
|
Definition
A relatively rare complication of anesthesia (most often halothane and succinycholine) associated with an inherited disruption of Ca++ regulation in skeletal muscle
Causes rigid limbs, chest, or jaw w/in 30 minutes
Discontinue anesthesia and give dantrolene |
|
|
Term
| What is Neuroleptic Malignant Syndrome? |
|
Definition
Iatrogenic hyperthermia most associated with haloperidol but may occur with levodopa withdrawal and other neuroleptic drugs. Can begin 1-3days
Causes hyperthermia, rigidity, dysphagia, tremors, altered mental status, rhabdomyolysis
Give dantrolene and dopamine agonists (bromocriptine) |
|
|
Term
| What is Myasthenia gravis? |
|
Definition
| A chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal muscles |
|
|
Term
| What causes myasthenia gravis? |
|
Definition
Flattened postsynaptic folds w/a widened synaptic space
ACh produces small end-plate potential that may fail to trigger muscle APs
Due to an autoimmune response mediated by specific anti-AChR antibodies which reduces the number of available AChRs |
|
|
Term
| How do specific anti-AChR antibodies reduce available AChRs in myasthenia gravis? |
|
Definition
Accelerated turnover
Blockade of the active site
Damage to the postsynaptic muscle membrane by the antibody in collaboration with complements |
|
|
Term
| The pathogenic antibodies in myasthenia gravis are dependent on what cells? |
|
Definition
IgG and are T cell dependent
Immunotherapeutic strategies directed against T cells are effective |
|
|
Term
| Myasthenia gravis is more prevalent among what demographic? |
|
Definition
Females over males 3:2
Can present at any age, may be a bit later in men |
|
|
Term
| What are the clinical signs of myasthenia gravis? |
|
Definition
Ptosis (unilateral or bilateral, increases with upward gaze) and diplopia in ½ of cases
Cogan's sign (lid twitch when gaze moves from down to primary)
Difficulty chewing/swallowing, talking, snarling expression while smiling, limb weakness (often proximal and asymmetric), deep tendon rerflexes preserved, no change in sensation |
|
|
Term
| How is the thymus usually involved in myasthenia gravis? |
|
Definition
| 2/3 also have thymic hyperplasia, 10-15% have thymoma |
|
|
Term
| What is the group 1 classification for myasthenia gravis? |
|
Definition
|
|
Term
| What is the group 2A classification for myasthenia gravis? |
|
Definition
|
|
Term
| What is the group 2B classification for myasthenia gravis? |
|
Definition
| Moderate-severe generalized |
|
|
Term
| What is the group 3 classification for myasthenia gravis? |
|
Definition
|
|
Term
| What is the group 4 classification for myasthenia gravis? |
|
Definition
|
|
Term
| What are some common autoimmune disorders that are often associated with myasthenia gravis? |
|
Definition
| Lupus erythematosus (SLE) and rheymatoid arthritis (RA) as well as many others |
|
|
Term
| What drugs can predispose people to myasthenia gravis? |
|
Definition
D-penicillamine
Nitrofurantoin
Aminoglycosides
Polymyxins |
|
|
Term
| How does D-penicillamine predispose people to myasthenia gravis? |
|
Definition
| It can induce true myasthenia with elevated anti-AChR antibody titers. However, the weakness is mild, and full recovery is achieved weeks to months after discontinuation |
|
|
Term
| How does nitrofurantoin predispose people to myasthenia gravis? |
|
Definition
| Has been linked to the development of ocular MG; discontinuation of the drug also resulted in complete recovery |
|
|
Term
| What type of physical finding would immediately be a red flag for myasthenia gravis? |
|
Definition
| Any acquired ocular motility disturbance with or without ptosis, but normally reacting pupils |
|
|
Term
| What is characteristics of the muscle weakness seen in myasthenia gravis? |
|
Definition
Weakness in facial, oropharyngeal, limbal, and trunk muscles, without any other sign of neurologic deficit such as sensory loss, change in deep tendon reflexes, or muscle atrophy
Weakness is usually proximal and asymmetric |
|
|
Term
| How is myasthenia gravis diagnosed? |
|
Definition
Mostly clinical but should be confirmed definitively before treatment due to probable surgery or drug therapy
The sleep test, edrophonium test, neostigmine test, and presence of ACh-R antibodies is indicative but not diagnostic of MG |
|
|
Term
| What is the sleep test for myasthenia gravis? |
|
Definition
Patient sleeps for 30min and is re-examined for changes
Typically, an improvement can be seen in MG patients lasting 2-5min |
|
|
Term
| What is the edrophonium test for myasthenia gravis? |
|
Definition
2mg intravenous edrophonium initially test for tolerance of the drug. If no response or adverse reaction within 1min, give remaining 8mg
Atropine should be readily available for cholinergic side-effects of the test, should they be severe
Can only be used when an observable abnormality such as ptosis is patient, improvement implies MG |
|
|
Term
| What are the contraindications for the Edrophonium test and the the Neostigmine test? |
|
Definition
A history of asthma or cardiac dysrhythmias is a relative contraindication
May cause sinus bradycardia
A small percentage of patients are hypersensitive |
|
|
Term
| What is the Neostigmine test? |
|
Definition
Performed when the edophonium test fails
Intramuscular neostigmine 0.5-1.0mg is injected to detect improvements in neuromuscular transmission, seen within 30 minutes
Most useful in infants and children who respond too briefly to edrophonium
Also requires an observable abnormality such as ptosis that notably improves |
|
|
Term
| What is the diagnostic test of choice for myasthenia gravis? |
|
Definition
Detection of ACh-receptor antibodies
Not entirely diagnostic, false negative is possible
Serum ocncentrations of Abs vary wildly |
|
|
Term
| What is repetitive nerve stimulation EMG and how is associated with myasthenia gravis? |
|
Definition
Repetitive stimulation of muscle at 1-5Hz; postsynaptic potentials do not reach threshold and fail to generate an action potential
Significant decrement seen in 60% of MG pts |
|
|
Term
| What is Single Fiber EMG? |
|
Definition
Simultaneously records potentials of two muscle fibers innervated by an individual axon
High sensitivity |
|
|
Term
| What is the most sensitive test for myasthenia gravis? |
|
Definition
|
|
Term
| Single Fiber EMG is best suited for confirming what types of myasthenia gravis? |
|
Definition
Generalized and ocular
When the test site includes facial muscles, abnormal jitter is not specific for MG; may occur in other neuromuscular disorders, including ALS, polymyositis or LEMS
More specific for MG if large degree of jitter occurs with mild or no other changes on EMG |
|
|
Term
| Though myasthenia gravis cannot be predicted or prevented, what existing drugs often aggravate the condition? |
|
Definition
Certain anti-infectives, including aminoglycoside antibiotics, ciprofloxacin, chloroquine
Some mood stabilizers such as lithium and phenothiazines
Certain antiarrhythmics or agents to lower blood pressure such as phenytoin, procainamide, quinidine, beta-blockers, calcium channel blockers
Magnesium-containing antacids
Iodinated contrast agents |
|
|
Term
| Myasthenia gravis patients should never be given what drugs? |
|
Definition
| Penicillamine (used for scleroderma or rheumatoid arthritis) or interferon-alpha |
|
|
Term
| What is Nonautoimmune Congenital Myasthenic Syndrome (CMS)? |
|
Definition
A heterogeneous group of disorders of the NMJ that are not autoimmune but rather are due to genetic mutations in which virtually any component of the NMJ may be affected
Alterations in function of the presynaptic nerve terminal or in the various subunits of the AchR or AChE have been identified in various forms |
|
|
Term
| When should Nonautoimmune Congenital Myasthenic Syndrome (CMS) be suspected? |
|
Definition
| If symptoms of myasthenia have begun in infancy or childhood and AChR antibody tests are consistently negative |
|
|
Term
| What is Lambert-Eaton myasthenic syndrome (LEMS)? |
|
Definition
A presynaptic disorder of the NMJ that can cause weakness similar to that of MG
Due to autoantibodies directed against P/Q type calcium channels at the motor nerve terminals (~85%; detected by radioimmunoassay) |
|
|
Term
| Most patients with Lambert-Eaton myasthenic syndrome (LEMS) also have what associated presentation? |
|
Definition
Malignancy, most commonly small-cell carcinoma of the lung
The proximal muscles of the lower limbs are most commonly affected but can affect other muscles too
Ptosis and diplopia are common
Depressed deep tendon reflexes |
|
|
Term
| How is Lambert-Eaton myasthenic syndrome (LEMS) treated? |
|
Definition
Plasmapheresis and immunosuppression (temporary relief)
3,4 Diaminopyridine (blocks K+ channels, prolongs depolarization to enhance ACh release)
Pyridostigmine (prolongs the action of ACh, allowing repeated interactions with AChRs) |
|
|
Term
| How is Lambert-Eaton myasthenic syndrome (LEMS) distinguished from myasthenia gravis? |
|
Definition
Patients with LEMS have depressed or absent reflexes, unlike in myasthenia gravis
LEMS patients show autonomic changes such as dry mouth and impotence
On repetitive nerve stimulation LEMS patients show incremental rather than decremental responses |
|
|
Term
|
Definition
The historic term for a myasthenia-like fatigue syndrome without an organic basis
These patients present with subjective symptoms of weakness and fatigue
Muscle testing usually reveals the "jerky release" or "give-away weakness" characteristic of nonorganic disorders
Usually due to fatigue or apathy, not muscle power |
|
|
Term
| What are potential differential diagnoses when suspecting myasthenia gravis? |
|
Definition
| Hyperthyroidism, botulism, inctracranial mass lesions |
|
|
Term
| What is chronic progressive external ophthalmoplegia? |
|
Definition
| A rare condition resulting in weakness of the EOMs, which may be accompanied by weakness of the proximal muscles of the limbs and other systemic features |
|
|
Term
| Chronic progressive external ophthalmoplegia is associated with what kind of disorders? |
|
Definition
Most have mitochondrial disordered detected on muscle biopsy via modified trichrome stains
Mitochondria exhibit "ragged red fibers"
Is NOT autoimmune |
|
|
Term
| How is myasthenia gravis treated? |
|
Definition
Virtually all patients can return to full productivity
Anticholinesterase drugs: Pyrodistigmine
Immunosuppressive agents
Thymectomy
Plasmapheresis (temporary)
IV immunoglobulin (Ig) |
|
|
Term
| What are the side effects of using Pyrodistigmine (anticholinesterase drug of choice) to treat myasthenia gravis? |
|
Definition
Increased weakness
Diarrhea
Abdominal cramps (Atropine/diphenoxylate or loperamide is useful)
Salivation
Nausea |
|
|
Term
| What should be given to patients with myasthenia gravis if immediate improvement is essential? |
|
Definition
| IV Ig alongside plasmapheresis |
|
|
Term
| What should be prescribed for myasthenia gravis for intermediate term improvement (1-3 months)? |
|
Definition
| Glucocorticoids and cyclosporine |
|
|
Term
| What are the pros and cons of using azathioprine and mycophenolate mofetil to treat myasthenia gravis? |
|
Definition
| Beneficial effects can take many months, up to a year, but can have long-term advantages |
|
|
Term
| Patients that are refractory to immunosuppressives when being treated for myasthenia gravis may benefit from what? |
|
Definition
| A high-dose cyclophosphamide may possibly "reboot" the immune system, possible permanent benefits |
|
|
Term
| When should thymectomy be prescribed in patients with myasthenia gravis? |
|
Definition
| Should be carried out in all patients between puberty and 55yo |
|
|
Term
| What is a myasthenic crisis? |
|
Definition
| A critical condition of myasthenia gravis where an an exacerbation of weakness is sufficient to endanger life; it usually consists of respiratory failure caused by diaphragmatic and intercostal muscle weakness |
|
|
Term
| How is myasthenic crisis treated? |
|
Definition
| In an ICU with physicians experienced in MG respiratory insufficiency, infectious dx, and fluid and electrolyte therapy |
|
|
Term
| What is the most common cause of myasthenic crisis? |
|
Definition
| Intercurrent infection, should be treated immediately |
|
|
Term
| What is essential in the treatment for myasthenic crisis? |
|
Definition
Early and effective antibiotic therapy, respiratory assistance, and pulmonary physiotherapy are essentials of the treatment program
Plasmapheresis or IVIg is frequently helpful in hastening recovery |
|
|
Term
| What lab value is considered indicative of anemia? |
|
Definition
|
|
Term
| How are RBC values measured? |
|
Definition
| RBC is not a measured value |
|
|
Term
|
Definition
|
|
Term
| When is anemia a normal process of aging? |
|
Definition
|
|
Term
| What are the symptoms of anemia? |
|
Definition
| Palor, pica, palpitations, orthostasis, edema, chest pain, dyspnea upon exertion |
|
|
Term
| What is often the first sign of anemia? |
|
Definition
|
|
Term
| How does anemia affect the reticulocyte count? |
|
Definition
Increased in hyperproliferative anemia
Decreased in hypoproliferative anemia |
|
|
Term
| What causes hyperproliferative anemia |
|
Definition
| Can either be due to bleeding or due to hemolysis |
|
|
Term
| What lab changes would you expect in hemolyzing hyperproliferative anemia? |
|
Definition
Increased LDH LDH due to RBC lysis
Dec haptoglobin which binds Hg. If Hg is high, haptoglobin is low
Increased bilirubin may be associated |
|
|
Term
| How do you screen for hemolytic anemia? |
|
Definition
Coomb's test is positive
Screens for antibodies directed against RBCs |
|
|
Term
| What do you screen for in a Coomb's test? |
|
Definition
IgG if ppl have been given penicillin (drugs are MC)
IgM otherwise |
|
|
Term
| IgG detection on a Coomb's test indicates what? |
|
Definition
Presence of a warm antibody directed against RBCs
Often associated with penicillin use |
|
|
Term
| IgM detection on a Coomb's test indicates what? |
|
Definition
| Presence of a cold antibody directed against RBCs |
|
|
Term
| What might cause Coomb's positive hemolytic anemia? |
|
Definition
SLE
Ideopathic
Drug Induced
Lymphoproliferative Disorders |
|
|
Term
| How do you treat a case of Coomb's positive hemolytic anemia? |
|
Definition
Steroids to suppress the offending antibody
Splenectomy if immunosuppression fails
Treat underlying causes (such as SLE)
Give folic acid to promote RBC production |
|
|
Term
| What might cause Coomb's negative hemolytic anemia? |
|
Definition
Membrane abnormality
Enzyme abnormality
Hemoglobinopathy such as sickle cell or sickle cell–hemoglobin C (HbSC) |
|
|
Term
| What is the most common membrane abnormality that causes Coomb's negative hemolytic anemia? |
|
Definition
|
|
Term
| What are some problems and complications associated with sickle cell disease? |
|
Definition
Painful crisis
Nonhealing ulcers
Sclerodactyly
Autosplennectomy (spleen is missing)
Pneumococcal pneumonia
Aplastic crisis
Cholelithiasis (young)
Stroke
Priapism
Chest Wall Syndrome (must give a transfusion) |
|
|
Term
| How can sickle disease cause a painful crisis? |
|
Definition
| Due to vaso-occlusion by sickle cells causing pain, discomfort, and organ dysfunction |
|
|
Term
| How can sickle cell disease cause an aplastic crisis? |
|
Definition
| Patients cannot produce RBCs as efficiently following the flu (parvovirus), Hg can plummet. Treated with a blood transfusion |
|
|
Term
| What events can exacerbate anemia in sickle cell patients? |
|
Definition
Aplastic crisis
Acute splenic sequestration
Acute chest syndrome
Vasoocclusive crisis
Folate deficiencies |
|
|
Term
| How is sickle cell treated? |
|
Definition
Hydration
Folate supplements
Pain control (narcotics only during a crisis)
Treat for encapsulated organisms
Hydroxyurea
Exchange transfusions |
|
|
Term
| What is the action of Hydroxyurea? |
|
Definition
Makes RBCs less sticky, increases fetal Hb and decreases SS Hb
Used to treat sickle cell anemia |
|
|
Term
| What enzyme abnormalities can cause anemia? |
|
Definition
G6PD deficiency often due to fava beans
Pyruvate kinase deficiencies |
|
|
Term
| How is Hypoproliferative anemia classified? |
|
Definition
MCV labs
Compare size of cell under light microscopy to a lymphocyte |
|
|
Term
| What is the difference between beta and alpha thallasemia? |
|
Definition
| Dictated by the absence of either alpha or beta chains |
|
|
Term
| What are the characteristics of Mediterranean anemia? |
|
Definition
Low levels of red blood cells
Poor growth
Huge abdominal organs
Early childhood death
RBC hyperproduction causes bone abnormalities and spleen enlargement |
|
|
Term
| How do you treat beta thallasemia? |
|
Definition
If minor (only 1 loci missing), no treatment needed
If major (both loci missing), do transfusion protocols and bone marrow transplants |
|
|
Term
| What lab values do you usually see in iron deficiency anemia? |
|
Definition
Fe low
TIBC is high
Ferritin is low
Tranferrin is low |
|
|
Term
| What is the best screening tool for iron deficiency anemia? |
|
Definition
| Ferritin levels will be low |
|
|
Term
| Iron deficiency anemia due to an inability to absorb enteral iron is often found in patients with what types of conditions? |
|
Definition
Celiac disease
Gastric bypass surgery patients
Chron’s Disease |
|
|
Term
| How do you treat iron deficiency anemia? |
|
Definition
Oral iron supplements (often inadequate)
IV iron, particularly in celiac or gastric bypass pts
Remember to give a test dose of iron to avoid anaphylaxis |
|
|
Term
| How do you distinguish anemia due to chronic disease from iron deficiency anemia |
|
Definition
| TIBC will be low instead of high as in iron deficiency anemia |
|
|
Term
| How do you treat anemia due to chronic disease? |
|
Definition
Treat underlying disease
Usually no treatment needed as Hb resets but you can give exogenous erythropoietin to stimulate RBC production |
|
|
Term
| What causes megaloblastic anemia? |
|
Definition
|
|
Term
| B12 deficiencies (and subsequent megaloblastic anemia) occurs as a result of what conditions? |
|
Definition
Chronic pancreatitis
Overgrowth
Inflammatory bowel disease
Gastric bypass surgery
Pernicious anemia
Strict vegans
Atrophic gastritis - impaired absorption
Medications: cholestyramine, metformin, colchicine
Rare congenital disorders (IF or receptor lacking) |
|
|
Term
| What is the difference between primary and secondary causes of myeloproliferative disorders? |
|
Definition
Bone marrow problems = primary
Responses to other disease processes = secondary |
|
|
Term
| What is the definition of a myeloproliferative disorder? |
|
Definition
| Blood disorders in which some component of the bone marrow is produced in excess, causing a clinical problem |
|
|
Term
| Polycythemia rubra vera is a myeloproliferative disorder associated with what cell types? |
|
Definition
|
|
Term
| Essential thrombocytosis is a myeloproliferative disorder associated with what cell types? |
|
Definition
|
|
Term
| Agnogenic myeloid metaplasia is a myeloproliferative disorder associated with what type of tissues? |
|
Definition
Stromal tissue
Also known as myelofibrosis, osteomyelofibrosis, or primary myelofibrosis |
|
|
Term
| Chronic myelogenous leukemia is a myeloproliferative disorder associated with what cell types? |
|
Definition
|
|
Term
| What gene mutation is associated with myeloproliferative disorders? |
|
Definition
| JAK2-V617F point mutation causing continual activation or hypersensitivity to signals |
|
|
Term
| How is the JAK2 gene associated with myeloproliferative disorders? |
|
Definition
| Acts as an intracellular signaling pathway of hematopoietic progenitor cells |
|
|
Term
| What is Relative Polycythemia? |
|
Definition
| A false appearance of an over-proliferation of blood cells due to a contracted plasma volume in response to diuretic therapy or dehydration |
|
|
Term
| What is Gaisbock’s syndrome? |
|
Definition
Secondary polycythemia seen in young, unhealthy males
Presentation: young male executive, sedentary, smoker, high stress = low plasma volume due to inadequate hydration |
|
|
Term
| What is true Polycythemia? |
|
Definition
| An over-proliferation of blood cells |
|
|
Term
| What is secondary true polycythemia? |
|
Definition
| An over-proliferation of blood cells due to hypoxia induced RBC production or erythropoietin secretion |
|
|
Term
| What might increases erythropoietin secretion, leading to secondary polycythemia? |
|
Definition
Cardiac abnormalities (R->L shunts)
Pulmonary abnormalities
Sleep apnea
Renal disease
Hepatoma |
|
|
Term
| What is primary true polycythemia (aka polycythemia rubra vera)? |
|
Definition
| Unopposed proliferation of red blood cells causing thickened blood theorized to be an inappropriate response to erythropoietin |
|
|
Term
| What are the potential complications of polycythemia rubra vera? |
|
Definition
Stoke, heart attack, DVT, Budd-Chiari syndrome
All due to thickened blood |
|
|
Term
| What are the symptoms of polycythemia rubra vera? |
|
Definition
Subtle and gradual changes, often unrecognized
Lethargy/fatigue
Palmar erythema
Systemic hypertension
Splenomegaly
Epistaxis, GI, or mucocutaneous bleeding
Plethora: A ruddy complexion in the face, palms, nailbeds, mucosa, and conjunctiva due to increased RBC mass, usually very gradual
Erythromelalgia - decreased blood flow in the digits causes sudden burning pain and a reddish/blue discoloration |
|
|
Term
| What common sign in polycythemia rubra vera indicates that the patient is due for phlebotomy treatment? |
|
Definition
| Palmar erythema (disappears after treatment) |
|
|
Term
| How is polycythemia rubra vera diagnosed? |
|
Definition
Clinical diagnosis based on increased RBC mass, splenomegaly, overlap with other myeloproliferative disorders, and a absence of secondary conditions to explain polycythemia
Must fullfil both major and one minor or the first major and two minor diagnostic criteria |
|
|
Term
| What are the major diagnostic criteria for polycythemia rubra vera? |
|
Definition
Either high Hct or high Hb
JAK2 mutation |
|
|
Term
| What are the minor diagnostic criteria for polycythemia rubra vera? |
|
Definition
Hypercellular bone marrow
Low erythropoietin level
Endogenous erythroid colony formation |
|
|
Term
| What is considered a high Hct and a high Hb? |
|
Definition
Hct > 55% in men, > 50% in women
Hb >18.5 in men, >16.5 in women |
|
|
Term
| What characterizes the spent phase of polycythemia? |
|
Definition
Seen late in life in patients having received phlebotomy therapy for years, Hb declines due to bone marrow becoming fibrotic. Therapy is no longer necessary
However, the decline is occasionally too sharp, causing anemia which requires transfusions |
|
|
Term
| What is the tipping point when blood viscosity begins to rapidly increase? |
|
Definition
|
|
Term
| What is the target range of Hct for Polycythemia rubra vera patients? |
|
Definition
|
|
Term
| How is polycythemia treated? |
|
Definition
Hydroxyurea to lower RBC production
Regular phlebotomy to develop iron deficiency |
|
|
Term
| What are the potential secondary causes of thrombocytosis that must be ruled out before making a diagnosis? |
|
Definition
Iron deficiency (reactive thrombocytosis)
Inflammatory conditions
Malignancy
Infection |
|
|
Term
| What are the major diagnostic criteria for essential thrombocytosis? |
|
Definition
Platelet count > 450k
Bone marrow with proliferation of megakaryocytic lineage
Not meeting WHO criteria for PCV, Primary myelofibrosis, CML, MDS or other
Demonstration of JAK 2 or other clonal marker or in the absence of a marker, no evidence for reactive thrombocytosis |
|
|
Term
| What are the clinical features of essential thrombocytosis? |
|
Definition
Vasomotor changes: visual disturbances, dizziness, burning of palms/soles, paresthesia, seizures
Thrombus or clot formation
High incidence of hemorrhage due to defective platelets |
|
|
Term
| What is the treatment goal for essential thrombocytosis? |
|
Definition
Prevent complications
Only treat if symptomatic or in high risk of bleeding or thrombus |
|
|
Term
| What drugs are used to control the platelet count in patients with essential thrombocytosis? |
|
Definition
Anegralide
Hydroxyurea
Interferon (only in pregnant or if refractory) |
|
|
Term
| What drugs are used to prevent thrombus formation? |
|
Definition
Aspirin
Plavix, Heparin, Coumadin (use with caution, bleeding hazard) |
|
|
Term
| What is Agnogenic Myeloid Metaplasia? |
|
Definition
Aka primary myelofibrosis (bone marrow fibrosis)
Extremely rare |
|
|
Term
| How is Agnogenic Myeloid Metaplasia diagnosed? |
|
Definition
Difficult using blood count alone
Confirmed by peripheral smear, exam, and bone marrow biopsy |
|
|
Term
| What are the symptoms of splenomegaly? |
|
Definition
Early satiety due to compression on stomach
Abdominal discomfort
Splenic infarcts (very painful, can present as chest or shoulder pain)
Portal hypertension |
|
|
Term
| What causes massive splenomegaly in patients with myelofibrosis disorders? |
|
Definition
| Compensatory extramedullary hematopoiesis |
|
|
Term
| A leukoerythroblastic peripheral smear classic for Agnogenic Myeloid Metaplasia would show what? |
|
Definition
Nucleated RBCs
Teardrop-shaped cells |
|
|
Term
| What is dry tap bone marrow? |
|
Definition
| Bone marrow so full of stromal tissue that there is very little liquid, preventing aspiration for biopsies |
|
|
Term
| What is needed to confirm fibrosis stromal tissue crowding the marrow? |
|
Definition
|
|
Term
| How is Agnogenic Myeloid Metaplasia treated? |
|
Definition
Transfusion support
Bone marrow transplant
JAK 2 inhibitors
Prognosis is poor |
|
|
Term
| Which myeloproliferative disorders have the greatest likelihood of transforming into acute leukemia? Which has the least? |
|
Definition
| Untreated chronic myeloid leukemia > agnogenic myeloid metaplasia > polycythemia Rubra Vera > essential thrombocytosis |
|
|
Term
| A bone marrow biopsy of a patient with Chronic Lymphocytic Leukemia would show what? |
|
Definition
| Resembles a peripheral smear with an overabundance of lymphocytes |
|
|
Term
| What is the difference between lymphocytes and neutrophils? |
|
Definition
Lymphocytes differentiate foreign from self, mediates immunity and antibody formation
Neutrophils actually fight infections
Both are very small cells |
|
|
Term
| What are the symptoms of Chronic Lymphocytic Leukemia? |
|
Definition
Fever, night sweats, weight loss, Abd discomfort
Early satiety due to splenomegaly, bleeding fatigue
Most do not have disease-related symptoms at diagnosis |
|
|
Term
| Chronic Lymphocytic Leukemia primarily affects what demographics? |
|
Definition
| Primarily elderly, more likely in males but can affect both genders |
|
|
Term
| What is the most common cause of death in Chronic Lymphocytic Leukemia? |
|
Definition
|
|
Term
| Secretion of abnormal Ig by lymphocytes in patients with Chronic Lymphocytic Leukemia has what consequence? |
|
Definition
| Without proper amounts of normal Ig, patients can develop hypogammaglobulinemia which predisposes them to infection |
|
|
Term
| What is most likely to cause infection Chronic Lymphocytic Leukemia prior to immunosuppression therapy? |
|
Definition
Streptococcus pneumoniae
Staphylococcus aureus
Haemophilus influenzae |
|
|
Term
| What is most likely to cause infection Chronic Lymphocytic Leukemia following to immunosuppression therapy? |
|
Definition
Gram-negative organisms
Candida, Listeria, Pneumocystis carinii, cytomegalovirus, Aspergillus, herpes virus |
|
|
Term
| How is chronic lymphocytic leukemia diagnosed? |
|
Definition
Lymphocytosis of >5e^9/L in blood persisting >4wks
>30 lymphocytosis in bone marrow
Cell markers confirm diagnosis: Monoclonal surface Ig: CD5+; sparse surface Ig |
|
|
Term
| What are the most common physical findings in chronic lymphocytic leukemia? |
|
Definition
Lymphadenopathy
Splenomegaly and/or hepatomegaly |
|
|
Term
| What is the most important diagnostic test for chronic lymphocytic leukemia? |
|
Definition
| Cell markers showing monoclonal surface Ig -> CD5+ sparse surface Ig |
|
|
Term
| What aggressive transformations are potential complications of chronic lymphocytic leukemia? |
|
Definition
Richter's Syndrome (large-cell lymphoma) occurs in 10%
Prolymphocytic Leukemia |
|
|
Term
| What are the symptoms of Richter's Syndrome (large-cell lymphoma, a complication of chronic lymphocytic leukemia)? |
|
Definition
Lymphadenopathy
Hepatosplenomegaly
Fever, abd pain, weight loss
Progressive anemia and Thrombocytopenia
Elevated lactate dehydrogenase
Increasing peripheral lymphocytosis
Markedly elevated WBCs |
|
|
Term
| What are the symptoms of prolymphocytic anemia (a complication of chronic lymphocytic leukemia)? |
|
Definition
Progressive anemia
Thrombocytopenia
>55% prolymphocytes in peripheral blood
Lymphadenopathy and hepatosplenomegaly
Wasting syndrome
Increasing resistance to therapy |
|
|
Term
| How does treatment of chronic lymphocytic leukemia differ when the patient presents with Richter's syndrome? |
|
Definition
| Will rapidly progress, requires treatment |
|
|
Term
| How does treatment of chronic lymphocytic leukemia differ when the patient presents with prolymphocytic leukemia? |
|
Definition
| Will rapidly progress, requires treatment |
|
|
Term
| What are the long term complications of chronic lymphocytic leukemia? |
|
Definition
Infections, many are life threatening
Autoimmune phenomena
Richter's transformation
Secondary malignancies
Bladder and prostate cancer, especially if family hx is present |
|
|
Term
| While the familial risk for chronic lymphocytic leukemia is low, it has the highest familial risk with what type of malignancies? |
|
Definition
| Hematological malignancies |
|
|
Term
| What Binet findings suggest the greatest odds for survival (>120mo) for chronic lymphocytic leukemia? |
|
Definition
| Hb > 10, Plts > 100, < 3 involved areas |
|
|
Term
| What Binet findings suggest intermediate odds for survival (84mo) for chronic lymphocytic leukemia? |
|
Definition
| Hb > 10, Plts > 100, > 3 involved areas |
|
|
Term
| What Binet findings suggest lowest odds for survival (24mo) for chronic lymphocytic leukemia? |
|
Definition
|
|
Term
| What are potential areas involved in chronic lymphocytic leukemia? |
|
Definition
Cervical, miliary, or inguinal nodes
Spleen or liver |
|
|
Term
| What are the important prognostic factors that help predict a poor outcome for chronic lymphocytic leukemia? |
|
Definition
Stage at diagnosis
Lymphocyte doubling time
Diffuse bone marrow infiltration
Older age, male gender
High serum B2-microglobulin
Soluble CD23 |
|
|
Term
| What is the most important sign indicating a patient with chronic lymphocytic leukemia needs to be treated? |
|
Definition
Shortened doubling time, anemia, or thrombocytopenia
Also in cases of bulky, uncomfortable adenopathy
Is otherwise an incurable disease |
|
|
Term
| How is chronic lymphocytic leukemia treatd? |
|
Definition
Chemotherapy most often using alkylating agents such as chlorambucil with prodnisone
Also purine analogs such as fludarabine, 2CDA, or pentostatin
Passive immune therapy with Rituximab or Alemtuzumab |
|
|
Term
| What needs to be present in order to confirm a diagnosis of chronic lymphocytic leukemia with flow cytometry of the peripheral blood? |
|
Definition
| Both B and T cell markers |
|
|
Term
| What is multiple myeloma? |
|
Definition
| Uncontrolled proliferation of Ig secreting plasma cells, most commonly IgG |
|
|
Term
| What is the most commonly secreted immunoglobulin in multiple myeloma? |
|
Definition
|
|
Term
| Multiple Myeloma is most common among what demographics? |
|
Definition
| Men and African AMericans |
|
|
Term
| What are the clinical markers that present with multiple myeloma? |
|
Definition
Monoclonal proteins (almost universal)
Increased plasma cells in the bone marrow (almost universal)
Lytic bone lesions
Anemia
Hypercalcemia
Renal failure
Infection |
|
|
Term
| Why are lytic bone lesions common in multiple myeloma? |
|
Definition
The plasma cells also secrete osteoclast necrosis factor
Also increases Ca++ levels, leading to kidney problems |
|
|
Term
| What are the major symptoms of multiple myeloma? |
|
Definition
Bone pain
Fatigue
Weight loss
Paresthesias (less common)
11% are asymptomatic or mild |
|
|
Term
| What do you expect to find on a serum protein electrophoresis in a patient with multiple myeloma? |
|
Definition
IgG (57%)
IgA (21%)
IgD (1%)
IgM or IgE almost never
Light chain (18%, more commonly seen in the urine) |
|
|
Term
| What x-ray findings are expected in multiple myeloma patients? |
|
Definition
|
|
Term
| What do bone scans detect in patients with multiple myeloma? |
|
Definition
| Blastic lesions, may appear normal |
|
|
Term
| What is the treatment goal for multiple myeloma? |
|
Definition
No cure, treated to decrease progression and prevent dialysis, hypercalcemia, or becoming crippled from bone damage
Hemodialysis in cases of renal insufficiency |
|
|
Term
| What is used to treat multiple myeloma? |
|
Definition
Thalidomide
Revlimid (synthetic thalidomide, more potent)
Steroids for hypercalcemia
Bisphosphonates for bone disease |
|
|
Term
| How is Revlimid different from Thalidomide? |
|
Definition
| More potent, no peripheral neuropathy or sedation |
|
|
Term
| What is required to confirm a diagnosis of multiple myeloma? |
|
Definition
| Bone marrow biopsy, classically shows sheets of plasma cells |
|
|
Term
| Why is handedness relevant when evaluating a potential stroke patient? |
|
Definition
| For right handed your language center is in your left hemisphere. If pt. is left-handed their language center could be on either side. This helps localize the area of stroke |
|
|
Term
| What are the characteristics of the mental status exam? |
|
Definition
Alertness
Orientation
Attention
Registration/Recall
Repetition (test ability)
Naming
Three stage commands
Writing
Construction/copying ability
Behavior/appearance/mood
Rate of speech
Content of thought
Intellectual capacity
Insight and judgement |
|
|
Term
| A loss of sense of smell is most likely due to what? |
|
Definition
Tumor until proven otherwise compressing CN I (olfactory)
Can precede parkinson's
Gradually lost in smokers |
|
|
Term
| A loss of CN VI (Abducens) causes what defect? |
|
Definition
| Associated eye is turned medially/inward |
|
|
Term
| A loss of CN IV (Trochlear) causes what defect? |
|
Definition
Associated eye is raised upward
Head tilts to opposite shoulder to compensate |
|
|
Term
| A loss of CN V (Trigeminal) will cause what defects? |
|
Definition
Loss of corneal reflex (eye closing when touched)
Sensation in V1, V2, and V3
Loss of mastication
Jaw jerk
Jaw deviates toward side of lesion if in motor part of CNV |
|
|
Term
| A loss of CN VII (Facial) will cause what defects? |
|
Definition
Facial expression loss
Taste loss in anterior 2/3 of tongue
Bell's palsy if LMN |
|
|
Term
| What tests are used to evaluate CN VIII (Vestibulocochlear)? |
|
Definition
Weber and Rinne
Audiology tests are more common |
|
|
Term
| Palate dropping and uvula deviation indicates what? |
|
Definition
|
|
Term
| A palate droop indicates a CN IX and X lesion on which side? |
|
Definition
|
|
Term
| Uvula deviation indicates a CN IX and X lesion on which side? |
|
Definition
| Contralateral to the deviation |
|
|
Term
| A loss of CN XII (Hypoglossal) will cause what defects? |
|
Definition
LMN causes atrophy and tongue deviation to lesion side
UMN will show less atrophy and tong deviation to contralateral side
Common in neck cancers |
|
|
Term
|
Definition
| Difficulty of movement in all directions |
|
|
Term
|
Definition
| Difficulty only in one direction of movement |
|
|
Term
| How do you test stereognosis? |
|
Definition
| Put something in their hand, like a key, and ask them to tell you what it is |
|
|
Term
| A loss of sterognosis is common in what kind of lesions? |
|
Definition
|
|
Term
|
Definition
| Neglect of one side of the body. They deny one part of their body is even theirs |
|
|
Term
| How do you test graphesthesia? |
|
Definition
| Draw a letter or number or letter on their hand and have them tell you what it is |
|
|
Term
| A patient with a cerebellar lesion would exhibit what symptoms? |
|
Definition
Wide based tremors when moving finger to nose or heel to shin on the ipsilateral side
Impaired rapid alternating movements/rapid repetitive movements also known as dysdiadochokinesia
Rebound
Wide based gait |
|
|
Term
| Upper motor nerve lesions tend to cause what reflexes? |
|
Definition
Hoffman's reflex
Tromner's reflex
Babinski sign |
|
|
Term
| What are frontal releasing signs for dementia patients? |
|
Definition
Palmomental twitch in the chin when stroking a specific part of the palm
Grasp, palm, and snout signs
Glabellar sign (can't stop blinking with forhead tap) |
|
|
Term
| Lower motor nerve lesions can be tested using what signs? |
|
Definition
Anal wink
Cremasteric reflex
Bulbocavernosus sign
Abdominal reflex (umbilicus goes to side of touch) |
|
|
Term
| The anal wink reflex uses which nerves? |
|
Definition
|
|
Term
| The cremasteric reflex uses which nerves? |
|
Definition
|
|
Term
| The abdominal reflex (umbilicus goes to side of touch) uses which nerves? |
|
Definition
|
|
Term
| The bulbocavernosus reflex uses which nerves? |
|
Definition
|
|
Term
| Muscle strength is graded out of what? |
|
Definition
|
|
Term
| Neural reflex is graded out of what? |
|
Definition
4/4 = clonus
2/4 = normal
0/4 = absent |
|
|
Term
| A spastic gait indicates what kind of injury? |
|
Definition
|
|
Term
| A wide leg swing gait indicates what kind of injury? |
|
Definition
|
|
Term
| A wide based gait indicates what kind of injury? |
|
Definition
|
|
Term
| What is the Romberg sign? |
|
Definition
Patient wobbles when their feet are together and eyes are closed
Difficulty going down stairs or walking at knight |
|
|
Term
| What is apraxia and where is it seen? |
|
Definition
| Loss of cortical brain functions, patients cannot walk, seen in dementia |
|
|
Term
| A shuffling gait indicates what kind of injury? |
|
Definition
|
|
Term
| What are some overt signs of increased intracranial pressure? |
|
Definition
| HTN, Bradycardia, Irregular Respirations |
|
|
Term
| What is indicated by battle sign/raccoon eyes? |
|
Definition
Fracture of the middle cranial fossa of the skull
Potential brain trauma |
|
|
Term
| Splinter hemorrhages on fingernails indicate what? |
|
Definition
|
|
Term
| How do you test whether patient is actually comatose or not? |
|
Definition
| Poke inside of their nose with a pin |
|
|
Term
| What metabolic dysfunctions affect the pupils? |
|
Definition
Normally not affected
Potentially impacted in toxic metabolic encephalopathy |
|
|
Term
| A midbrain injury can have what kind of effect on the pupils? |
|
Definition
| Mid-position pupils that are difficult to see react |
|
|
Term
|
Definition
| Pupils that are small but react |
|
|
Term
| How do most drugs affect pupils? |
|
Definition
Most often larger
Opiates make them small, miotic |
|
|
Term
| Unilateral, fixed, and dilated pupils indicate what kind of injury? |
|
Definition
Aneurysm - patient is usually awake, may complain of the "worst headache in their life"
Herniation - patient is usually comatose |
|
|
Term
| Bilateral, fixed, and dilated pupils indicate what kind of injury? |
|
Definition
| Most likely dead or close to death |
|
|
Term
| What effect does hypothermia have on the pupils? |
|
Definition
|
|
Term
| How do active seizures affect pupils? |
|
Definition
|
|
Term
| What drugs can make pupils non-reactive? |
|
Definition
| Barbiturates, succinylcholine, lidocaine, phenothiazines, methanol, aminoglycocydes |
|
|
Term
| What causes "blown" pupils? |
|
Definition
| Increased pressure causes the brainstem to herniate and cause a CN III lesion, patient is comatose |
|
|
Term
| Roving eye movements with eyes closed indicates what? |
|
Definition
|
|
Term
| How do eyes tend to move during seizures? |
|
Definition
|
|
Term
| How do eyes tend to move during a hemispheric stroke? |
|
Definition
|
|
Term
| How do eyes tend to move during a brainstem stroke? |
|
Definition
|
|
Term
| If there is a severe ocular motility defect with normal pupils, what would you suspect? |
|
Definition
| Benzos, barbituates, or alcohol |
|
|
Term
| What is the oculocephalic reflex? |
|
Definition
"Doll's eyes"
Turning the head causes eyes to go in the opposite direction |
|
|
Term
| What drugs inhibit the oculovestibular reflex? |
|
Definition
Gentamycin
Dilantin
TCA's
Succinylcholine |
|
|
Term
| What is the oculovestibular reflex? |
|
Definition
Water is placed in the ear canal
Normal response is nystagmus with the fast component away
If eyes deviate towards the water, problem is TME and brainstem is intact
Vertical gaze disturbances = brainstem disease
Ocular bobbing = pons defect |
|
|
Term
| What is Bell's phenomenon |
|
Definition
| The eye will roll upwards when touched during the corneal reflex |
|
|
Term
| A unilateral lack of the corneal reflex implies what? |
|
Definition
| A focal lesion, either cortical or brainstem |
|
|
Term
| What is a decorticate motor response? |
|
Definition
| Arms flexed, or bent inward on the chest, the hands are clenched into fists, and the legs extended and feet turned inward |
|
|
Term
| What is a decerebrate motor response? |
|
Definition
Head is arched back, arms are extended by sides, legs extended
Hallmark is extended elbows
The arms and legs are extended and rotated internally
Patient is rigid with the teeth clenched
Signs can be on just one side of the body or on both sides
May be just in the arms and may be intermittent |
|
|
Term
| Cheyne-Stoke respiration indicates what type of lesion? |
|
Definition
|
|
Term
| Deep rapid hyperventilating suggests what type of lesion? |
|
Definition
|
|
Term
| Apneustic breathing suggests what type of lesion? |
|
Definition
|
|
Term
| Cluster breathing suggests what type of lesion? |
|
Definition
|
|
Term
| Ataxic breathing suggests what type of lesion? |
|
Definition
|
|
Term
| Loss of pain and temperature on one side of face and other side of body suggests what type of lesion? |
|
Definition
|
|
Term
| Loss of pain and temp on one side of the body with weakness and/or vibration on the opposite side suggests what type of lesion? |
|
Definition
|
|
Term
| Pure motor symptoms suggests what type of lesion? |
|
Definition
| Contralateral small vessel lacunar infarct in the internal capsule, pons, or basal ganglia |
|
|
Term
| Pure sensory symptoms suggests what type of lesion? |
|
Definition
| Contralateral small vessel lacunar infact, most often in the thalamus |
|
|
Term
| Homonymous field cuts indicate the lesion is on which side? |
|
Definition
|
|
Term
| What CNS injury must be ruled out in cases of diplopia? |
|
Definition
Brainstem lesion
A neurologic issue will go away when one eye is covered, an opthalogic issue will persist with diplopia |
|
|
Term
| What CNS injury must be ruled out in cases of ataxia and dysphagia? |
|
Definition
|
|
Term
| Hypotonia/hyporeflexia in the first 24-48 hours before the appearance of other symptoms suggests what type of lesion? |
|
Definition
| Upper motor neuron lesion |
|
|
Term
| Loss of pain and temperature on one side of face and other side of body suggests what type of lesion? |
|
Definition
|
|
Term
| Loss of pain and temp on one side of the body with weakness and/or vibration on the opposite side suggests what type of lesion? |
|
Definition
|
|
Term
| Pure motor symptoms suggests what type of lesion? |
|
Definition
| Contralateral small vessel lacunar infarct in the internal capsule, pons, or basal ganglia |
|
|
Term
| Pure sensory symptoms suggests what type of lesion? |
|
Definition
| Contralateral small vessel lacunar infact, most often in the thalamus |
|
|
Term
| Homonymous field cuts indicate the lesion is on which side? |
|
Definition
|
|
Term
| What CNS injury must be ruled out in cases of diplopia? |
|
Definition
Brainstem lesion
A neurologic issue will go away when one eye is covered, an opthalogic issue will persist with diplopia |
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|
Term
| What CNS injury must be ruled out in cases of ataxia and dysphagia? |
|
Definition
|
|
Term
| Hypotonia/hyporeflexia in the first 24-48 hours before the appearance of other symptoms suggests what type of lesion? |
|
Definition
| Upper motor neuron lesion |
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|
Term
| What are the hyperkinetic movement disorders? |
|
Definition
Tremor
Dystonia
Chorea
Myoclonus
Ballismus
Tics
Tardive syndromes |
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|
Term
| What are the hypokinetic movement disorders? |
|
Definition
A disease of slowness but still presents w/ tremors
Heredogenerative diseases: Wilson's and Huntington's
Parkinsonism |
|
|
Term
|
Definition
| Rythmic oscillation of agonist and antagonist muscles |
|
|
Term
| What are the qualities of an action tremor? |
|
Definition
Depends on posture, worse when arms are out, resisting gravity
Task specific (worsens when writing)
Increases towards target |
|
|
Term
| What is a physiologic tremor? |
|
Definition
Mild tremor that is normally present, especially when taking stimulates, under anxiety, withdrawal, or metabolic issues
8-12Hz |
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|
Term
| What common drugs cause tremor? |
|
Definition
All antidepressants
Lithium/psych meds
Caffeine
Thyroid hormone replacement
ADD meds such as amphetamines
Corticosteroids |
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|
Term
| What is an essential tremor? |
|
Definition
Tremor when writing or a vocal tremor (such as with an "E" sound). Improves with alcohol, most commonly genetic
4-12Hz |
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|
Term
| What is the most common movement disorder? |
|
Definition
|
|
Term
| How are essential tremors treated? |
|
Definition
Only use treatment if needed or intolerable
Beta-blockers
Anticonvulsants (Primidone)
Benzodiazepines (Clonazepam)
Botulinum toxin |
|
|
Term
| What is a cerebellar tremor? |
|
Definition
Gross/big tremors, usually secondary to big stroke, rarely gets better, doesn’t respond to meds
Always get MRI if possible
<5Hz |
|
|
Term
| What are potential causes of cerebellar tumors? |
|
Definition
Etoh/Drugs
MS
Genetic Chiari malformation
Mass/Infectious
Ischemia |
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|
Term
|
Definition
Involuntary movements that result in twisting, abnormal postures, and repetitive movements of a body part. Movements are sustained
May be exacerbated by action
Often painful |
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|
Term
| What is the "sensory trick" for dystonia? |
|
Definition
| Putting something the mouth such as candy/mints will stop the tremor |
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|
Term
| What drugs can induce dystonia? |
|
Definition
Parkinson drugs
Anti-emetics
Anti-psychotics |
|
|
Term
|
Definition
Muscle relaxants
Trihexyphenidyl (Artane)
Botulinum toxin
Surgery
Deep brain stimulation |
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|
Term
|
Definition
Sudden, brief, shock-like involuntary movements caused by muscle contractions (positive) or inhibitions (negative)
Seen in alcoholism and renal failure |
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|
Term
| What are the different types of physiologic myoclonus? |
|
Definition
Sleep jerks
Anxiety induced
Exercise induced
Hiccups (singultus) |
|
|
Term
| What are the different types of essential myoclonus? |
|
Definition
Hereditary (atosomal dominant)
Sporadic |
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|
Term
| What is the other type of myocolonus aside from physiologic, essential, and symptomatic myocolnus? |
|
Definition
|
|
Term
| What are the different causes of symptomatic myoclonus? |
|
Definition
Storage diseases
Spinocerebellar degeneration
Basal ganglia degeneration
Dementia
Infectious or post-infectious syndrome
Metabolic disorders |
|
|
Term
| What are the characteristics of myoclonus caused by cortical problems? |
|
Definition
Is multi-focal
Must rule out epilepsia partialis continua status epilepticus of partial seizures
Usually due to metabolic or degenerative disorders |
|
|
Term
| What are the characteristics of myoclonus caused by brainstem problems? |
|
Definition
Presents facial and axial due to sensory stimuli
Hyperekplexia |
|
|
Term
| What type of myoclonus suggests propriospinal pathology? |
|
Definition
|
|
Term
|
Definition
|
|
Term
| What type of drugs induce myoclonus? |
|
Definition
Levodopa
Anticonvulsants
TCAs |
|
|
Term
|
Definition
Continuous, unsustained, non-stereotyped movements of variably changing speed and direction that seemingly flow from one muscle group to another, thus giving the appearance of "dancing"
Most often due to meds or Huntington's |
|
|
Term
| What type of CNS lesions can cause chorea? |
|
Definition
| Lesions of the basal ganglia at the caudate nucleus |
|
|
Term
| What metabolic conditions are potential causes of chorea? |
|
Definition
Wilson's disease
Hyperthyroidism
Hyperglycemia
Hypoglycemia
Electrolyte |
|
|
Term
| What systemic disorders are potential causes of chorea? |
|
Definition
Sydenham's chorea
SLE
Polycythemia |
|
|
Term
| What drugs can cause chorea? |
|
Definition
Levodopa
DA agonists
Anti-psychotics
Anti-emetics
Anti-cholinergics
Anti-epileptics |
|
|
Term
|
Definition
Brief, intermittent, repetitive, non-rhythmic, unpredictable, purposeless, stereotyped movements (motor tics) or sounds (phonic or vocal tics)
Associated with an urge, anxiety follows if suppressed |
|
|
Term
| What are primary tic disorders? |
|
Definition
|
|
Term
| What are secondary inherited tic disorders? |
|
Definition
HD
WIlson's
Neurocanthocytosis |
|
|
Term
| What type of infections can cause tics? |
|
Definition
Encephalitis
Prions
Sydenham's |
|
|
Term
| What defines a transient tic disorder? |
|
Definition
| Lasts between 4wks and a year |
|
|
Term
| What defines a chronic tic disorder? |
|
Definition
| Motor or phonic tic disorders lasting more than a year |
|
|
Term
| What are the characteristics of tourette's? |
|
Definition
Multiple motor and at least one phonic tic
Onset prior to age 21
Tics distress patient
Often overlap with OCD and ADD
No known cause |
|
|
Term
| What drugs can induce tics? |
|
Definition
Levodopa
DA agonists
ADPs
Carbamazepine |
|
|
Term
| What is first line in the treatment of tics? |
|
Definition
Clonidine
Guanfacine
Baclofen
Clonazapa |
|
|
Term
| What is tardive syndrome? |
|
Definition
| Abnormal involuntary movements caused by chronic exposure to dopamine receptor blocking drugs within 6 months of the onset of the symptoms persisting for one month after stopping the offending agent |
|
|
Term
| Which types of tardive syndrome are most common? |
|
Definition
Orofacial bucolingual movements
Most often caused by neuroleptics or anti-emetics |
|
|
Term
| How is tardive syndrome treated? |
|
Definition
| Remove offending agent, supplement with benzo's, reserpine, low dose neuroleptics. Is very tough to treat |
|
|
Term
| What are hypokinetic movement disorders? |
|
Definition
Idiopathic Parkinson's (responds to treatment)
Secondary parkinsonism
Parkinson's plus
Can also be due to Huntington's and Wilson's |
|
|
Term
| What can cause Parkinson's plus? |
|
Definition
Progressive supranuclear palsy
Corticobasal degeneration
Multiple System Atrophy
Dementia (Lewy Body) |
|
|
Term
| What are the cardinal symptoms of idiopathic parkinson's disease? |
|
Definition
Resting tremor
Bradykinesia
Postural instability
Rigidity |
|
|
Term
| What are the associated symptoms of idiopathic parkinson's? |
|
Definition
Hypophonia
Gait festination (can't stop)
Sialorrhea
Seborrhea
Micrographia, decreased blink rate, masked facies |
|
|
Term
| What can be given as neuroprotection to idiopathic parkinson's? |
|
Definition
|
|
Term
| What can be given for tremors in idiopathic parkinsons? |
|
Definition
Amantadine
Antichonilergics - Trihexyphenidyl or Benztropine |
|
|
Term
| What can be given for restless leg syndrom in idiopathic parkinsons |
|
Definition
| Dopamine Agonists: Pramipexole or Ropinirole |
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|
Term
| How do COMT-Is help treat symptomatic parkinson's? |
|
Definition
| Given with levodopa, helps it stay longer |
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|
Term
| What should be taken into account before prescribing levodopa for symptomatic parkinson's? |
|
Definition
| Brain develops resistance, using later in life is preferred |
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|
Term
| What are the characteristics of normal pressure hydrocephalus induced secondary parkinsonism? |
|
Definition
Very treatable
Associated with urinary incontinence
Shows "magnetic gait" where feet can't be lifted off the ground
Presents with dementia |
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|
Term
| What are some causes of secondary parkinsonism? |
|
Definition
Normal pressure hydrocephalus
Drug induced (anti-psychotics, anti-emetics)
Vascular strokes
Infections, trauma, toxins |
|
|
Term
| What kind of toxins can cause secondary parkinsonism? |
|
Definition
MPTP (designer Demerol)
CO
Manganese
Cyanide
Methanol
Post-encephalitic ("sleeping sickness") |
|
|
Term
| What kind of patients present with manganese induced secondary parkinsonism? |
|
Definition
|
|
Term
| What are some causes of parkinsonism plus syndrome? |
|
Definition
Progressive supranuclear palsy
Corticobasal degeneration
Multiple system atrophy
Dementia |
|
|
Term
| What are the characteristics of progressive supranuclear palsy induced parkinsonism plus syndrome? |
|
Definition
Early onset gait instability
Supranuclear gait palsy, loses inferior gaze first
Axial rigidity (Parkinson's is appendicular)
Total lack of tremor
Midbrain atrophy
Little response to meds |
|
|
Term
| What are the characteristics of progressive corticobasal degeneration induced parkinsonism plus syndrome? |
|
Definition
Unilateral akinetic-rigid syndrome
Apraxia causes "alien limb" (can't control it)
Sensory findings
Startle myoclonus
Dystonia
Tremor |
|
|
Term
| What kinds of multiple system atrophy are associated with parkinsonism plus syndrome? |
|
Definition
Shy-drager
Olivopontocerebellar atrophy
Striatonigral degeneration |
|
|
Term
| What is characteristic of Shy-Drager induced parkinsonism plus syndrome? |
|
Definition
Parkinson's features
Erectile dysfunction
Loss of bladder control
Orthostatic hypotension, causes syncope |
|
|
Term
| What is characteristic of dementia induced parkinsonism plus syndrome? |
|
Definition
Caused by diffuse Lewy Body dementia
Parkisonism
Subcortical demential (long term memory intact but hallucinations present)
May also be due to Alzheimer's, frontotemporal dementia, or NPH |
|
|
Term
| What causes Wilson's disease? |
|
Definition
| Autoimmune mutation on chromosome 13 causes a copper metabolism defect |
|
|
Term
| What are the symptoms of Wilson's disease? |
|
Definition
"Wing beating" tremor
Dysarthria, dystonia, Parkinsonism
Affects the proximal musculature |
|
|
Term
| What causes Huntington's disease? |
|
Definition
| Autosomal dominant mutation on chromosome 4 |
|
|
Term
| What are the symptoms of Huntington's disease? |
|
Definition
Depression
Chorea
Dementia
Marked caudate atrophy |
|
|
Term
| What is significant about the Westphal variant of Huntington's disease? |
|
Definition
Younger onset
Akinetic-rigid seizures and dementia more closely resembles Parkinson's |
|
|
Term
| Pilocytic astrocytoma most commonly effects what demographic? |
|
Definition
| Children, often associated with NF |
|
|
Term
| How does Pilocytic astrocytoma clinically present? |
|
Definition
| Cerebellar findings or symptoms secondary to hydrocephalus |
|
|
Term
| Where are pilocytic astrocytomas located? |
|
Definition
| Most often in the cerebellum |
|
|
Term
| How are Pilocytic astrocytomas detected? |
|
Definition
| MRI shows cyst with an enhancing mural nodule (enhancing ring) most often in the cerebellum |
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|
Term
| Pilocytic astrocytomas are composed of what type of tissue? |
|
Definition
|
|
Term
| How is Pilocytic astrocytoma treated? |
|
Definition
Surgically curable, can use chemo in children
90% 10yr survival with resection |
|
|
Term
| Subependymal giant-cell astrocytomas are associated with what other condition? |
|
Definition
|
|
Term
| Subependymal giant-cell astrocytomas presents with what clinical features? |
|
Definition
| Asymptomatic until it begins to obstruct the foramen of Monro which causes hydrocephalus |
|
|
Term
| Where are subependymal giant-cell astrocytomas located? |
|
Definition
| The wall of the lateral ventricle, seen as an intraventricular enhancing mass on imaging studies |
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|
Term
| Subependymal giant-cell astrocytomas are composed of what type of cells? |
|
Definition
|
|
Term
| How are subependymal giant-cell astrocytomas treated? |
|
Definition
Remains untreated until it causes obstruction
Surgical debulking, difficult to get to due to location
Usually slow growing and benign |
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|
Term
| Pleomorphic xanthoastrocytomas tend to present at what age? |
|
Definition
|
|
Term
| What is the characteristic clinical feature of pleomorphic xanthoastrocytomas? |
|
Definition
|
|
Term
| Where are pleomorphic xanthoastrocytomas located? |
|
Definition
| Superficial temporal lobes |
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|
Term
| How are pleomorphic xanthoastrocytomas detected? |
|
Definition
| Radiology shows a superficial meningo-cerebral nodule with a cyst at the end of the superficial temporal lobes near the meninges |
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|
Term
| How are pleomorphic xanthoastrocytomas treated? |
|
Definition
| Surgical resection provides a good prognosis |
|
|
Term
| What are low grade astrocytomas? |
|
Definition
| Low density, non-enhancing tumors composed of hypercellular, well defined astrocytes |
|
|
Term
| Where do low grade astrocytomas form? |
|
Definition
Supratentorial region in adults
Infratentorial region in children |
|
|
Term
| Low grade astrocytomas produce what symptoms? |
|
Definition
| Often asymptomatic but they can cause seizures |
|
|
Term
| How are low grade astrocytomas treated? |
|
Definition
| Often left untreated, surgical resection or radiotherapy are possible treatment options |
|
|
Term
| Anaplastic astrocytomas most often appear at what age? |
|
Definition
|
|
Term
| Where do anaplastic astrocytomas form? |
|
Definition
Supratentorial region in adults
Infratentorial region in children |
|
|
Term
| What causes anaplastic astrocytomas? |
|
Definition
| Caused by nuclear atypia without necrosis |
|
|
Term
| What are the clinical features of glioblastoma multiform? |
|
Definition
Focal deficits
Seizures
Headaches |
|
|
Term
| Where are glioblastoma multiform tumors located? |
|
Definition
| Frontal and temporal bone |
|
|
Term
| How are glioblastoma multiform tumors detected? |
|
Definition
| Radiology shows a ring-like enhancement with central necrosis and vasogenic edema found in the frontal and temporal bones |
|
|
Term
| What causes glioblastoma multiform tumors? |
|
Definition
|
|
Term
| How are glioblastoma multiform tumors treated? |
|
Definition
Surgical resection, radiotherapy, chemotherapy
Prognosis is poor, mean survival is 6mo |
|
|
Term
| Oligodendrogliomas most often appear at what age? |
|
Definition
|
|
Term
| What clinical features present with oligodendrogliomas? |
|
Definition
|
|
Term
| Where are oligodendrogliomas located? |
|
Definition
| Frontal or temporal region |
|
|
Term
| How are oligodendrogliomas detected with radiology? |
|
Definition
| Presence of calcification is visible in the frontal or temporal region |
|
|
Term
| Oligodendrogliomas are composed of what type of cells? |
|
Definition
|
|
Term
| How are oligodendrogliomas treated? |
|
Definition
Surgical resection and chemotherapy
Median survival is 5yrs |
|
|
Term
| Ependymomas appear among what age group? |
|
Definition
|
|
Term
| Ependymomas are often secondary to what other condition? |
|
Definition
| Obstructed CSF flow causing hydrocephalus and brainstem/cerebellar compression |
|
|
Term
| Where are ependymomas located? |
|
Definition
| Most often in the 4th ventricle or the spinal cord |
|
|
Term
| How are ependymomas detected with imaging? |
|
Definition
| Seen intraventricularly within the 4th ventricle with enhancement or intradural and extramedullar within the spinal cord |
|
|
Term
| How are ependymomas treated? |
|
Definition
Surgery followed by radiotherapy
High 5yr survival rate |
|
|
Term
| Gliomatosis cerebri presents clinically alongside what symptoms? |
|
Definition
Papilledema
Altered mentation
Headaches |
|
|
Term
| Where do gliomatosis cerebri form? |
|
Definition
| Deep thalamus and basal ganglia, will infiltrate deep structures, not finite |
|
|
Term
| How is gliomatosis cerebri detected on imaging studies? |
|
Definition
| Visible grey/white junction around the deep thalamus and basal ganglia with homogeneous hypodensities |
|
|
Term
| Gliomatosis cerebri is formed by what type of cells? |
|
Definition
|
|
Term
| How is gliomatosis cerebri treated? |
|
Definition
| No treatment, prognosis is poor, months to years |
|
|
Term
| Gliomatosis cerebri tend to form in what age group? |
|
Definition
| Peak incidence in the 1st and 2nd decades of life |
|
|
Term
| When do medulloblastomas usually present in life? |
|
Definition
| By the first decade of life |
|
|
Term
| What are the most common pediatric posterior fossa tumors? |
|
Definition
|
|
Term
| Medulloblastoma tumors appear in what location? |
|
Definition
Midline cerebellum with "drop mets"
Found within the SC, drops down the spinal cord |
|
|
Term
| How are medulloblastomas detected on imaging studies? |
|
Definition
|
|
Term
| Medulloblastomas are composed of what type of tissue? |
|
Definition
|
|
Term
| Retinoblastomas appear in what demographic? |
|
Definition
Children younger than 3
Autosomal dominant, 40% are bilateral |
|
|
Term
| What are the clinical features of retinoblastomas? |
|
Definition
| Leukocoria (red painful eye) and glaucoma |
|
|
Term
| Where are retinoblastoma tumors located? |
|
Definition
In the orbits, can present with pineal tumors as well
Found in bone marrow mets |
|
|
Term
| How are retinoblastomas treated? |
|
Definition
| Surgery, may cause vision loss but patient survives |
|
|
Term
| Neuroblastomas tend to affect what demographic? |
|
Definition
| Majority occur before the age of 5 |
|
|
Term
| What are the clinical features that present with neuroblastomas? |
|
Definition
Opsoclonus-myoclonus encephalopathy
Irregular occular movements |
|
|
Term
| Where are neuroblastomas found? |
|
Definition
|
|
Term
| How are neuroblastomas detected with imaging? |
|
Definition
| Contrast shows large enhancing calcified lesions |
|
|
Term
| How are neuroblastomas treated? |
|
Definition
Surgical resection and radiotherapy
Frequently reoccur |
|
|
Term
| Where do neurofibroma tumors occur? |
|
Definition
Most often within dorsal spinal roots
Rarely within cranial nerve roots |
|
|
Term
| How are neurofibromas treated? |
|
Definition
Surgical decompression
Repetitive lesions are common |
|
|
Term
| Meningiomas are found among what demographics? |
|
Definition
Between 20-60yo
More common in females |
|
|
Term
| What are the clinical features that present with meningiomas? |
|
Definition
Headaches and seizures
Growth is slow, can be asymptomatic or brain may adapt to its growth, causing simple partial seizures to manifest |
|
|
Term
| Where are meningiomas located? |
|
Definition
Extra-axial brain (meninges)
Spinal-thoracic region (meninges) |
|
|
Term
| How are meningiomas detected using radiology? |
|
Definition
| Homogenous enhancement shows a dural tail sign |
|
|
Term
| Meningiomas are composed of what type of tissue? |
|
Definition
|
|
Term
| How are meningiomas treated? |
|
Definition
Surgical resection, about 5% are malignant
Prognosis is good |
|
|
Term
| Gangliocytomas cause what symptoms? |
|
Definition
|
|
Term
| Gangliocytomas are found in what part of the brain? |
|
Definition
| Temporal and frontal lobes |
|
|
Term
| How are gangliocytomas treated? |
|
Definition
|
|
Term
| How are gangliocytomas detected? |
|
Definition
| Cystic nodule calcification is seen with imaging, usually diagnosed with a biopsy |
|
|
Term
| Pituitary adenomas present with what clinical features? |
|
Definition
| Visual and hormonal disturbances |
|
|
Term
| Pituitary adenomas are found in what part of the brain? |
|
Definition
| At the sella turcica, are fairly common |
|
|
Term
| Pituitary adenomas cause excess production of what hormones? |
|
Definition
Primarily prolactin
GH and ACTH as well |
|
|
Term
| How are pituitary adenomas treated? |
|
Definition
| Surgery or bromocriptine (dopamine agonists) |
|
|
Term
| Dermoid cysts tend to appear among what demographic? |
|
Definition
|
|
Term
| Where are dermoid cysts commonly located within the brain? |
|
Definition
Midline or temporal
Temporal placement can cause seizures
Hydrocephalus is common |
|
|
Term
| How are dermoid cysts detected? |
|
Definition
| MRI shows heterogenous hair and sebaceous material |
|
|
Term
| Arachnoid cysts cause what symptoms? |
|
Definition
Usually asymptomatic, are congenital and usually normal
Usually found incidentally and left untreated |
|
|
Term
| Hemangioblastomas tend to appear among what demographics? |
|
Definition
30-65yo in people with von Hippel-Lindau disease
The most common posterior fossa in adults (?) |
|
|
Term
| Where are hemangioblastomas located? |
|
Definition
| Most often in the cerebellum |
|
|
Term
| Primary CNS lymphoma affects patients with what condition? |
|
Definition
|
|
Term
| What are the clinical features of primary CNS lymphoma? |
|
Definition
| Headache, seizures, mental status changes |
|
|
Term
| How are primary CNS lymphomas detected? |
|
Definition
Imaging shows dense uniform ring enhancing tumor
Must be screened for metastasis |
|
|
Term
| Primary CNS lymphomas are composed of what cells? |
|
Definition
|
|
Term
| How are primary CNS lymphomas treated? |
|
Definition
Steroids and radiotherapy
Prognosis is worse in HIV pts |
|
|
Term
| How common is metastasis to the brain? |
|
Definition
| 50% of all brain tumors are metastases |
|
|
Term
| Tumor metastasis to the brain is most common among what age group? |
|
Definition
|
|
Term
| Leukemia is most common among what demographic? |
|
Definition
|
|
Term
| What must you do if you find multiple ring enhancing lesions in the brain? |
|
Definition
| Locate the primary tumor, often MCC in the lung |
|
|
Term
| Cancer usually metastasizes to the brain from what locations? |
|
Definition
| Lung > breast > melanoma > colorectal > renal cell |
|
|
Term
| What type of cancer never metastasizes to the brain? |
|
Definition
|
|
Term
| Metastases to the skull or meninges is usually from what location? |
|
Definition
| Breast > prostate* > multiple myeloma > lymphoma > leukemia |
|
|
Term
| What are the characteristics of vasogenic brain edema? |
|
Definition
Most commonly seen with brain tumors*
Affects the white matter |
|
|
Term
| How is vasogenic brain edema treated? |
|
Definition
|
|
Term
| What are the characteristics of cytotoxic brain edema? |
|
Definition
Occurs secondary to hypoxia and ischemic stroke
Grey and white matter are affected
Steroids are not beneficial |
|
|
Term
| What are the characteristics of interstitial brain edema? |
|
Definition
Caused by obstruction in CSF flow
Affects the periventricular white matter |
|
|
Term
| How is interstitial brain edema treated? |
|
Definition
Shunting if needed
Dextromethosone (or other steroids), SE limit use |
|
|
Term
| What is the worse type of brain herniation? |
|
Definition
|
|
Term
| Uncal herniations present with what clinical features? |
|
Definition
Midbrain is compressed by the uncus, pupils are dilated
Fatal |
|
|
Term
| A cingulate herniation can cause what other problems? |
|
Definition
| Opposite anterior cerebral artery may stroke, causes lower extremity weakness |
|
|
Term
| Antoni A & B patterns are characteristic of what? |
|
Definition
|
|
Term
| Lafora bodies are associated with what? |
|
Definition
|
|
Term
| What is the most common reason people see a neurologist? |
|
Definition
Headaches
Also Alzheimer's, tremors, and seizures |
|
|
Term
| What are the red flags of a headache? |
|
Definition
Sudden onset, worsening pattern, papilledema
Association with systemic illness
Focal neurologic signs suggest tumor
Valsalva trigger indicates brain tumor
New headache in pt with lyme dz, cancer, or HIV |
|
|
Term
| What should be given extra attention when doing a physical exam on a patient complaining of a headache? |
|
Definition
Nuchal signs
Papilledema
Focal signs
Visual fields
Tender points
Autonomic signs |
|
|
Term
| Optic neurotic precedes what other condition? |
|
Definition
|
|
Term
| What is the most common cranial neuralgia that causes headaches? |
|
Definition
| Trigeminal and occipital neuralgias |
|
|
Term
| The majority of migraines present in what fashion? |
|
Definition
Without aura (85%)
Unilateral (65%)
4-72hrs
Photophobia/phonophobia
Aggravated by activity |
|
|
Term
| What is a transformed/chronic migraine? |
|
Definition
| Migraine becomes more and more frequent and are usually combined with tension migraines |
|
|
Term
| What are the complicated migraines? |
|
Definition
Basilar (stroke-like symptoms, vertigo)
Confusional
Migraine induced seizues |
|
|
Term
| What is status migrainosis? |
|
Definition
|
|
Term
| New onset of a headache after 50yo suggests what? |
|
Definition
|
|
Term
| Migraines are most common among what age group? |
|
Definition
| 22-45yo but can be before 10yo |
|
|
Term
| What are the phases of migraine attack? |
|
Definition
Prodrome
Aura
Headache
Postdrome |
|
|
Term
| What characterizes the prodrome phase of a migraine attack? |
|
Definition
| Precedes attack by a couple days with numbness, tingling, appetite change, mood changes |
|
|
Term
| What characterizes the aura phase of a migraine attack? |
|
Definition
Visual**, tunnel, scintillating scotoma
Olfactory and sensory changes, motor weakness
Language changes, delusions in severe cases |
|
|
Term
| What characterizes the postdrome phase of a migraine attack? |
|
Definition
Feels like a hangover
Fatigued and irritable |
|
|
Term
| What is the purpose of abortive treatment for migraines? |
|
Definition
| Taken as needed during a migraine to stop it once it's already started |
|
|
Term
| What drugs can be used for abortive treatment for migraines? |
|
Definition
Triptans like sumatriptan
OTC NSAIDs (allows control without consultation) |
|
|
Term
| What drugs can be used as prophylaxis to prevent migraines? |
|
Definition
Anti-hypertensives
Anti-depressants, TCAs, SSRIs
Anti-seizure topiramate, valproic acid, pregabalin, gabapentin
Muscle relaxants (for tension headaches)
OMM and physical therapy |
|
|
Term
| What is the most common type of headache? |
|
Definition
|
|
Term
| What are the characteristics of tension headaches? |
|
Definition
Usually associated with stres
Lasts from 30 min to days
Evolves from episodic to chronic
Bilateral and mild to moderate
Squeezing pressure, band/vice-like |
|
|
Term
| Tension headaches are often associated with that type of neuralgia? |
|
Definition
Occipital neuralgia
Rarely associated with photophobia or phonophobia
Usually not associated with N/V |
|
|
Term
| What psychophysiological treatments are there for tension headaches? |
|
Definition
Counseling
Stress management
Relaxation therapy
Biofeedback |
|
|
Term
| What are cluster headaches? |
|
Definition
| Severe unilateral headache lasting 15 minutes to 3 hours (shorter than migraine) but meds usually don’t start to work in time |
|
|
Term
| How do cluster headaches present? |
|
Definition
The most painful neurological condition
Patient will pace, bang head on wall, consider suicide
Many women say its more painful than childbirth |
|
|
Term
| Cluster headaches present with what associated symptoms? |
|
Definition
Conjunctival injection
Lacrimation
Nasal congestion
Rhinorrhea
Forehead and facial sweating
Miosis and Ptosis
Horner's on one side |
|
|
Term
| What abortive treatments exist for cluster headaches? |
|
Definition
7-10L of O2 for 30 min
DHE
Triptans
Intranasal lidocaine
NSAIDs
Corticosteroids |
|
|
Term
| What prophylactic treatments for cluster headaches work the best? |
|
Definition
Ca++ channel blockers
Topiramate |
|
|
Term
| What is chronic paroxysmal hemicrania? |
|
Definition
| Similar to cluster headache but shorter-lasting, more frequent, less painful, occur more commonly in females and respond absolutely to indomethacin (this can be used to diagnose) |
|
|
Term
| What is hemicrania continua? |
|
Definition
Persistent strictly unilateral headache responsive to indomethacin
Lasts >3mo, moderate intensity with moments of severity |
|
|
Term
| What are primary stabbing headaches? |
|
Definition
| Transient and localized stabs of pain in the head that occur spontaneously in the absence of organic disease of underlying structures or of the cranial nerves |
|
|
Term
| What is a hypnic headache (aka alarm clock headaches) |
|
Definition
| Headache that wakes the patient at the same time every night for 5-60min, bilateral pain, no autonomic features, occurs 15/month |
|
|
Term
| Hypnic headaches are most common among what demographic? |
|
Definition
|
|
Term
| How are hypnic headaches treated? |
|
Definition
Lithium
Caffeine
Anti-inflammatory drugs |
|
|
Term
| Benign exertional, cough, and thunderclap headaches are common among what demographic? What are their characteristics? |
|
Definition
Young males
Lasts 5min to 24hrs, provoked by physical exercise (lifting)
Must r/o aneurysm |
|
|
Term
| What is a type I sexual headache? |
|
Definition
| Dull headache in the head and neck as sexual excitement increases |
|
|
Term
| What is a type II sexual headache? |
|
Definition
| Severe explosive headache at the time of orgasm lasting 2 minutes to 48 hours |
|
|
Term
| What is a type III sexual headache? |
|
Definition
| Postural headache after coitus resembling a spinal headache |
|
|
Term
| How are sexual headaches treated? |
|
Definition
| Daily anti-inflammatory treatment or a few hours before sex |
|
|
Term
| What is a post concussion headache? |
|
Definition
Diffuse throbbing steady cephalgia 1-3 days post head injury that begins with vertigo followed by headache
Usually not chronic, remits in wks-months |
|
|
Term
| What are the symptoms of a post-concussion headache? |
|
Definition
Impaired attention, concentration, or memory
Irritability, emotional lability
Vertigo, dizziness, or nausea |
|
|
Term
| How are post-concussion headaches treated? |
|
Definition
Amitryptiline (helps them sleep too), Imipramine, Depakote, NSAIDS, topiramate
Pphysical therapy, Manipulation |
|
|
Term
| What are the characteristics of a epidural hematoma? |
|
Definition
Tearing of middle meningeal artery after significant head trauma
Presents acutely
Lucid interval
Neurosurgical decompression |
|
|
Term
| What are the characteristics of a subdural hematoma? |
|
Definition
Tearing of bridging veins often after minor trauma
Symptoms may occur weeks later and include focal neurological signs like headaches/seizures
Treatment is often surgical
More subtle than epidural hematoma |
|
|
Term
| Why are the elderly more prone to subdural hematomas? |
|
Definition
| The brain shrinks which stretches bridging veins |
|
|
Term
| What is a subarachnoid hemorrhage? |
|
Definition
Sudden onset of a severe headache “worst headache of my life” that reaches maximum intensity in one minute
Associated with stiff neck, photophobia, N/V leading to obtundation, paralysis, CN III paralysis with pupil changes, and com |
|
|
Term
| How is a subarachnoid hemorrhage diagnosed? Treatment? |
|
Definition
Lumbar tap for xanthochromia followed by CT if negative
Requires immediate neurosurgery |
|
|
Term
| What are the characteristic symptoms of a central venous thrombosis? |
|
Definition
| Triad of headache, seizures, MS changes/focal neurological signs |
|
|
Term
| A central venous thrombosis is most common in what demographic? |
|
Definition
| Pregnant or post-partum females, those on BCP's, or patients with hypercoaguable states |
|
|
Term
| How is a central venous thrombosis diagnosed? |
|
Definition
|
|
Term
| How is a central venous thrombosis treated? |
|
Definition
|
|
Term
| What is the most common symptom of an internal carotid artery dissection? |
|
Definition
|
|
Term
| Internal carotid artery dissections are associated with what other symptoms? |
|
Definition
Bruit, tinnitus,syncope, ipsilateral tongue paresis or Horner’s syndrome
CVA/TIA symptoms may follow |
|
|
Term
| Giant cell arteritis affects what size arteries? |
|
Definition
Large and medium arteries
Considered an emergency |
|
|
Term
| What are the symptoms of giant cell arteritis? |
|
Definition
Headache in 70-90% of patients
Fatigue, myalgias, depressed mood, jaw claudication on side of headache, diminished temporal pulse, scalp tender, and visual loss
Risk of stroke |
|
|
Term
| How is giant cell arteritis diagnosed? |
|
Definition
Temporal artery biopsy is gold standard
ESR/CRP is often but not always elevated |
|
|
Term
| How is giant cell arteritis treated? |
|
Definition
|
|
Term
| What is pituitary apoplexy? |
|
Definition
| Severe acute retro-orbital, frontal or diffuse headache accompanied by at least one of the following |
|
|
Term
| What are the usual symptoms of chronic myelogenous leukemia? |
|
Definition
Fatigue due to anemia
Abdominal discomfort due to enlarged spleen
Infections are not a common feature
Asymptomatic for years |
|
|
Term
| What kind of mutation causes chronic myelogenous leukemia? |
|
Definition
| Chromosome 9 and 22 translocation between ABL and BCR genes creates the BCR-ABL1 fusion gene which produces abnormal proteins causing neutrophil proliferatin |
|
|
Term
| Why are chromosome 9 and 22 specifically more susceptible to the translocation mutation which leads to CML? |
|
Definition
Lay in close proximity during interphase
Homologous sequences may facilitate aberrant recombination |
|
|
Term
| What are phases of chronic myelogenous leukemia? |
|
Definition
Chronic phase (asymptomatic, 4-6yrs, high WBCs)
Accelerated phase (potential blasts, splenomegally, failure to thrive, fever, sweats)
Blastic phase (acute leukemia, poor prognosis)
Try to catch it early |
|
|
Term
| What is the progression of the neutrophil cell lineage? |
|
Definition
| Blast -> promyeloblast -> myelocyte -> metamyelocyte -> band -> neutrophil |
|
|
Term
| What is used to treat chronic myelogenous leukemia? |
|
Definition
Imatinib is now the drug of choice
Previously, gleevec (tyrosine kinase inhibitor) was used to inhibit the abnormal protein, completely preventing overproduction of WBCs |
|
|
Term
| What is the danger of using Gleevac or other tyrosine kinase inhibitors to treat chronic myelogenous leukemia? |
|
Definition
| Poor compliance increases the chances of mutations |
|
|
Term
| What type of lab studies would suggest chronic myelogenous leukemia? |
|
Definition
| Increased WBCs with higher amounts of immature WBC lineages/neutrophils. Reverse transcriptase PCR for the BCR-ABL mutation confirms |
|
|
Term
| What is the most common lymphoma in young adults? |
|
Definition
Hodgkin's Disease
Seen in both young and very old |
|
|
Term
| What kind of prognosis can be expected with Hodgkin's disease? |
|
Definition
| Very high cure rate if detected in younger at an early stage. Elderly don't do as well with chemo |
|
|
Term
| Non-Hodgkin's disease refers to what? |
|
Definition
A heterogenous group of lymphoid malignancies
May manifest a very aggressive tumor growing within days-weeks or be benign and not require treatment |
|
|
Term
| What are the A symptoms of Non-Hodgkin's lymphoma? |
|
Definition
| Asymptomatic, patient presents with a persisting lump resistant to antibiotics |
|
|
Term
| What are the B symptoms of Non-Hodgkin's lymphoma? |
|
Definition
Fever
Sweats
>10% weight loss |
|
|
Term
| What factors are used to stage Non-Hodgkin's Lymphoma? |
|
Definition
Age (older is worse due to comorbidities and chemo)
LDH is a marker for cell turnover
Tumor type and staging
A or B symptoms |
|
|
Term
| What are risk factors in the development of Non-Hodgkin's lymphoma? |
|
Definition
Ebstein Barr Virus
HTLV-1
Impaired/altered immunity
Wiskott Aldrich syndrome
HIV
Organ or stem cell transplant
Autoimmune disease
Herbicides and pesticides |
|
|
Term
| Why do Non-Hodgkin's patients often report early satiety? |
|
Definition
| Splenomegaly impacts stomach distention |
|
|
Term
| What are the physical signs of Non-Hodgkin's lymphoma? |
|
Definition
Hepatosplenomegally
Axillary adenopathy |
|
|
Term
| What are the characteristics of indolent Non-Hodgkin's lymphoma? |
|
Definition
Usually widespread at diagnosis but controllable
No cure or treatment unless it obstructs structures |
|
|
Term
| What are the characteristics of intermediate Non-Hodgkin's lymphoma? |
|
Definition
Large B cells are present
Treated with CHOP/Rituxin
More localized by grows rapidly (weeks to months) |
|
|
Term
| What are the characteristics of high grade Non-Hodgkin's lymphoma? |
|
Definition
Also known as Burkitt's lymphoma
Caused by t(8:14) myc oncogene
Potentially curable with aggressive therapy |
|
|
Term
| What type of cells are unique to Hodgekin's lymphoma? |
|
Definition
|
|
Term
| What are the different categories of Hodgkin's disease? |
|
Definition
Lymphocyte predominant
Lymphocyte depleted
Nodular sclerosing
Mixed cellularity |
|
|
Term
| Which Hodgkin's disease type has the best prognosis? |
|
Definition
Lymphocyte predominant type
Treated with radiation or excision |
|
|
Term
| Which Hodgkin's disease type has the worst prognosis? |
|
Definition
|
|
Term
| Which Hodgkin's disease type is most commonly diagnosed? |
|
Definition
|
|
Term
| How is Hodkin's disease treated? |
|
Definition
Radiation therapy
ABVD-chemotherapy
Combining treatments lowers the doses of each therapy |
|
|
Term
| What is the most common cause of seizures in children 14yo and under? |
|
Definition
|
|
Term
| What is the most common cause of seizures in individuals 15-35yo? |
|
Definition
|
|
Term
| What is the most common cause of seizures in individuals 35yo+ |
|
Definition
| Cerebrovascular accident (stroke) |
|
|
Term
| What are the motor changes associated with a partial seizure? |
|
Definition
| Tonic/clonic, grimacing, aversive, posturing, Postictal Todd's paralysis (affected portion of the brain temporarily loses function while neurochemicals are replenished) |
|
|
Term
| What type of sensory changes are associated with partial seizures? |
|
Definition
Tingling, numbness
Vrtigo
Odd mental and emotional events
Hallucinations |
|
|
Term
| What is the difference between a simple and complex seizure? |
|
Definition
Simple = no loss of consciousness
Complex = loss of consciousness |
|
|
Term
| Simple partial seizures are usually caused by what pathology? |
|
Definition
| Slowly growing benign tumor on opposite side of affected body, may stimulate parkinson's disease |
|
|
Term
| What is Jacksonian march? What causes it? |
|
Definition
Tingling and jerking that may begin in one part of the body and spread to various parts of the body on the same side
Seen in simple partial seizures |
|
|
Term
| Seizures that induce autonomic changes are most often located in what part of the brain? |
|
Definition
|
|
Term
| What is the most common type of seizures in adults? |
|
Definition
|
|
Term
| What are the symptoms of simple partial seizures? |
|
Definition
| Convulsive jerking, tingling, scintillation, sweating, dilation of pupils, déjà vu, Jacksonian march, hallucinations, autonomic changes |
|
|
Term
| What symptoms are characteristic of complex partial seizures? |
|
Definition
| Aura of motionless stare automatisms (chewing, smacking, mimicry, tapping, etc.) followed by secondary general tonic/clonic seizures |
|
|
Term
| What are the different types of generalized seizures? |
|
Definition
Absence
Tonic
Clonic
Atonic
Myoclonic
Generalized Tonic/Clonic |
|
|
Term
| What are atypical absence seizures? |
|
Definition
| Often w/mental retardation and progresses to other seizure types, ECG is <2 or >4hz, more tone changes |
|
|
Term
| What typical absence seizures? |
|
Definition
Almost always before age 20, usually seen as staring episodes with simple automatisms, onset 3-23yo, can treat by forcing child to hyperventilate
Also known as "petit mal" |
|
|
Term
| Absence seizures are often accompanied by general tonic/clonic seizures. What can be used to treat both? |
|
Definition
|
|
Term
| What are the characteristics of general tonic/clonic seizures? |
|
Definition
Loss of consciousness
Tonic, clonic, and postictal phases
Sudden onset, gradual recovery
Epileptic cry, cyanosis, upward gaze, tongue-bite, salivation, sphincter incontinence
An aura indicates a partial onset
Common in non-REM sleep |
|
|
Term
| Benign Rolandic epilepsy (now called epilepsy with centrotemporal spikes) generally only occurs under what circumstances? |
|
Definition
While asleep
Disappears by adolescence |
|
|
Term
| What is juvenile myoclonic epilepsy? |
|
Definition
Onset 13-19yo, may develop into GTCS/Absence seizures
Quick myoclonic jerks (throwing things) due to sleep deprivation, alcohol, photosensitivity, etc.
Most require medication for life (valproic acid) |
|
|
Term
| What are the characteristics of Lennox-Gastaut syndrome? |
|
Definition
Seizure onset during childhood, pretty much all types of seizures, associated with mental retardation and status epilepticus
Poor prognosis |
|
|
Term
| What are febrile convulsions? |
|
Definition
Generalized T/C seizures associated with fevers, seen in children, usually only occurs once but can recur, increases risk of chronic epilepsy
Onset <13mo associated with prior neurological problems |
|
|
Term
| What are the characteristics of pseudoseizures? |
|
Definition
F:M = 4:1, most are 15-35yo
Seizures with no associated electrical discharge, occurs only w/witnesses, normal EEG, associated with psychosocial/emotional problems
Rosenthal sign fluttering, retaining of eyes, and recollection of full body seizures are giveaways |
|
|
Term
| A general T/C seizure requires a trip to the ER under what circumstances? |
|
Definition
Convulsion > 5 min.
Cluster of seizures
First seizure |
|
|
Term
| What is the MoA of phenytoin? |
|
Definition
Na+ channel blocker
Metabolized in the liver |
|
|
Term
| Phenytoin can be used for what seizure types? |
|
Definition
| Partial seizures, Generalized tonic-clonic, Status epilepticus |
|
|
Term
| Phenytoin should NEVER be used with which seizure type? |
|
Definition
| Absence seizures, may induce status epilepticus |
|
|
Term
| What are the side effects of phenytoin? |
|
Definition
Coursening of face, hirsutism, gingival hyperplasia
Nystagmus, ataxia, drowsiness, mental status changes
Osteopenia, Neuropathy, multiple drug interactions |
|
|
Term
| Carbamazepine can be used for what seizure types? |
|
Definition
|
|
Term
| What are the side effects of carbamazepine? |
|
Definition
Sedation
Blurred vision
Dizziness
Rashes
Hyponatremia* |
|
|
Term
| What is the MoA of valproic acid? |
|
Definition
| Blocks Na+ channels, Reduces Ca++ flow & Enhances GABA firing |
|
|
Term
| Valproic acid can be used to treat what seizure types? |
|
Definition
| ALLLLL OF THEEEEEM MUAUAHAHAHA |
|
|
Term
| What are the side effects of Valproic acid? |
|
Definition
N/V, tremor, hair loss
Significant weight gain
Liver toxicity in adults
Pancreatitis, thrombocytopenia, osteopenia
Neural tube defects in newborns
Multiple drug interactions (Warfarin) |
|
|
Term
| What is first line in the treatment of status epilepticus? |
|
Definition
|
|
Term
| Gabapentin is used for the treatment of what seizures types? |
|
Definition
|
|
Term
| What are the side effects of Gabapentin? |
|
Definition
Low extremity edema
Significant weight gain
Dizziness
Mental status changes |
|
|
Term
| Levetiracetam is used in the treatment of what seizure types? |
|
Definition
Partial epilepsy
Generalized seizures |
|
|
Term
| What are the side effects of Levitiracetam? |
|
Definition
Somnolence
Asthenia
Dizziness
May interact with oral contraceptives |
|
|
Term
| Topiramate can be used to treat what seizure types? |
|
Definition
Partial epilepsy
Generalized seizures |
|
|
Term
| What is the problem with using Topiramate in the treatment of partian and generalized seizures? |
|
Definition
| Must titrate slowly over 2 months |
|
|
Term
| What are the side effects of Topiramate? |
|
Definition
Parasthesias Finger tips & Toes
Psychomotor (slowing word finding difficulties)
Renal stones
Angle closure glaucoma
Weight loss
Carbonated beverages taste terrible |
|
|
Term
| Zonisamide is most similar to what other anti-epileptic? |
|
Definition
| Topiramate (generalized and partial seizures), avoid with sulfa allergies |
|
|
Term
| The anti-epileptic DoC for women looking to get pregnant is what? |
|
Definition
|
|
Term
| Lamotrigine can be used in the treatment of what seizure types? |
|
Definition
Partial epilepsy
Generalized seizures |
|
|
Term
| What are the side effects of Lamotrigine? |
|
Definition
Stevens-Johnson syndrome (if not well titrated)
Vertigo, dizziness, ataxia, meningitis |
|
|
Term
| What drug is similar to carbamezapine but with less side effects? |
|
Definition
| Oxcarbazepine (thought it does make hyponatremia worse) |
|
|
Term
| Any woman taking an anti-epileptic trying to get pregnant should supplement with what? |
|
Definition
|
|
