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PA neuro concerns T3
PA neuro concerns T3
119
Medical
Graduate
10/03/2018

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Term
Huntington’s Disease
Definition
• Aninheritedprogressiveneurodegenerativedisorder characterized by choreiform movements, psychiatric problems, and dementia.
• Itiscausedbyacytosine-adenine-guanine(CAG) trinucleotide repeat expansion in the huntingtin (HTT) gene on chromosome 4p and inherited in an autosomal-dominant pattern
• Symptoms:chorea,psychiatricproblemscaninclude irritability, depression, dysphoria, agitation, apathy, anxiety, paranoia, delusions, and hallucinations. With disease progression, motor function deteriorates
• Management:MRI,genetictesting,familyhistory; treatment is supportive. MRI shows enlarged ventricles and atrophy of cerebral nerve tissue and basal ganglia
Term
Muscular Dystrophy
Definition
• TheDuchenneandBeckermusculardystrophies(as well as a third intermediate form) are caused by mutations of the dystrophin gene and are therefore named dystrophinopathies
• Progressiveweaknessistheprincipalsymptomas muscle fiber degeneration is the primary pathologic process
• X-linkedrecessive;genetictesting
• CanpresentinadulthoodwithDMD-associateddilated cardiomyopathy with little or no muscle weakness
• Management:Glucocorticoidsmayimprovemotor function, strength, pulmonary function, reducing the risk of scoliosis, and possibly for delaying the onset of cardiomyopathy.
Term
Duchenne Muscular Dystrophy (DMD)
Definition
• Onset of weakness usually occurs between 2-3years of age
• Muscle weakness affects the proximal before the distal limb muscles.
• Cardiomyopathy and conduction abnormalities, bone fractures, and scoliosis.
• Serum creatine kinase (CK) concentrations are markedly elevated.
• Affected children frequently have varying degrees of mild cognitive impairment.
• Physical examination: pseudohypertrophy of the calf and (occasionally) quadriceps muscles, lumbar lordosis, a waddling gait, shortening of the Achilles tendons, and hyporeflexia or areflexia in weak muscles.
• Patients with DMD are often confined to a wheelchair by about age 12 to 13 years and die in their late teens or twenties from respiratory insufficiency or cardiomyopathy.
• Rarely, patients survive in their early thirties.
Term
Becker Muscular Dystrophy (BMD)
Definition
• The age of onset of symptoms of those with BMD is usually later and the degree of clinical involvement milder
• Patients with BMD typically remain ambulatory beyond the age of 16 years and often well into adult life, and usually survive beyond the age of 30 years
Term
Spina Bifida
Definition
• AKAMyelomeningocele
• Mostcommonneuraltubedefect
• Acleftinthevertebralcolumn, with a corresponding defect in the skin so that the meninges and spinal cord are exposed.
• Symptoms:mayhaveweakness and absence of sensation affecting the lower extremities and bowel/bladder dysfunction, depending upon the level of the spinal lesion.
• Prenatal counseling includes discussion of the natural history of myelomeningocele and the prenatal management decisions including termination of the pregnancy, pursuit of additional prenatal testing, choice of delivery setting, and, when applicable, the possibility of fetal surgery.
• The postnatal management choices are also discussed, including surgical closure of the defect and possible need for ventriculoperitoneal shunt placement, orthopedic management to correct the deformities and maximize ambulation
Term
Dementia
Definition
• Adisorderthatischaracterizedbyadeclineincognition involving one or more cognitive domains (learning and memory, language, executive function, complex attention, perceptual-motor, social cognition)
• Diagnosisbyhistoryandclinicalassessment:decline from a previous level of functioning, cognitive deficits must interfere with the independence in everyday activities, independent from delirium, the disturbances are not better accounted for by another mental disorder
• Alzheimerdisease(AD)isthemostcommonformof dementia in older adults, accounting for 60 to 80 percent of cases
• Most patients with dementia do not present with a complaint of memory loss; it is often a spouse or other informant who brings the problem to the clinician's attention
• Patients with dementia may have difficulty with one or more of the following: Retaining new information (trouble remembering events), Handling complex tasks (balancing a checkbook), Reasoning (unable to cope with unexpected events), Spatial ability and orientation (getting lost in familiar places), Language (word finding), Behavior
Term
Dementia Syndromes
Definition
• Alzheimerdisease(AD)
• DementiawithLewybodies(DLB)
• Frontotemporaldementia(FTD)
• Vascular(multi-infarct)dementia(VaD)
• Parkinsondiseasewithdementia(PDD)
• Lesscommondisorderssuchasprogressive supranuclear palsy (PSP) and Huntington disease can also be associated with dementia
Term
Dementia management and PE
Definition
• Management:Cognitivetesting-MMSE,MoCA,clinical dementia rating, Mini-Cog, clock drawing, neuropsych testing
• PhysicalExam:focusuponfocalneurologicdeficitsthat may be consistent with prior strokes, signs of Parkinson disease (cogwheel rigidity and/or tremors), gait abnormalities or slowing, and eye movements
• Labs:B12,CBC,TSH
• Management:noncontrastheadCT,MRI,Memantine plus cholinesterase inhibitors, vitamin E, Selegiline, cognitive rehab, nutrition as patients have a decreased smell and lose appetite, OT. Other unproven drugs: estrogen, antiinflammatory drugs, ginkgo biloba, statins, Vitamin B and omega 3 fatty acids
Term
Delirium
Definition
• Delirium is usually acute or subacute in onset and is associated with a clouding of the sensorium; patients have fluctuations in their level of consciousness and have difficulty maintaining attention and concentration
• Management: supportive care, reorientation, avoiding triggers such as restraints, utilization of orientation protocols, cognitive stimulation, visual and hearing aids, early mobilization, thiamine, Haldol, Benzos
Term
Parkinson’s Disease
Definition
• Aprogressiveneurodegenerativedisease
• Thecardinalfeaturesaretremor,bradykinesia,and rigidity. Postural instability occurs later
• OthermotorfeaturesofPDincludecraniofacial (masked facial expression, hypophonia), visual (hypometric saccades, eyelid opening apraxia), musculoskeletal (micrographia, stooped posture), and gait (shuffling, short-stepped gait, freezing) abnormalities
• Otherneuropsychiatricandnonmotormanifestations include: cognitive dysfunction, psychosis, mood disorders, sleep disturbances, fatigue, automatic dysfunction, olfactory dysfunction, pain and sensory disturbances, seborrhea dermatitis
• Management: MRI-but not diagnostic, Levadopa, Dopamine agonists, MAO B Inhibitors, Anticholinergic agents, Amantadine, COMT inhibitors,
Term
Alzheimer’s Disease
Definition
• A neurodegenerative disorder of uncertain cause and pathogenesis that primarily affects older adults and is the most common cause of dementia
• Symptoms: memory impairment, executive dysfunction and visuospatial impairment are often present relatively early, while deficits in language and behavioral symptoms often manifest later in the disease course
• Rate of progression varies
• Management: neuropsychological testing, MRI, PET, biomarkers-A protein deposition, tau deposition (key component of neurofibrillary tangles), genetic testing (not routine), histopathologic exam (rarely done), cognitive rehab, exercise programs, OT, cholinesterase inhibitor, vitamin E, memantine
• Drugs with unproven benefit: estrogen, anti-inflammatory drugs, statins, vitamin B and omega 3 fatty acids
Term
Tremor
Definition
• Tremor is defined as an involuntary, rhythmic, and oscillatory movement of a body part
• It is caused by either alternating or synchronous contractions of antagonistic muscles
• Classification of tremor is based on history, tremor characteristics, associated neurologic and systemic signs, and, in some cases, additional testing.
• A vast number of diseases, disorders, medications, toxins, and substances cause tremor
• Rest tremor occurs in a body part that is fully supported and not voluntarily activated.
• Parkinson disease (PD) and other parkinsonian syndromes are the most common causes of rest tremor.
• Action tremor occurs with voluntary muscle contraction; can be further characterized as kinetic, postural, and isometric
• Action tremors include physiologic, essential, primary writing, orthostatic, intention, cerebellar, rubral, neuropathic, and dystonic
• Functional tremor, also known as psychogenic tremor, is typically characterized by complex rest, postural, and kinetic tremor with abrupt onset, a static course, changeable features, functional disability out of proportion to tremor magnitude, and resistance to treatment. Any body part may be involved
• The routine laboratory evaluation of tremor should include tests of thyroid function, diagnostic studies to exclude Wilson disease, and screening for heavy metal poisoning such as mercury or arsenic if an environmental cause is suspected.
Term
Chorea
Definition
• A hyperkinetic movement disorder characterized by rapid and unpredictable contractions affecting mostly distal limbs, but also the face and trunk. The movements are involuntary and nonpatterned with variable speed, timing, and direction.
• Chorea, athetosis (slower, writhing movements with a sinuous quality), and ballism (involuntary movements that are proximal and large amplitude) frequently coexist in the same patient and are felt to be part of the same choreiform spectrum
• Chorea is usually classified as being primary (idiopathic, hereditary) or secondary (acquired)
Term
Chorea Causes
Definition
X-Linked
• McLeod syndrome
• Lesch-Nyhan disease

Maternal Inheritance
• Mitochondrial disorders
Term
Acquired Chorea
Definition
• A variety of acquired conditions can cause chorea.
• These include central nervous system vascular lesions, autoimmune/inflammatory disorders, particularly Sydenham chorea, metabolic and endocrine disorders, infectious diseases, toxins, drugs, structural lesions of the basal ganglia, and so-called senile chorea.
Term
Functional Movement Disorders
Definition
• Clinical syndromes defined by the occurrence of abnormal involuntary movements that are incongruent with a known neurologic cause and are significantly improved on neurological exam with distraction or nonphysiologic maneuvers
• Types: functional tremor, functional dystonia, functional gait, functional myoclonus, and functional Parkinsonism
Term
Functional tremor
Definition
is typically a complex resting, postural, and action tremor of abrupt onset. Any body part may be involved although the fingers are often spared
Term
Functional dystonia
Definition
may affect any part of the body or be generalized. Features include inconsistent dystonic movements over time, dystonia presenting as a fixed posture or a paroxysmal disorder, the presence of incongruous dystonic movements and postures, excessive slowness, marked resistance to passive movements, multiple somatizations, foot dystonia in an adult, and pain as a prominent aspect
Term
Dystonia that occurs after peripheral trauma
Definition
is known by a number of terms, including fixed dystonia, traumatic or post-traumatic dystonia, post-traumatic cervical dystonia, post-traumatic painful torticollis, peripherally induced dystonia, causalgia-dystonia syndrome, and complex regional pain syndrome dystonia. Although controversial, many cases are probably functional.
Term
In functional gait disorders
Definition
walking is often bizarre and does not conform to any of the usual patterns observed with neurologic gait disorders
Term
The characteristics of functional myoclonus
Definition
include features similar to other FMDs such as acute onset, spontaneous periods of remission, inconsistent character of the movements in terms of amplitude, frequency, and distribution, and marked reduction with distraction
Term
ThetremoroffunctionalParkinsonism
Definition
sharesthe characteristics of isolated functional tremor, manifesting as a complex resting, postural, and action tremor with abrupt onset, a static course, and changeable features
Term
Withfunctionalbradykinesia
Definition
movementsareslowand effortful, but usually lack the typical reduction in speed or amplitude that is observed with successive movements in true bradykinesia.
Term
Increasedmuscletone
Definition
isaresultofvoluntaryopposition rather than true rigidity seen with Parkinsonism, and cogwheeling is absent.
Term
Atypicalgaitabnormalitiesandposturalinstability
Definition
are often present, but postural stability testing may reveal bizarre responses. in functional movement disorders
Term
InformingthepatientofthediagnosisofFMD
Definition
isoften problematic, especially if the term "psychogenic" is used; most patients do not accept that the trouble may be psychiatric, and many become alienated and seek another opinion
• Amultidisciplinarytreatmentregimenemphasizing physical therapy and aimed at restoring movement and function is the preferred approach for the management of most patients, psychotherapy with or without pharmacotherapy, CBT, antidepressants, exercise, hypnosis, transcranial magnetic stimulation
Term
Tourette Disorder
Definition
• A common movement and neurobehavioral disorder in children characterized by multiple motor and vocal tics
• Symptoms: multiple motor and vocal tics, with onset before age 21. The diagnosis is often supported by the presence of coexisting behavioral disorders such as ADHD and/or OCD, and a family history of similar symptoms
• Management: antidopaminergic drugs, alpha adrenergic agonists, topiramate, botulinum toxin, habit reversing training, deep brain stimulation
Term
CVA
Definition
• Thesymptomsofbrainischemiamaybetransient, lasting seconds to minutes, or may persist for longer periods of time
• Symptomsandsignsremainindefinitelyifthebrain becomes irreversibly damaged and infarction occurs
• Neurologicsymptomsdonotaccuratelyreflectthe presence or absence of infarction, and the tempo of the symptoms does not indicate the cause of the ischemia
• Musttakeagoodhistory,PE,andperformaCTstat
• Mustdifferentiatethetypeofstrokeoccurring
Term
right vs left cva
Definition
[image]
Term
CVA Pathophysiology
Definition
• The process may be intrinsic to the vessel, as in atherosclerosis, lipohyalinosis, inflammation, amyloid deposition, arterial dissection, developmental malformation, aneurysmal dilation, or venous thrombosis.
• The process may originate remotely, as occurs when an embolus from the heart or extracranial circulation lodges in an intracranial vessel.
• The process may result from inadequate cerebral blood flow due to decreased perfusion pressure or increased blood viscosity.
• The process may result from rupture of a vessel in the subarachnoid space or intracerebral tissue.
• The first three processes can lead to transient brain ischemia (transient ischemic attack [TIA]) or permanent brain infarction (ischemic stroke), while the fourth results in either subarachnoid hemorrhage or an intracerebral hemorrhage (primary hemorrhagic stroke)
• Blood pressure is usually elevated, increased ICP due to hemorrhage or ischemia, decreased respiratory drive, fever
• Take a good history/drug use: seizures, syncope, migraine, and hypoglycemia can mimic acute ischemia
• Management: CT, MRI, lumbar puncture, monitor for a-fib, echo, TEE, complete blood count, platelet count, prothrombin time and partial thromboplastin time, and serum lipids, hypercoagulable studies, check for bleeding disorders, thrombolytic therapy, thrombectomy
Term
TIA
Definition
• Defined as a transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischemia, without acute infarction
• Absence of end organ failure
Term
Stroke: Intracerebral Hemorrhage
Definition
• Derived from arterioles or small arteries
• Neurological symptoms progress during minutes or hours
• Risk factors: Hypertension, trauma, bleeding diatheses, illicit drugs (amphetamines, cocaine), vascular malformations. More common in blacks and Asians than in whites. May be precipitated by sex or other physical activity. Patient may have reduced alertness.
Term
Stroke: Subarachnoid Hemorrhage
Definition
• Rupture of arterial aneurysms is the major cause of subarachnoid hemorrhage (SAH). Aneurysm rupture releases blood directly into the cerebrospinal fluid (CSF) under arterial pressure. The blood spreads quickly within the CSF, rapidly increasing intracranial pressure
• Symptoms: abrupt, sudden, severe headache, “worst headache of my life”, seizure, N/V, focal neurologic deficit, stiff neck
• Risk factors: Smoking,
hypertension, moderate to heavy alcohol use, genetic susceptibility (polycystic kidney disease, family history of subarachnoid hemorrhage) and sympathomimetic drugs (cocaine), sex, physical activity
Term
Stroke: Ischemic (Thrombotic)
Definition
• Thrombusformationinanarteryproducesastroke either by reduced blood flow distally (low flow) or by an embolic fragment that breaks off and travels to a more distant vessel (artery-to-artery embolism)
• Stutteringprogressionwithperiodsofimprovement. Lacunes develop over hours or at most a few days; large artery ischemia may evolve over longer periods.
• Riskfactors:Atheroscleroticriskfactors(age, smoking, diabetes mellitus, etc.). Men affected more commonly than women. May have history of TIA.
• Neckbruitmaybepresent
Term
Stroke: Ischemic (Embolic)
Definition
• Embolismreferstoparticlesofdebrisoriginating elsewhere that block arterial access to a particular brain region. Since the process is not local (as with thrombosis), local therapy only temporarily solves the problem; further events may occur if the source of embolism is not identified and treated
• Suddenonsetwithdeficitmaximalatonset.Clinical findings may improve quickly.
• Riskfactors:Atheroscleroticriskfactorsaslisted above. Men affected more commonly than women. History of heart disease (valvular, atrial fibrillation, endocarditis), can be precipitated by getting up at night to urinate, or sudden coughing or sneezing.
Term
Aphasia
Definition
• Thelossofabilitytoproduceand/orunderstand language
• Usuallymanifestsasadifficultyspeakingor understanding spoken language; reading and writing also may be impacted
• Languagecenters:Broca’sorBrodmann’sarea44- mouth, larynx and controls output of spoken language; Wernicke’s or Broadmann’s area 22, receives information from the auditory cortex and accesses a network of cortical associations to assign word meanings; Angular gyrus, perception of written language and processes; other areas as well
• Causes: stroke, neoplasm, cerebral abscess, encephalitis, nervous system infections, and TBI; MS and encephalomyelitis uncommon; TIA, migraine, and seizures-transient aphasia
• Management: neuroimaging, speech and communicative disorders consult
Term
broca's area
Definition
expressive aphasia, infarct of superior division of left mca
Term
wernicke's area
Definition
receptive aphasia, infarct of inferior division of left mca
Term
Aphasia: Broca’s
Definition
• Characterized by nonfluency with sparse output and agrammatism
• Repetition is also impaired. Comprehension is relatively spared but is usually not completely normal, particularly for grammatically complex speech.
• Writing is generally affected in proportion to speech.
• The association between Broca's aphasia and Broca's area is most consistently observed in acute rather than chronic stroke
Term
Aphasia: Wernicke’s
Definition
• A fluent aphasia with markedly impaired comprehension.
• Speech is voluminous, but meaningless, containing paraphasic errors and neologisms. This is often described as "word salad". The speech usually retains normal cadence and intonation
• Comprehension and production of written language is similarly impaired. The patient appears unaware of the deficit.
• Associated with lesions in the posterior superior temporal gyrus (Wernicke's area), there is typically no motor deficit with this syndrome
• However, a right superior visual field defect may be present
Term
Aphasia: Global
Definition
• Includes deficits in all language functions
• Patients are often mute or produce only nonword utterances, patients generally have a right hemiparesis and often a right visual field deficit
• They cannot follow commands, although they may respond to the intonation or expression of the speech.
Term
Syncope
Definition
• A clinical syndrome in which transient loss of consciousness (TLOC) is caused by a period of inadequate cerebral nutrient flow, most often the result of an abrupt drop of systemic blood pressure. Typically, the inadequate cerebral nutrient flow is of relatively brief duration (8 to 10 seconds) and, in syncope, is by definition spontaneously self-limited.
• Patient may fall which may result in injury
Term
Reflex syncope
Definition
– Vasovagal syncope (also known as the "common" or "innocent" faint), a type of neurally-mediated syncope, is a common cause of transient loss of consciousness (TLOC), particularly in patients without apparent cardiac or neurologic disease
– Carotid sinus syncope
– Syncope triggered by micturition, defecation, swallowing, or coughing
Term
Cardiac Arrhythmias
Definition
the heart rate is either too slow or too fast to permit maintenance of an adequate cardiac output and systemic arterial pressure- can cause syncope
– AV block, cardiac pauses, VT, Ventricular bigeminy, SVT
– Individuals with underlying heart disease (eg, previous myocardial infarction [MI], valvular heart disease), a channelopathy (long QT syndrome, Brugada syndrome), HCM, congenital heart disease, or significant vascular disease are at greatest risk for syncope due to a tachyarrhythmia (usually VT)
Term
Orthostatichypotensionsyncope
Definition
mostoftenoccurring following movement from lying or sitting to a standing position
– Decreased intravascular volume, drugs, primary autonomic insufficiency or failure, ETOH, aging
Term
StructuralCardiopulmonarydisease
Definition
-can cause syncope
– due to inadequate cardiac output include cardiac valvular disease (particularly aortic stenosis), HCM, atrial myxoma, pulmonary embolus, pulmonary hypertension, pericardial tamponade, acute MI/ischemia, and acute aortic dissection.
– Channelopathies such as long QT syndrome, Brugada syndrome, catecholaminergic polymorphic VT, and others that are less frequent should also be included here, although the "structural" disease is at the cellular level.
Term
Cerebrovasculardisease
Definition
-can cause syncope
– Atherosclerotic disease of the cerebral arteries is almost never the cause of true syncopal symptoms, as the brain has a very redundant blood supply. Instead, stroke and transient ischemia attacks cause focal neurologic deficits that do not recover rapidly or completely
– Examples:
• If the posterior cerebral circulation is impaired (vertebrobasilar artery insufficiency), symptoms such as dizziness are more apt to occur than syncope. Other causes of syncope should be excluded in patients with syncope and suspected vertebrobasilar ischemia.
• If the anterior circulation is compromised, a focal neurologic deficit and not a global decrease in consciousness will occur.
Term
vascular steal syndrome
Definition
-can cause syncope
occurs when the arterial circulation to the arm is blocked, resulting in a shunt of blood through the cerebrovascular system that supplies both parts of the brain and the arm. Impairment in brain perfusion during arm exercise may cause loss of consciousness. Vertebrobasilar steal is associated with vertigo, diplopia, blurred vision, cranial nerve dysfunction, drop attacks (sudden fall without loss of consciousness), and syncope
• Management: history, ECG, cardiac monitoring, Holter, electrolytes, CBC, MRI if suspecting stroke or seizure, ECHO,
Term
Complex Regional Pain Syndrome
Definition
• Adisorderofabodyregion,usuallyofthedistallimbs, which is characterized by pain, swelling, limited range of motion, vasomotor instability, skin changes, and patchy bone demineralization.
• Itfrequentlybeginsfollowingafracture,softtissue injury, or surgery
• Thepainisregional(notinaspecificnerveterritoryor dermatome)
• Twotypes:Type1andType2,somesayWarmand Cold Types distinguished by temperature of skin
• Morecommoninwomen
• Causenotfullyunderstood
• Symptoms: pain that is burning, stinging, or tearing felt deep inside the limb; sensory changes such as hyperalgesia, allodynia or hypesthesia, sometimes sensory disturbances are like in a stocking/glove pattern; motor disturbances-due to edema, pain or contractures; autonomic changes in temperature, skin color, sweat or edema; trophic changes such as increased hair growth, nail changes, skin atrophy, and fibrosis of joints and fascia
• The diagnosis of CRPS is based upon the clinical features as determined by the history and physical examination. Diagnostic criteria are based upon four symptom/sign categories (sensory, vasomotor, sudomotor/edema, and motor trophic) and require that the patient report one symptom in three of the four categories and display one sign at the time of evaluation in two of the four categories. Importantly, the criteria require that no other diagnosis better explains the symptoms and signs
• Management: patient education, psychosocial and behavioral management, PT, OT, NSAIDS, anticonvulsants, antidepressants, bisphosphonates, topical lidocaine and capsaicin, calcitonin, glucocorticoids; interventional procedures such as trigger point injections, spinal cord stimulation, epidurals, nerve blocks
Term
Central nervous system disorders are
Definition
crippling, as they affect our movements as well as the sensory functions of the body.
• Nerve cells are called neurons.
• Each neuron has a main body and radiating fibrils of short and long lengths.
– The short fibrils are called dendrons while the long ones are called axons.
– Both the dendrons and axons are responsible for transmission of information in the form of chemical and electrical impulses to other neurons and other parts of the body like muscles.
Term
Axonal Neuropathy vs Demyelinating Neuropathy
Definition
• A neuropathy is the medical term for any condition involving the neurons.
• Axonal neuropathy is a neurological disorder that involves degeneration and loss of axons, whereas demyelinating neuropathy causes degeneration of myelin (fatty layer of insulating substance) surrounding axons of neurons.
• If demyelinating diseases are not treated in time, then they will eventually damage the axons too.
• The leading cause of axonal damage is any auto-immune condition where auto-antibodies attack the coating of motor nerves without causing inflammation or loss of myelin.
• Usually, the sensory nerves are spared but the motor nerves are affected.
• Motor nerves are the ones that conduct commands from the brain to the body, for example, movements, etc.
• In the case of acute diseases, axonal neuropathy is seen in a variant of Guillian Barre Syndrome.
• In the case of chronic disorders, it is seen in conditions like diabetes, leprosy, etc. where both motor and sensory nerves are damaged as the disease progresses
• Demyelinatingdisordersaregenerallyseendueto genetic causes, infectious agents and autoimmune reactions.
• Inthese,themyelinwhichsurroundstheaxonsof neurons is gradually destroyed by antibodies.
• Thismyelincoatingisneededforfastertransmissionof impulses and hence, once myelin is lost, impulse transmission is either very slow or completely lost.
• Indemyelinatingneuropathy,bothsensoryandmotor nerves are affected.
• Thisisaverycharacteristicdifferencebetweenthetwo neuropathies.

• Clinically, in an axonal neuropathy, movements of muscles are affected. There is tingling, numbness and a prickling sensation noted. Sensitivity to temperature may be lost in the affected area. There is loss of reflexes without sensory loss.
• There is a paralytic feeling of limbs in axonal neuropathy, whereas in demyelinating neuropathy there is a progressive weakness and fatigue of muscles.
• Also, there is blurred vision, double vision, movement difficulties, incoordination of muscles. Hence, walking and balancing become difficult. There is incontinence of urine and stools. Loss of reflexes in muscles that are not particularly weak or wasted is a classic feature of demyelination.
• On the other hand, selective loss of the ankle jerk reflex in the presence of wasting and weakness of the foot is more typical of an axonal damage.
• In patients with axonal neuropathies, the impulse conduction velocity recorded from distant muscles like foot and ankle is less but relatively normal near proximal sites like arms. In the case of demyelinating neuropathy, patients show slowing of conduction of impulses at both proximal and distal sites on electromyogram.
Term
Polyneuropathy
Definition
isaspecifictermthatreferstoa generalized, relatively homogeneous process affecting many peripheral nerves, with the distal nerves usually affected most prominently.
Term
Peripheralneuropathy
Definition
isalessprecisetermthatis frequently used synonymously with polyneuropathy, but can also refer to any disorder of the peripheral nervous system including radiculopathies and mononeuropathies.
Term
Neuropathy
Definition
whichagainisfrequentlyused synonymously with peripheral
neuropathy and/or polyneuropathy, can refer even more generally to disorders of the central and peripheral nervous system.
Term
polyneuropathies
Definition
must be distinguished from other diseases of the peripheral nervous system, including the mononeuropathies and mononeuropathy multiplex (multifocal neuropathy), and from some disorders of the central nervous system.
Term
Mononeuropathy
Definition
refers to focal involvement of a single nerve, usually due to a local cause such as trauma, compression, or entrapment. Carpal tunnel syndrome is a common example of a mononeuropathy.
Term
Mononeuropathy multiplex
Definition
refers to simultaneous or sequential involvement of noncontiguous nerve trunks. Used loosely, this term can refer to multiple compressive mononeuropathies. However, in its more specific meaning, it identifies multiple nerve infarcts due to a systemic vasculitic process that affects the vasa nervorum.
• Diseases of the central nervous system such as a brain tumor, stroke, or spinal cord lesion occasionally present with symptoms that are difficult to distinguish from polyneuropathy
Term
Peripheral Nerves vs. Muscle Disease
Definition
• Patients with disorders of nerves, muscle, or the neuromuscular junction can present with a similar constellation of symptoms
• Diseases of the nerve (polyneuropathy), muscle (myopathy), and neuromuscular junction (myasthenia gravis) may present with varying degrees of sensory loss and weakness
• In addition, even when the process is localized to the nerve, it is not always clear if the origin of the problem is in the central or peripheral nervous system.
Term
CLINICAL DISTINCTION BETWEEN NERVE, MUSCLE AND NEUROMUSCULAR JUNCTION DISEASE
Definition
• History: Focal sensory loss in the absence of a known central nervous system (CNS) lesion suggests a peripheral problem occurring somewhere along the length of the nerve between the nerve root and the most distal fibers.
• The loss of certain modalities (vibration
or sharp/dull discrimination) may also help identify the problem.
• Weakness tends to be a difficult symptom for patients to quantify and localize. Frequently patients will complain of pain and associated weakness. However, since pain may limit the action and mobility of a joint, the finding of weakness in the setting of pain may not indicate true neurologic dysfunction. Patients who complain of painless weakness generally have significant abnormalities on examination. Similar to sensory loss, the distribution of weakness is extremely important.
• History: Cranial nerve complaints can be very useful in localizing the site of the problem. In the absence of CNS disease, diplopia is likely secondary to a neuromuscular junction defect. Progressive dysarthria without associated eye symptoms suggests motor neuron disease (ALS). Less frequently, myopathies or acute motor polyneuropathies (Guillain-Barré syndrome) can present with dysarthria and eye movement abnormalities
Term
Neuro Exam CLINICAL DISTINCTION BETWEEN NERVE, MUSCLE AND NEUROMUSCULAR JUNCTION DISEASE
Definition
should focus upon the distribution of muscle weakness and sensory loss and the DTR’s
• Proximal weakness is most consistent with a myopathy or neuromuscular junction disorder. Weakness in these individuals is usually symmetric, with involvement of head extensors and flexors, although mild asymmetries are not unusual. However, some patients with myasthenia may present with or develop predominately distal weakness. Repetitive movements may increase the weakness in myasthenia gravis
• If the weakness involves predominantly only one extremity, it is useful to determine if the lesion is in the distribution of a single root, a region of the plexus, or a single nerve territory;
• Observation of the muscles themselves is often helpful. Important features include the presence of fasciculations, whether the muscle produces a painful cramp after activation, and the presence of atrophy (ALS)
Term
Neuro Exam Sensory CLINICAL DISTINCTION BETWEEN NERVE, MUSCLE AND NEUROMUSCULAR JUNCTION DISEASE
Definition
In axonal polyneuropathies, "glove and stocking" sensory loss is most prominent, initially affecting the feet and later the hands
• Root lesions may cause sensory loss in a dermatomal pattern.
• Numbness in a single nerve territory might suggest an entrapment neuropathy.
• Multiple areas of numbness in different extremities point to a mononeuropathy multiplex.
Term
Neuro Exam Reflexes CLINICAL DISTINCTION BETWEEN NERVE, MUSCLE AND NEUROMUSCULAR JUNCTION DISEASE
Definition
The loss of reflexes suggests a neuropathic lesion, generally affecting either sensory or motor fibers. Globally depressed or absent reflexes are frequently associated with Guillain-Barré syndrome
• Reflexes are generally preserved in muscle disease unless it is severe.
• Neuromuscular junction disorders do not follow a clear pattern: in myasthenia gravis reflexes are generally normal; in Lambert-Eaton myasthenic syndrome, reflexes are reduced or absent.
Term
PointsofConfusion CLINICAL DISTINCTION BETWEEN NERVE, MUSCLE AND NEUROMUSCULAR JUNCTION DISEASE
Definition
Painisgenerallynotaprominent symptom of myopathy. Most myopathic processes are relatively painless, including polymyositis, dermatomyositis, toxic myopathies, and inclusion body myositis. Generally, muscle pain is confined to patients with acute rhabdomyolysis, a few metabolic myopathies (such as McArdle Disease), and myopathies associated with connective tissue disease (the pain perhaps secondary to joint involvement rather than muscle involvement itself).
• Myopathiesmaybesuperimposedupon polyneuropathies
Term
DISTINGUISHING PERIPHERAL FROM CENTRAL NERVOUS SYSTEM DISEASE
Definition
Diseases of the peripheral nervous system include the mononeuropathies, polyneuropathies, and mononeuropathy multiplex, each of which may be caused by a variety of underlying systemic illnesses, toxic exposures, and rheumatic genetic diseases.
• Distinguishing peripheral from CNS disease (that due to stroke, brain tumor, or spinal cord lesions) is straightforward in most cases. Weakness or sensory loss confined to one limb generally suggests a peripheral nerve problem. If there is associated pain in the extremity, then the likelihood of a peripheral process is even greater.
• However, localization of the problem is less clear when weakness or sensory loss is no longer confined to one limb or when no associated pain/discomfort is present. A patient who presents with painless sensory loss and numbness in the feet may have a polyneuropathy or a spinal cord lesion. Complaints of poor coordination or unsteady gait can also represent primarily central or peripheral dysfunction.
Term
Both central and peripheral nervous system disorders
Definition
can be present simultaneously; in such a situation, even detailed questioning of a patient may not clarify the origin of the problem.
• The neurologic examination plays an important role in differentiating central and peripheral nervous system disease
Term
Examination of the deep tendon reflexes
Definition
is the single most important test in determining whether a problem is peripheral or central.
• Brisk reflexes suggest central pathology.
• Hyporeflexia or areflexia points to a peripheral problem.
Term
Weakness caused by upper motor neuron (CNS) problems
Definition
generally follows a specific pattern in which weakness is predominant in extensor muscles in the arm (deltoid, triceps, wrist, and finger extensors) and flexor muscles in the leg (iliopsoas, hamstrings, and foot dorsiflexors).
• Increased tone on passive movement of the extremities also suggests a CNS process.
• Atrophy of muscles isolated to a specific root(s) or peripheral nerve places the problem in the peripheral nervous system.
Term
CLINICAL DISTINCTION BETWEEN NERVE, MUSCLE AND NEUROMUSCULAR JUNCTION DISEASE
• Management
Definition
Electromyography and nerve conduction studies, genetic testing, Muscle biopsy may assist in the accurate diagnosis of myopathy.
• For patients with polyneuropathy, nerve biopsy is occasionally helpful to define whether the process is predominantly axonal or demyelinating.
• Epidermal skin biopsy is an especially useful test in the diagnosis of polyneuropathy that predominantly affects small, unmyelinated nerve fibers
Term
Peripheral Neuropathies: Lower Extremity
Definition
• The lumbosacral plexus has two main components: the lumbar plexus (made up of nerve fibers from the L2 through L5 roots) and the sacral plexus (made up of nerve fibers from the S1 through S4 roots)
• The major mechanisms of lower extremity peripheral nerve injury are compression, transection, ischemia, radiation exposure, inflammation, and neuronal degeneration
• The most common nerve problem affecting the lower extremities is probably lumbosacral radiculopathy
Term
Peripheral Neuropathies: Upper Extremity
Definition
• The brachial plexus anatomy is delineated through trunks, divisions, and cords
• The major mechanisms of upper extremity peripheral nerve injury are compression, transection, ischemia, inflammation, neuronal degeneration, and radiation exposure
• Carpal tunnel syndrome is the most frequent mononeuropathy affecting the upper extremities, while ulnar neuropathy at the elbow is the second most frequent. Cervical radiculopathy is also common
• Classic carpal tunnel syndrome is pain or paresthesia in a distribution that includes the median nerve territory, with involvement of the lateral portion of the hand
• Ulnar neuropathy: sensory loss and paresthesias over digits 4 and 5
• Theradialnerveispredisposedtocompressioninthe region where it runs adjacent to the humerus, known as the spiral groove.
• Thetricepsretainsfullstrengthbutthereisweakness of the wrist extensors (wrist drop), finger extensors, and brachioradialis. Sensory loss over the dorsum of the hand, possibly extending up the posterior forearm, may also be present
• Proximalfocalneuropathiesoftheupperextremity include suprascapular neuropathy, long thoracic neuropathy, and axillary neuropathy.
• Cervical radiculopathy is a common cause of both acute and chronic neck pain. Most radiculopathies arise from nerve root compression due to cervical
spondylosis and/or disc herniation. Lower cervical roots, particularly C7, are more frequently affected by compression.
• Some causes of noncompressive radiculopathy include infection (especially herpes zoster and Lyme disease), nerve root infarction, infiltration by tumor, infiltration by granulomatous tissue, root avulsion, and demyelination.
• Additional uncommon peripheral nerve syndromes affecting the upper extremities include focal amyotrophy, mononeuropathy multiplex, multifocal motor neuropathy, and zoster radiculoganglionitis
Term
Demyelinating Disorders
Definition
• A demyelinating disease is any disease of the nervous system in which
the myelin sheath of neurons is damaged
• This damage impairs the conduction of signals in the affected nerves.
• In turn, the reduction in conduction ability causes deficiency in sensation, movement, cognition, or other functions depending on which nerves are involved.
Term
Demyelinating Disorders of the CNS
Definition
• The demyelinating disorders of the central nervous system include:
– Myelinoclastic disorders, in which myelin is attacked by external substances
• standard multiple sclerosis, Devic's disease and other disorders with immune system involvement called inflammatory demyelinating diseases.
– Leukodystrophic disorders, in which myelin is not properly produced:
– CNS neuropathies like those produced by vitamin B12 deficiency
– Central pontine myelinolysis
– Myelopathies like tabes dorsalis (syphilitic myelopathy)
– Leukoencephalopathies like progressive multifocal leukoencephalopathy
– Leukodystrophies
• These disorders are normally associated also with the
conditions optic neuritis and transverse myelitis, which are inflammatory conditions, because inflammation and demyelination are frequently associated. Some of them are idiopathic and for some others the cause has been found, like some cases of neuromyelitis optica.
Term
Demyelinating Diseases of the Peripheral Nervous System
Definition
• GBS
• Anti-MAG peripheral neuropathy
• Charcot-Marie-Tooth Disease
• Copper Deficiency
• Progressive inflammatory neuropathy
Term
Disuse Atrophy
Definition
• Disuse atrophy is caused by not using the muscles enough.
• This type of atrophy can often be reversed with exercise and better nutrition.
• People who are most affected are those who:
– Have seated jobs, health problems that limit movement, or decreased activity levels.
– Are bedridden
– Cannot move their limbs because of stroke or other brain disease
Term
Disuse Atrophy Due to Disease
Definition
• Muscle atrophy disease conditions ranging from cancer AIDs congestive heart failure renal failure, severe burns.
• Inadequate dietary intake or malnutrition which causes progressive weakening resulting in inability to use the muscles.
• Aging: Elderly people normally lose their ability to maintain muscle function and mass.
• Injury, such as a broken arm or leg that must be immobilized
• Dermatomyositis which is a skin condition characterized by inflammation of the skin and muscle.
• Muscular dystrophy which includes a group of muscle weakening disorders causing progressive loss of muscle and muscle weakness due to deterioration of muscle cells and death of muscle tissue.
• Arthritis: Rheumatoid arthritis and osteoarthritis are known to cause joint inflammation making mobility difficult. Pain associated with arthritis may cause patients to become sedentary leading to muscle wasting.
• Multiple sclerosis may also cause disuse atrophy due to weakness of certain limbs and inability to use them.
Term
Neurogenic Atrophy
Definition
• Neurogenic atrophy is the most severe type of muscle atrophy.
• It can be from an injury to, or disease of, a nerve that connects to the muscle.
• This type of muscle atrophy tends to occur more suddenly than disuse atrophy.
• Examples of diseases affecting the nerves that control muscles: – Amyotrophic lateral sclerosis (ALS, or Lou Gehrig disease)
– Damage to a single nerve, such as carpal tunnel syndrome
– Guillain-Barre syndrome
– Nerve damage caused by injury, diabetes, toxins, or alcohol – Polio (poliomyelitis)
– Spinal cord injury
– Multiple Sclerosis
– Stroke
Term
Disuse and Neurogenic Atrophy Management
Definition
identify the cause, PT, OT, nutrition (increase protein, omega 3 fatty acids), electrical simulation, TENS unit, acupuncture,
Term
Muscle Weakness
Definition
• The evaluation of the patient presenting with a complaint of "weakness" involves three steps:
• 1. Distinguishing true muscle weakness from lassitude or motor impairment not due to loss of muscle power
• 2. Localizing, within the neuromuscular system, the site of the lesion that is producing weakness
• 3. Determining the cause of the lesion
• Must distinguish true muscle weakness from lassitude or motor impairment not due to loss of muscle power; localizing, within the neuromuscular system, the site of the lesion that is producing weakness; and determining the cause of the lesion
• If the weakness is not generalized, it can be characterized as symmetric or asymmetric.
• Asymmetric weakness most likely reflects disease of the central or peripheral nervous system
• Lesions of the motor cortex, spinal cord, spinal nerve root, and peripheral nerve each have distinct distribution patterns.
• Symmetric patterns of weakness can be divided into distal, proximal, or specific distributions.
• Distal weakness is characterized by decreased grip strength, weakness of wrist flexion or extension, decreased plantar flexion strength, and foot drop.
• These patients have difficulty walking on their heels or toes.
• Foot drop can be detected by opposing the patient's attempt to dorsiflex the ankle.
• Distal symmetric weakness is characteristic of early motor neuron disease or peripheral neuropathy
• Proximal weakness involves the axial muscle groups, deltoids, and hip flexors.
• Affected patients may have difficulty flexing or extending the neck against resistance.
• One way to detect the presence of neck flexor weakness is to observe the patient sitting up from the supine position. In this setting, the head will lag behind as the patient sits up. Sitting up may be difficult or even impossible in patients with more severe proximal muscle weakness or, at times, may be the only objective evidence of weakness.
• Deltoid muscle strength can be assessed by pressing down on the patient's fully abducted arms with the elbows flexed. The examiner should not be able to overcome the patient's resistance if strength is normal.
• The patient with quadriceps weakness may be unable to rise from a seated position without the use of the upper extremities and is usually unable to perform a deep knee bend. These individuals may suddenly drop into the chair when trying to sit down slowly. Patients with proximal leg weakness may rise from sitting on the floor by "climbing up their legs with their hands." This is termed Gower's sign and is characteristic of, but is not specific for, Duchenne muscular dystrophy. Proximal muscle weakness is typically seen in the various myopathies, certain muscular dystrophies, and myasthenia gravis
• Upper motor neuron impairment can occur with the common acute stroke syndromes, space occupying lesions of the central nervous system, and lesions of the spinal cord. Spinal cord lesions can be related to trauma, infection, tumor, vascular anomalies, hypertrophic degenerative skeletal changes, demyelinating diseases, and congenital leukodystrophies
• Weakness due to involvement of the anterior horns cells is seen in motor neuron disease, familial spinal atrophy, lead poisoning, and poliomyelitis, which is still a threat to unimmunized individuals. West Nile and other virus infections represent additional causes of lower motor neuron disease
• Lesions of the peripheral nervous system: Involvement of peripheral nerves typically presents in one of two patterns:
• A symmetric polyneuropathy with weakness and sensory symptoms is a frequent sequela of diabetes mellitus. Other etiologies include a variety of toxic or metabolic insults, as well as heritable disorders
• Mononeuropathy may be the result of nerve compression (carpal or tarsal tunnel syndrome, ulnar neuropathy, radial neuropathy). Mononeuropathy multiplex (asymmetric polyneuropathy) occurs in diabetes mellitus or one of the vasculitic syndromes such as polyarteritis nodosa
• Weakness may result from involvement of the neuromuscular junction. This can be induced by anti-acetylcholine receptor antibodies in myasthenia gravis or drug- induced myasthenia, inhibition of acetylcholinesterase by organophosphate poisoning, or interference with presynaptic calcium channel function in Lambert-Eaton syndrome
Term
muscle weakness management
Definition
serum electrolytes, calcium, magnesium, phosphate, creatine kinase, aldolase, lactate dehydrogenase, serum aminotransferases, and thyroid stimulating hormone, NCS, EMG, muscle biopsy if no identifiable cause
Term
Weakness: Myopathy
Definition
• The major categories of muscle disease include inflammatory disorders, endocrinopathies, metabolic myopathies, drugs and toxins, infections, and the various causes of rhabdomyolysis
Term
Myasthenia Gravis
Definition
• Themostcommondisorderofneuromuscular transmission.
• Itisnowoneofthebestcharacterizedandunderstood autoimmune disorders.
• Thehallmarkofthedisorderisafluctuatingdegreeand variable combination of weakness in ocular, bulbar, limb, and respiratory muscles.
• Weaknessistheresultofanantibody-mediated,T-cell dependent immunologic attack directed at proteins in the postsynaptic membrane of the neuromuscular junction (acetylcholine receptors or receptor-associated proteins).
• Age of onset is characterized by an early peak in the second and third decades (female predominance) and a late peak in the sixth to eighth decade (male predominance).
• About 10 to 15 percent of those with myasthenia gravis have an underlying thymoma
• Thecardinalfeatureisfluctuatingskeletalmuscle weakness, often with true muscle fatigue. The fatigue is manifest by worsening contractile force of the muscle.
• Therearetwoclinicalforms:ocularandgeneralized.
• Inocularmyasthenia,theweaknessislimitedtothe eyelids and extraocular muscles.
• Ingeneralizeddisease,theweaknessmayalso commonly affect ocular muscles, but it also involves a variable combination of bulbar, limb, and respiratory muscles
• More than 50 percent of patients present with ocular symptoms of ptosis and/or diplopia.
• Of those who present with ocular manifestations, about half will remain purely ocular.
• About 15 percent of patients present with bulbar symptoms. These include fatigable chewing, dysphagia, and dysarthria. Less than 5 percent present with proximal limb weakness alone.
• Eyelid muscle weakness can lead to ptosis that can vary throughout the day. Extraocular muscle weakness produces binocular diplopia that disappears when the patient closes or occludes one eye.
• Muscles of the face, jaw, neck extensor and flexor muscles producing a “dropped head syndrome,” muscles of respiration creating respiratory insufficiency “myasthenic crisis”
• Management:icepacktest,Tensilontestforptosis
http://webeye.ophth.uiowa.edu/eyeforum/video/Tensil on.htm
• Immunologicand/orelectrophysiologictesting, autoantibodies against the acetylcholine receptor (AChR-Ab), Antibodies to muscle specific tyrosine kinase (MuSK), repetitive nerve stimulation (RNS) studies and single-fiber electromyography (SFEMG), electrophysiologic tests usually allow confirmation of the diagnosis in patients who have seronegative myasthenia gravis, chest CT/MRI to look for thyoma
• The four basic therapies for myasthenia gravis (MG) include symptomatic treatments (anticholinesterase agents), chronic immunotherapies (glucocorticoids and other immunosuppressive drugs), rapid immunotherapies (plasma exchange and intravenous immune globulin [IVIG]), and thymectomy.
Term
Seizures
Definition
• Seizures are typically referred to as either acute symptomatic seizures or unprovoked seizures depending on the clinical circumstances at the time of the event.
• Epilepsy refers to the tendency for recurrent, unprovoked seizures
• Acute symptomatic seizures are those that occur in the setting of acute medical (hypoglycemia, hyponatremia) or neurologic illness or injury (stroke, traumatic brain injury, meningitis, anoxic encephalopathy).
• Such seizures may recur during the index illness but generally don’t progress to epilepsy
• Unprovoked seizure refers to a seizure of unknown etiology as well as one that occurs in relation to a preexisting brain lesion or progressive nervous system disorder.
• Approximately one-third of adults with a first unprovoked seizure will have a recurrent seizure (epilepsy)
• Causes: genetic, structural, metabolic, immune, infectious, and unknown.
• While a significant proportion of epilepsy in childhood has a genetic, metabolic, or congenital structural basis, epilepsy diagnosed in adults is more likely to be due to an acquired vascular, degenerative, or neoplastic etiology.
• Types: focal or generalized according to whether the onset of electrical activity involves a focal region of the brain or the entire cortex simultaneously. Focal seizures are further classified according to whether consciousness is altered or not during the event
• The clinical manifestations of seizures vary based on the location of the seizure in the brain and the amount of cortex that is involved.
• History-including possible triggers or precipitants, the ictal behaviors, and the postictal state, prior similar events including subtle myoclonus or auras
• Physical Exam: detailed neuro exam
• Objective Tests: electrolytes, glucose, calcium, magnesium, complete blood count, renal function tests, liver function tests, urinalysis, and toxicology screens, EKG, EEG (urgently when impaired sensorium is persistent), and a neuroimaging study, occasionally an LP
Term
Focal Seizures
Definition
• Two Types:
• 1. Focal seizures with retained awareness (previously called simple partial seizures)
• 2. Focal seizures with impaired awareness (previously called complex partial seizures, are the most common type of seizure in adults with epilepsy)
• Focal seizures of both types may propagate diffusely to cause bilateral tonic-clonic seizures (previously referred to as secondarily generalized seizures
Term
Focal Seizures with Retained Awareness
Definition
• Varyfromonepatienttoanotheranddependentirelyon the part of the cortex that is disrupted at the onset of the seizure.
• Aseizurethatbeginsintheoccipitalcortexmayresultin flashing lights
• Aseizurethataffectsthemotorcortexwillresultin rhythmic jerking movements of the face, arm, or leg on the side of the body opposite to the involved cortex (Jacksonian seizure)
• Aseizurethatbeginsintheparietalcortexmaycause distortion of spatial perception
• Aseizurethatbeginsinthedominantfrontallobemay cause sudden speech difficulties.
Term
Focal Seizures with Retained Awarenes
Definition
• The symptoms that a patient experiences at the beginning of a seizure are sometimes referred to as the warning or aura.
• Auras are focal seizures that affect enough of the brain to cause symptoms, but not enough to interfere with consciousness
• Postictally, patients may return immediately to their pre-event baseline or may experience a period of worsened neurologic function related to the location of the seizure in the brain.
– Patients with a simple motor seizure involving the left arm may have postictal weakness lasting for minutes to hours, referred to as a postictal Todd paralysis.
– Postictal weakness or aphasia is more prominent than the ictal phase of the seizure, leading clinicians to consider transient ischemic attack or stroke as a possible diagnosis at the time of initial presentation.
Term
Focal Seizures with Impaired Awareness
Definition
• Focalseizuresassociatedwithalteredawarenessatthe onset of the seizure or as it progresses; most common type of seizure in adults with epilepsy
• Patientsappeartobeawakebutarenotincontactwith others in their environment and do not respond normally to instructions or questions, may stare into space and either remain motionless or engage in repetitive behaviors, called automatisms, such as facial grimacing, gesturing, chewing, lip smacking, or repeating words or phrases. Some patients may become hostile or aggressive if physically restrained
• Typically last less than 3 minutes and may begin with a phase of preserved awareness, which the patient sometimes can later describe, then patient enters the postictal phase, often characterized by somnolence, confusion, and headache for up to several hours Patients often have no memory of happened other than, sometimes remembering the aura.
Term
Generalized Seizures
Definition
• Generalized tonic-clonic seizures (also called grand mal seizures, major motor seizures, or convulsions) are the most common type of generalized seizures.
• Begin with an abrupt loss of consciousness, sometimes in association with a scream or choking sound, muscles of the arms and legs as well as the chest and back then become stiff, patient appears cyanotic during this tonic phase.
• After approximately one minute, the muscles begin to jerk and twitch for an additional one to two minutes.
• During this clonic phase the tongue can be bitten, and frothy and bloody sputum may be seen coming out of the mouth.
• The postictal phase begins once the twitching movements end. The patient is initially in a deep sleep, breathing deeply, and then gradually wakes up. Postictal confusion or agitation is common
• Absence seizures (also called petit mal) usually occur during childhood and typically last between 5 and 10 seconds.
• Occur in clusters and may happen dozens or even hundreds of times a day.
• May cause sudden staring with impaired consciousness.
• If it lasts for 10 seconds or more, there may also be eye blinking and lip smacking
Term
Clonic seizures
Definition
cause rhythmic jerking muscle contractions that usually involve the arms, neck, and face.
Term
Myoclonic seizures
Definition
consist of sudden, brief muscle contractions that may occur singly or in clusters and that can affect any group of muscles, although typically the arms are affected. Consciousness is usually not impaired.
Term
Tonic seizures
Definition
cause sudden muscle stiffening, often associated with impaired consciousness and falling to the ground.
Term
Atonic seizures
Definition
(also known as drop seizures) produce the opposite effect of tonic seizures: a sudden loss of control of the muscles, particularly of the legs, that results in collapsing to the ground and possible injuries
Term
seizures management
Definition
acute: benzos
Term
Status Epilepticus
Definition
• Status epilepticus is a condition resulting from either the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms that lead to abnormally prolonged seizures
• Status epilepticus is a condition that can have long-term consequences including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures
• ≥5 minutes of continuous seizures, or
• ≥2 discrete seizures between which there is incomplete recovery of consciousness
• Controversial regarding times
• Etiologies include acute brain injury or infection, noncompliance with antiseizure drug treatment, drug or alcohol withdrawal syndromes, and metabolic disturbances
• The four major forms of convulsive status epilepticus are generalized convulsive, focal motor, myoclonic, and tonic
• A clinical diagnosis, confirmed in most cases by the presence on exam of sustained and rhythmic generalized or focal tonic and clonic motor activity lasting for ≥5 minutes and later EEG
• Management: IV Benzos, neuro exam, O2, electrolytes, glucose, cardiac and oximetry monitoring, frequent VS, glucose and thiamine,
Term
Multiple Sclerosis
Definition
• Most common immune-mediated inflammatory demyelinating disease of the central nervous system
• Most common phenotypes are relapsing and progressive disease
• A clinically isolated syndrome (CIS) is the first attack of a disease compatible with MS that exhibits characteristics of inflammatory demyelination but has yet to fulfill MS diagnostic criteria. If untreated, many if not most patients with CIS will eventually be diagnosed with MS
Term
Relapsing-remitting multiple sclerosis (RRMS)
Definition
is characterized by clearly defined relapses with full recovery or with sequelae and residual deficit upon recovery. There is no or minimal disease progression during the periods between disease relapses. This type of MS accounts for approximately 85 to 90 percent of MS cases at onset
Term
Primaryprogressivemultiplesclerosis(PPMS)
Definition
is characterized by disease progression from onset with occasional plateaus and temporary minor improvements allowed; acute attacks may also occur
• Symptoms:sensorydeficitsinlimbs,visualloss, diplopia, gait disturbance, motor problems, balance problems, sensory in face deficits, Lhermitte sign, vertigo, bladder problems, limb ataxia, pain
• Management:MRI,immunemodulatingtreatments: Ocrelizumab, Azathioprine, Cladribine; Glucocorticoids, cyclophosphamide, cyclosporine, Methotrexate, interferons, plasma exchange
Term
Cerebral Palsy
Definition
• Refers to a heterogeneous group of conditions involving permanent nonprogressive central motor dysfunction that affect muscle tone, posture, and movement
• These conditions are due to abnormalities of the developing fetal or infantile brain resulting from a variety of causes
• The most commonly identified prenatal risk factors for CP are prematurity and/or low BW. Other commonly associated factors include congenital abnormalities, multiple pregnancy, placental pathology, and intrauterine infection
• Signs/Symptoms: motor impairment, altered sensation or perception, intellectual disability, communication and behavioral difficulties, seizure disorders, visual and hearing impairment, and speech disorders, growth failure, pulmonary disease, orthopedic problems (joint subluxations and dislocations and hip dysplasia), osteopenia, urinary disorders, sleep disturbance, and sialorrhea
• Signs of CP in infancy and early childhood include neurobehavioral abnormalities (excessive docility or irritability), abnormal tone, abnormal posture, persistence of primitive reflexes, and failure to achieve motor milestones
• After age 18 to 24 months, signs and symptoms generally point to a specific subtype of CP
Term
Spastic CP
Definition
includes spastic diplegia, spastic hemiplegia, and spastic quadriplegia. Spastic CP is an upper motor neuron syndrome, which includes spastic hypertonia, hyperreflexia, extensor plantar responses, and clonus. Affected patients also have slow effortful voluntary movements, impaired fine- motor function, difficulty in isolating individual movements, and fatigability
Term
Dyskinetic CP
Definition
is characterized by involuntary movements of athetosis, chorea, and dystonia.
Term
Ataxic CP
Definition
is characterized by ataxic movements and speech, usually associated with widespread disorder of motor function. Ataxic CP is rare
Term
Cerebral Palsy
• Preventativemeasures and mngmnt
Definition
Provisionofroutineprenatal care, including measures to reduce the likelihood of preterm birth, mag sulfate for women at risk for preterm delivery, supportive therapy for neonates at risk of neurologic injury, therapeutic hypothermia for term or late preterm infants with neonatal asphyxia and/or encephalopathy
• Management:collaborativeapproachfrom multidisciplinary team, botulinum toxin for spasticity and hyperreflexia, PT for disorders of hip subluxation/dislocation, PT/OT for ADL’s, electrical stimulation to increase muscle strength, G-tubes to reduce risk of aspiration, nutrition consults, surgical intervention for refractory drooling, survival and motor function in children with CP is highly variable
Term
Concussion
Definition
• Also known as mild traumatic brain injury
• May result after blunt force or acceleration/deceleration head injury
• Most obvious when patient has a brief LOC or demonstrates confusion or amnesia
• Use the Standardized Assessment of Concussion (SAC) scale
• Patients who have suffered loss of consciousness or have persistent symptoms should be referred to an emergency department
• Management: CT/CTA, observe for 24 hours, follow up imaging with MRI if neuro status is deteriorating,
Term
Post-concussion Syndrome
Definition
• A common constellation of symptoms reported by patients after mild traumatic brain injury (TBI)
• Symptoms: headache, dizziness, cognitive impairment, and psychological symptoms
• Women and older patients appear to be more susceptible
• Management: MRI, neuropsych testing, symptomatic treatment: migraine meds, analgesics, counseling, psychotropic medications, education and reassurance; most recover quickly
Term
ALS Amyotrophiclateralsclerosis
Definition
• Arelentlesslyprogressiveneurodegenerative disorder that causes muscle weakness, disability, and eventually death, with a median survival of three to five years.
• ALSisalsoknownbytheeponym"LouGehrig's Disease," after the famous baseball player who was affected with the disorder.
• Motorneurondisease(MND)isthepreferredtermin the United Kingdom, but in the United States ALS and MND are sometimes used interchangeably.
• Characterizedbymotorneurondegenerationand death with gliosis replacing lost neurons.
• Corticalmotorcells(pyramidalandBetzcells) disappear, leading to retrograde axonal loss and gliosis in the corticospinal tract.
• Thespinalcordbecomesatrophic.
• Theventralrootsbecomethin,andthereisalossof large myelinated fibers in motor nerves.
• Theaffectedmusclesshowdenervationatrophywith evidence of reinnervation such as fiber type grouping.
• Intracellularinclusionsindegeneratingneuronsand glia are frequent neuropathological findings of ALS
• Classified as
sporadic or familial
• Risk factors:
age and family history
• Management:EMG,NCS,electrodiagnostics, Repetitive nerve stimulation, single fiber EMG, transcranial magnetic stimulation
• ObjectiveTests:MRI,CK-elevated,CSF,genetic testing if thought to be familial, muscle biopsy
• Diseasemodifyingtreatments-Riluzole,Edaravone; however, mostly symptomatic treatments managing dysarthria, dyspnea, fatigue, muscle spasms, spasticity, weakness, sialorrhea, thick mucus, pain, sleep disturbances, psychosocial aspects, and pseudobulbar affect (emotional lability or emotional incontinence)
Term
Carpal Tunnel Syndrome
Definition
• Refers to the complex of symptoms and signs brought on by compression of the median nerve as it travels through the carpal tunnel.
• Symptoms: pain and paresthesia, and less commonly weakness, in the median nerve distribution, worse at night, waking patient up
• Risk factors: obesity, female, genetic predisposition, Coexisting conditions (diabetes, pregnancy, rheumatoid arthritis, hypothyroidism, connective tissue diseases, preexisting median mononeuropathy), Aromatase inhibitor use, repetitive hand/wrist motion
• Includes the median nerve territory, with involvement of the first three digits and the radial half of the fourth digit
• Physical exam: flexing and extending make it worse, Phalen’s/Tinel’s sign, manual carpal compression
• Management: NCS, wrist splinting, glucocorticoids, surgical decompression
Term
Guillain-Barre Syndrome
Definition
• GBS is a heterogeneous condition with several variant forms.
• Most often, presents as an acute, monophasic paralyzing illness provoked by a preceding infection.
• GBS is thought to result from an immune response to a preceding infection that cross-reacts with peripheral nerve components because of molecular mimicry. The immune response can be directed towards the myelin or the axon of peripheral nerve, resulting in demyelinating and axonal forms of GBS
• Campylobacter jejuni infection is the most commonly identified precipitant of GBS. Cytomegalovirus, Epstein-Barr virus, human immunodeficiency virus (HIV), and Zika virus have also been associated with GBS
• A small percentage of patients develop GBS after another triggering event such as immunization, surgery, trauma, and bone- marrow transplantation
• Symptoms: progressive, fairly symmetric muscle weakness accompanied by absent or depressed deep tendon reflexes; weakness can vary from mild difficulty with walking to nearly complete paralysis of all extremity, facial, respiratory, and bulbar muscles; weakness usually starts in the legs, but it begins in the arms or facial muscles;
• Severe respiratory muscle weakness necessitating ventilatory support; Facial nerve palsies; Oculomotor weakness; Decreased or absent reflexes in affected arms or legs; Paresthesias in the hands and feet accompany the weakness; Pain due to nerve root inflammation, typically located in the back and extremities; SIADH, dysautonomia (diarrhea/constipation, hyponatremia, bradycardia, urinary retention, tachycardia, reversible cardiomyopathy, and Horner Syndrome)
• GBS is a heterogeneous syndrome with several variant forms. Acute inflammatory demyelinating polyneuropathy (AIDP) is the most common variant of GBS in North America, Europe, and most of the developed world. The Miller Fisher syndrome (MFS) is a GBS variant characterized by ophthalmoplegia with ataxia and areflexia.
• Axonal forms of GBS include acute motor axonal neuropathy (AMAN), most common in Japan and China, and acute motor and sensory axonal neuropathy (AMSAN).

• Management:history,CSFanalysis-anincreasedCSF protein with a normal CSF white blood cell count (albuminocytologic dissociation), serum IgG antibodies to GQ1b is useful for the diagnosis of MFS, EMG, NCS, supportive care, respiratory & cardiac monitoring, vent support, pain management, plasma exchange, IVIG
• AtoneyearafteronsetandtreatmentofGBS,full recovery of motor strength occurs in about 60 percent of patients, while severe motor problems persist in about 14 percent. Approximately 5 to 10 percent of patients have a prolonged course with very delayed and incomplete recovery, and 3 to 7 percent die despite intensive care.
Term
Spinal Cord Injury
Definition
• Traumatic spinal cord injury (TSCI) is a problem that largely affects young male adults as a consequence of motor vehicle accidents, falls, or violence
• The mechanisms surrounding injury to the spinal cord itself are often discussed in terms of primary and secondary injury.
• The primary injury refers to the immediate effect of trauma which includes forces of compression, contusion, and shear injury to the spinal cord.
• A secondary, progressive mechanism of cord injury usually follows, beginning within minutes and evolving over several hours after injury; as a result of these secondary processes, spinal cord edema develops within hours of injury
• Injury may result in complete cord injury, incomplete injury, central cord syndrome, anterior cord syndrome (also known as Beck's syndrome or anterior spinal
artery syndrome is a clinical subset of
spinal cord injury syndromes, due to ischemia/infarction of the anterior two-thirds of the spinal cord), transient paralysis and spinal shock
• Management: ABCDE’s, X-rays, CT, MRI, myelography, DVT prophylaxis, steroids controversial, surgery
Term
Herniated Discs
Definition
• The discs that sit between the vertebrae have a tough outer shell and jelly-like center. The outer shell of the discs can sometimes break open, spilling the jelly material inside.
• Herniated discs can cause symptoms, because the jelly material that spills out of them can irritate nearby nerves. Plus, the disc itself can bulge and press on nerves
• Symptoms: pain, numbness, or tingling
• Management: MRI, pain management, injections, PT, chiro, acupuncture, Rolfing, massage
Term
Spinal Cord Disease
Definition
• There are 31 spinal cord segments, each with a pair of ventral (anterior) and dorsal (posterior) spinal nerve roots, which mediate motor and sensory function, respectively.
• The ventral and dorsal nerve roots combine on each side to form the spinal nerves as they exit from the vertebral column through the neuroforamina
Term
Cauda Equina Syndrome
Definition
• The cauda equina is a bundle of nerves that spread out from the bottom of the spinal cord
• Symptoms: Pain, numbness, or tingling in the lower back and spreading down 1 or both legs, leg weakness or a problem called "foot drop," problems with bowel or bladder control, problems with sex

• Causes: anything that presses on or irritate the nerves in the cauda equina: herniated disc, infection or inflammation, cancer, spinal stenosis
Term
Spinal Cord Tumors
Definition
• May occur within or adjacent to the spinal cord. They are considered to be intraaxial in location and can be either primary or metastatic.
• May be classified according to their anatomic location
– Intramedullary: either ependymomas or astrocytomas
– Intradural-extramedullary: arising within the dura but outside the actual spinal cord are termed intradural- extramedullary. The most common tumors in this group are meningiomas and nerve sheath tumors
– Extradural: often arise in the vertebral bodies
• Symptoms: local and distal effects; The most frequent local effect is pain that causes nocturnal awakening. Patients often describe this pain as a gnawing and unremitting
• Neurologic dysfunction distal to the lesion is due to interruption of ascending and descending spinal cord pathways.
• The most common sequelae are sensory dysesthesias and muscular weakness, especially of the iliopsoas musculature. Patients often report progressive difficulty in ambulation. Severe distal sensory loss and sphincter dysfunction also may occur. Although neurologic manifestations may begin unilaterally, they can progress to involve both sides of the spinal cord and thereby produce bilateral symptoms and signs
• Management: MRI, surgery, RT, chemo.
Term
Post-Herpetic Neuralgia
Definition
• Varicella-zostervirus(VZV)isthecausativeagentof varicella, or "chickenpox," and herpes zoster, or "shingles." Most cases of acute herpes zoster are self- limited, although the pain can cause significant suffering, particularly in older adults.
• Symptoms:mayinterferewithsleep,appetite,orsexual function; pain for months to years after the resolution of the rash; burning, sharp, stabbing pain-constant or intermittent
• Thoracic(especiallyT4toT6),cervical,andtrigeminal nerves are most commonly affected
• Management:tricyclics,gabapentin,pregabalin,topical capsaicin, intrathecal glucocorticoid injections, opioids avoided due to abuse
Term
Brain Tumors
Definition
• Brain tumors are a diverse group of neoplasms arising from different cells within the CNS or from systemic cancers that have metastasized to the CNS.
• Systemic cancers most likely to metastasize to the CNS include lung cancer, melanoma, and breast cancer.
• Primary brain tumors include a number of histologic types with markedly different tumor growth rates
• Thefrequencyofvarioustumortypesandgrades varies by age group. In adolescents and young adults, primary brain tumors are more common than metastatic tumors, and among primary brain tumors, low-grade gliomas predominate
• Morethan120typesofbrainandCNStumors
• Inadultsabovetheageof30to40years,metastatic brain tumors become increasingly prevalent, accounting for more than half of all brain tumors.
• Glioblastomaisthemostcommonmalignantprimary brain tumor in adults, with a median age at diagnosis of 64 years
• Symptomsgeneralized:headaches,seizures, nausea/vomiting, depressed level of consciousness, neurocognitive dysfunction
• Symptomsfocal:seizures,weakness,sensoryloss, aphasia, visual spatial dysfunction
• Headachesareusuallydullandconstant,bifrontal and worse on same side as tumor; Patients may report worsening of headache after a change in body position, such as bending over, or with maneuvers that raise intrathoracic pressure, such as coughing, sneezing, or the Valsalva maneuver; morning nausea and vomiting, headaches may awaken patient from sleep
• Management: history and neurologic examination and brain MRI with contrast
• A CT of the chest and abdomen should be performed when metastatic disease is suspected.
• Unless imaging features are atypical, additional testing is often not necessary prior to diagnostic biopsy or resection
• Glucocorticoids are used to decrease peritumoral edema and elevated ICP in patients with severe symptoms or threatened herniation.
• Steroids are not necessary in patients with minimal symptoms and should be avoided when there is high clinical suspicion for lymphoma
• Accurate diagnosis of a brain tumor requires an adequate tissue sample for histopathologic and molecular analysis. This may be obtained by stereotactic biopsy or open surgery.
• Procedure selection is individualized based on suspected tumor type and grade, location, and operability
Term
Encephalitis Or Meningitis
Definition
• The presence or absence of normal brain function is the important distinguishing feature between encephalitis and meningitis.
• Symptoms with meningitis may be uncomfortable, lethargic, or distracted by headache, but their cerebral function remains normal. Seizures and postictal states still may occur; photophobia, nuchal rigidity
• Aseptic Meningitis starts off with fever, headache, N/V, photophobia, stiff neck
Term
Encephalitis
Definition
• Symptoms in encephalitis: abnormalities in brain function are a differentiating feature, including altered mental status, motor or sensory deficits, altered behavior and personality changes, and speech or movement disorders, hemiparesis, flaccid paralysis, and paresthesias, exaggerated deep tendon/or pathological reflexes, seizures. Patients are confused, agitated or obtunded
• Types: viral and post-infectious (after infection has resolved) (also called acute disseminated encephalomyelitis, or ADEM)
• Viruses: Arboviruses (eastern equine, western equine, St. Louis {St. Louis encephalitis virus infection occurs in North America, predominantly throughout the Midwest and South in the United States}, Venezuelan equine encephalitis, Zika, and West Nile virus) cause disease when mosquitoes are active; herpes simplex virus can occur at any time.
• In contrast, walking in woods or marshy areas with high tick populations might suggest tick-borne encephalitides seen in Eastern Europe, Colorado tick fever (western United States), Powassan virus (transmitted by Ixodes ticks in eastern Canada and the north central and northeastern United States), or nonviral etiologies, such as Lyme disease or Rocky Mountain spotted fever
• West Nile virus has been by far the most common cause of proven viral encephalitis in the United States
• Nipah virus encephalitis, which appeared in Malaysia and Singapore and is now present in Bangladesh, may be associated with exposure to pigs or bats
• Avian influenza infection was associated with encephalitis in two patients from Vietnam; the source of exposure was unclear but both children swam in a canal that was frequented by ducks
• Lymphocytic choriomeningitis virus (LCMV) is a human-acquired zoonosis caused by a rodent-borne arenavirus, which can be transmitted through exposure to secretions of mice, rats, and hamsters.
• Encephalitis resulting from a bornavirus was reported in three German breeders of variegated squirrels; the virus was also detected in a squirrel from the breeding population of one of the patients
• Prior history of an animal exposure or bite may also suggest the possibility of rabies encephalitis
• Parotitis strongly suggests the diagnosis of mumps encephalitis in an unvaccinated patient with mental status changes.
• Flaccid paralysis that evolves into an encephalitis strongly suggests the possibility of West Nile virus infection-it has been misdiagnosed as Guillain-Barré syndrome. A maculopapular rash is also seen in approximately half of patients with this infection and is not expected in other viral encephalitides.
• Tremors of the eyelids, tongue, lips, and extremities may suggest the possibility of St. Louis encephalitis or West Nile encephalitis in the appropriate geographic location or travel history.
• Findings of hydrophobia, aerophobia, pharyngeal spasms, and hyperactivity suggest encephalitic rabies. Atypical presentations of rabies include seizures, cranial nerve palsies, and myoclonus.
• Grouped vesicles in a dermatomal pattern may suggest varicella zoster virus (VZV)
• Management: CT, MRI-not always conclusive, IGM antibody on CSF, viral cultures of CSF, CSF PCR for HSV1, Serologic studies for West Nile, mumps & EBV, brain biopsy.
• Start acyclovir 10mg/kg tid IV while awaiting results
Term
Meningitis: Bacterial
Definition
• An inflammatory disease of the leptomeninges, the tissues surrounding the brain and spinal cord, and is defined by an abnormal number of white blood cells in the cerebrospinal fluid (CSF).
• The meninges consist of three parts: the pia, arachnoid, and dura maters.
• Bacterial meningitis reflects infection of the arachnoid mater and the CSF in both the subarachnoid space and the cerebral ventricles.
• Symptoms: most common triad: fever, nuchal rigidity, and a change in mental status
Term
Meningitis
Definition
• Labs: 2 sets of Blood cultures
• Lumbar puncture
• CT scan if immunocompromised, history of CNS disease, new onset seizure, papilledema, abnormal LOC, focal neuro deficit
• Gram stain and culture help differentiate between bacterial and viral infection
Term
Meningitis Management
Definition
• Management: empiric antibiotics until cultures return, discontinue if negative for bacteria; antivirals
• Counsel: hygiene; vaccines-polio, influenza, rabies; personal protection
• Clarification: aseptic meningitis refers to patients who have clinical and laboratory evidence for meningeal inflammation with negative routine bacterial cultures. Viruses cause most aseptic meningitis cases, which is why the condition is also known as viral meningitis
Term
Bell’s Palsy
Definition
• Presentswiththesuddenonset (usually over hours) of unilateral facial paralysis.
• Commonfindings:eyebrow sagging, inability to close the eye, disappearance of the nasolabial fold, and the mouth drawn to the unaffected side
• Associatedmanifestationsmay include decreased tearing, hyperacusis, and/or loss of taste sensation on the anterior two- thirds of the tongue.
• Facial nerve palsy may be caused by a variety of disorders that can be confused with Bell's palsy: Herpes zoster, OM, Lyme disease, Guillain-Barre, HIV, Sarcoidosis, Sjogren syndrome, Tumor, Stroke, Melkersson-Rosenthal Syndrome
• Herpes simplex virus activation is the likely cause in most cases for Bell’s Palsy
• Diagnosis:
– There is a diffuse facial nerve involvement manifested by paralysis of the facial muscles, with or without loss of taste on the anterior two-thirds of the tongue or altered secretion of the lacrimal and salivary glands
– Onset is acute, over a day or two; the course is progressive, reaching maximal
clinical weakness/paralysis within three weeks or less from the first day of visible weakness; and recovery or some degree of function is present within six months
• Treatment: Glucocorticoid, antiviral therapy, physical therapy, artificial tears, surgical decompression
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