1. Signs, symptoms and etiology of AIDs dementia complex (ADC)*

ADC, also known as HIV-associated dementia complex (HAD), is characterized by cognitive, motor, & behavioral features in adults, which usu develops in advanced AIDS when CD4⁺ lymphocyte counts fall below 200 cells/mm³.  Pts often present w/insidious onset of reduced work productivity, poor concentration, mental slowness, decreased libido, and forgetfulness. Apathy & w/drawal from hobbies or social activities are common & must be differentiated from depression. Rare features include sleep disturbances, psychosis (with mania), and seizures. Motor problems include imbalance, clumsiness, and weakness. Early sxs are subtle and may be overlooked. Later, these symptoms evolve into a global dementia w/memory loss & language impairment. This can lead to a vegetative state. The neuropsychological examination reveals features more suggestive of subcortical dementia, as seen in Parkinson disease, than of cortical dementia of the Alzheimer type. Early on, neuropsychological examination reveals psychomotor slowing, memory loss, and word-finding difficulties. Subcortical involvement with impaired retrieval and manipulation of acquired knowledge is prominent. Later, severe psychomotor retardation and language impairment become obvious, leading to akinetic mutism. Neurologic examination in adults early on may be NL or reveal subtle impairment of rapid limb and eye movements. Later, frontal lobe release signs, tremor, hyperreflexia, clonus, spasticity, weakness, and poor coordination develop. These signs may reflect a concomitant vacuolar myelopathy. The terminal stage of ADC, after progression over several months, includes severe psychomotor retardation and dementia, apraxia, paraparesis, and akinetic mutism. Death ensues within a few months of reaching this stage. Seizures are rare and warrant exclusion of other conditions. Price and Brew in 1988 outlined a clinical staging of ADC: 

Stage 0 (normal): Mental and motor functions are normal. 

Stage 0.5 (equivocal/subclinical): sxs may be absent, minimal, or equivocal, w/no impairment of work or performance of activities of daily living (ADL). Mild signs (snout response, slowed ocular or extremity movements) may be present. Gait and strength are normal.

Stage 1 (mild): The patient is able to perform all but the more demanding aspects of work or ADL but has unequivocal evidence of functional, intellectual, or motor impairment. Signs or symptoms may include diminished performance on neuropsychological testing. Patient can walk without assistance.

Stage 2 (moderate): The patient is able to perform basic activities of self-care but cannot work or maintain the more demanding aspects of daily life. The patient is ambulatory but may require a single prop.

Stage 3 (severe): The patient has major intellectual incapacity (cannot follow news or personal events, cannot sustain complex conversation, considerable slowing of all outputs). Motor disability precludes walking unassisted (ie, without walker or personal support); walking usually slowed and accompanied by clumsiness of arms.

Stage 4 (end stage): The patient is in a nearly vegetative state. Intellectual and social comprehension and output are at a rudimentary level. The patient is nearly or absolutely mute. The patient is paraparetic or paraplegic with urinary and fecal incontinence. 

2. Physiology/pathophysiology of sleep*

Physiology:
- Sleep is an active process under the control of the RAS
- RAS sends info to higher brain areas to maintain wakefulness and arousal in a functional process (not anatomical)

- Brainstem inhibitory signals (GABA) arrive at neurons in RAS and inhibit RAS firing, ending conscious behavior

- This occurs 2° to serotonin release from reticular formation

- RAS is stimulated by all sensory stimulation, including pain (can wake you from sleep)

Pathophysiology: 

- sleep disorders include sleep apneas, narcolepsy, insomnia, restless leg syndrome, and circadian rhythm disorders

- Mass lesions in the brain stem cause severe alterations in level of consciousness, such as coma, due to effects on RAS

- B/L damage to RAS of the midbrain may lead to death or coma

- Lesions in RAS have been found in brains of people who have post-polio syndrome, and abnormal activity in RAS has been found in people with chronic fatigue syndrome. indicating likelihood that damage to RAS can cause fatigue seen in these dzs

3a. Signs/symptoms of Epidural hematoma*

pg.918 - headache, confusion,somnolence, seizures, & focla deficits occur severa lhours after injury & lead to coma, respiratory depression,& death unless txt by surgical evacuation

3b. Signs/symptoms of Subdural hematoma*

pg.918 - acute: headache, confusion,somnolence, seizures & focal deficits occur (takes longer than epidural hemorrhage) after injury & lead to coma, respiratory depression,& death unless txt by surgical evacuation. 

chronic: the relationship of a chroinc hemorrhage is not always clear. In many elderly pts, there is no h/o trauma but in other cases a head injury, often trivial, precedes the onset of sxs by several weeks. SXS: mental status changes: slowness, drowsiness, HA, confusion, memory disturbances, personality changes, or even dementia. Focal neuro deficits: hemiparesis/hemisensory disturbance (are less common).

3c. Signs/symptoms of Cerebral ischemia*

Brain ischemia, aka cerebral ischemia, is insufficient bld flw to the brain to meet metabolic demand. This leads to poor oxygen supply or cerebral hypoxia & thus to death of brain tissue or cerebral infarction /ischemic stroke. It is a sub-type of stroke along w/subarachnoid hemorrhage & intracerebral hemorrhage. 

3d. Signs/symptoms of Basalar skull fracture*

pg 918 - bruising around the orbit (racoon sign), bld in the external auditory canal (Battle sign), and leakage of cerebrospinal fluid (which can be identified by its glucose content) fromt the ear & nose. Cn palsies (esp 1-5,7 & 8) may be presents. 

4. Symptoms of post-concussive syndrome*

Although no universally accepted definition of pcs exists, most define it as the development of at least 3 of the following: HA, dizziness, fatigue, irritability, impaired memory & concentration, insomnia & lowered tolerance for noise/light. Some definine it as sxs of at least 3 months' duration, while others define it as sxs appearing w/the 1st wk. Persistent postconcussive syndrome (PPCS) is generally defined as sxs lasting > 6 months. 

-HA: the most common sxs. 

-Cranial nerve sxs: dizziness (2nd most common sxs), vertigo, nausea, tinnitus, blurry vision, hearing loss, diplopia, diminished sense of taste and smell, light and noise sensitivity

-Psychological and neurovegetative problems: anxiety, irritability, depression, sleep disturbance, change in appetite, decreased libido, fatigue, personality change

-Cognitive impairment: memory impairment, diminished concentration & attention, delayed info processing & reaction time

epressed affect, decreased ability to smell/taste, neurasthenia or hyperesthesia (nondermatomal distribution), cognitive deficits-P/E: In general,P/E is normal. The pt may exhibit subtle neurologic findings, but objective focal motor deficits should raise a concern about an undiagnosed intracranial bleed. 

5a. S/S and imaging findings in Multiple sclerosis*

Must have 2 episodes (>24hrs) of CNS lesions (white plaques) on MRI in 2 separate locations to fulfil MS dx. Sxs: Optic neuritis, sensory disturbances (tingling, burning, tightness, numbness, balance & gait abnormalities, bladder urgency/frequency), motor dysfxn, Suspect if MS if: Lhermitte sign, diurnal fatigue, postpartum sxs onset, sxs induced by heat or exercise, trigeminal neuralgia in pts<50, useless hand, dbl vision, afferent pupillary defect, imparied color vision, optic nerve pallor, central scotoma, lessened visual acuity. Dysmetria is also common.

5b. S/S and imaging findings in Syringomyelia*

Pg 920 - Characteristic clinical pictures, w/segmental atrophy & areflexia & loss of pain & temp appreciation in a "cape" distribution owing to the destruction of fibers crossing in front of the central canal. Throacic kyphoscoliosis is usu present. W/progression, involvement of the long motor & sensory tracts occurs as well, so that a pyramidal & sensory deficit develpos in the legs. Upward extension of the cavitiation (syringobulbia) leads to dysfxn of the lower brainstem & thus to bulbar palsy, nystagmus, sensory imparing over one of both sides of the face. 

5c. S/S and imaging findings in Cervical spondylosis*

- Results form chronic cervical disk degeneration, with disk herniation, 2° calcification, and associated osteophytic outgrowths

- one or more cervical nerve roots may be compressed, stretched or angulated

- myelopathy may develop as a result of compression, vascular insufficiency or recurrent minor trauma. Presents with spastic paraparesis, and possible posterior column or spinothalamic sensory deficits in the legs

- Pts may present with: neck pain, restricted head movement, occipital HA, radicular pain and other sensory disturbances in the arms, weakness of arms or legs

- Exam: limited ROM (lateral flexion and rotation), unilateral or b/l segmental pattern of weakness or dermatomal sensory loss in UE, depressed DTRs of affected roots

- C5 & C6 most often involved (affects deltoids, supra  & infraspinatus, biceps & brachioradialis, muscles &  DTRs affected) 

- Xray: osteophyte formation, narrowing of disk spaces, adn encroachment on the intervertebral foramina

- CT or MRI: exclude other structural causes of myelopathy 

5d. S/S and imaging findings in Paget’s disease*

5e. S/S and imaging findings in Pott’s disease*

6. CSF findings for a subarachnoid hemorrhage*

RBCs or xanthochromia (ylw colored csf) - the yellow appearance is d/t RBCs entering the CSF during the bleeding. The cells are eventually destroyed by the body, releasing their oxygen-carrying molecule heme, which is degraded by enzymes into the yellow-green pigment bilirubin.

7a. S/S of Generalized seizure disorder*

Diffuse involvement of brain at onset

There are 5 categories of generalized seizures:

1. Absence (petit mal)
2. Myoclonic
3. Tonic-clonic (grand mal)
4. Tonic
5. Atonic

7b. S/S of Partial seizure disorder*

• Involvement of only restrited part of brain, may become 2° generalized
• Patients do not become unconscious unless 2° generalization occurs (but consciousness may be altered in complex)
• 2 types: simple & complex

7c. S/S of Attention deficit disorder*

A chronic condition that affects millions of children and often persists into adulthood. 

- includes a combination of inattention and hyperactive impulse problems, though many children tend toward one category over the other

- S/S of inattention: including difficulty sustaining attention esp to details, poor performance in school, seems not to listen even when spoken directly to, organizational problems, easily distracted, forgetful, daydream

- S/S of hyperactivity and impulsive behavior: fidgets or squirms frequently, restlessness, excessive talking, impatient

- ADHD patients often have low self-esteem  troubled relationships

7d. S/S of Complex partial seizure disorder*

• Changes in alertness
• Changes in level of consciousness, but not lost
• Partial amnesia
• Automatic, involuntary, stereotyped, repetitive, facial, lingual and oropharyngeal movements and gestures often present. Automatisms
• Pts can perform simple motor tasks and may even walk during the seizure
• Usually originate in Temporal lobe (but may start in inferior frontal lobe and spread to temporal lobe)
• Have a focal orign
• EEG: interictal: spike discharges in 1 or both temporal lobes. Ictal: usually abnormal (recurrent ictal spikes or rhythmic activity). 

7e. S/S of Simple motor seizure disorder*

• A simple partial seizure with localized motor activity. Originates in the frontal lobe.
• There may be spasm or clonus (jerking) of one muscle or a muscle group and this may remain localized or it may subsequently spread to adjacent muscles as a Jacksonian seizure. (on same side of corpus callosum)
• No change in level of consciousness
• preserved responsiveness to external environment

8a. S/S of Jacksonian epilepsy*

Jacksonian epilepsy is a neurological disorder characterized by simple partial seizures that usually occur in only one side of the body. Like other forms of epilepsy, Jacksonian epilepsy occurs due to spurts of irregular or elevated electrical impulses in the brain that cause neurons to fire at an extremely rapid rate. However, this mild form of epilepsy is unique in that excessive neural activity begins in the general motor cortex region of the brain, producing a contralateral effect. This means that seizure activity occurs on the side of the body that is controlled by the opposite side of the brain in which electrical impulses have momentarily gone awry.Another unique characteristic of Jacksonian epilepsy is that abnormal neural firing localized to the motor cortex tends to trigger a cascade of partial seizures in associated muscles in a predictable succession. For example, the first sign of seizure may be experienced as twitching or a tingling sensation in a finger, a big toe, or the corner of the mouth, which then advances to the entire hand, foot, or surrounding facial muscles, respectively. This progression of seizure activity is described as a Jacksonian march.

8b. S/S of temporal lobe seizure*

• Most common type is complex partial seizures
• May be preceded by an aura
• pts usually remain partially conscious
• duration: 30 sec-2 min
• characteristic s/s: loss of awareness of surroundings, staring, lip smacking, repeated swallowing or chewing, automatisms 
• Post-itcal: brief pd of confusion and difficulty speaking, amnesia of events
• usually begin deep in temporal lobe (limbic system, which controls emotion and memory)
• may evolve into a generalized tonic-clonic seizure

9. dosing strategy for epilepsy*

• Begin with one drug, gradually increase the dose until seizures are controlled or side effects prevent further increases
• if seizures are not controlled then increase dose gradually. 
• Use laboratory findings to guide dose adjustments, but don't treat serum levels, treat the patient. many pts require levels that exceed the therapeutic range but tolerate these without ill effects
• if 1st choice not effective then try a 2nd line drug
• try to overlap meds to stay on monotherapy (taper off 1st drug, while tapering on new drug)
• avoid polypharmacy if possible (refer to center)
• #1 cause of lower than expected drug [ ] is non compliance

10a. Etiology of febrile seizure*

• Usually triggered by fevers from: ear infections, roseola (fever adn rash caused by several different viruses), upper respiratory infection caused by virus
• Viral illnesses are the predominant cause of febrile seizures
• Tends to occur in families
• increased risk with previous occurance

• 2-4% of all children will have at least 1 febrile seizure
• usually brief, generalized tonic clonic attacks
• simple febrile is lasts few seconds to 10 minutes. Complex febrile if lasts longer than 15 min
• may present mild as the child's eyes rolling or limbs stiffening
• LP: negative

11b. S/S of Lacunar stroke*

contralateral pure motor or pure sensory deficit, ipsilateral ataxia w/crural paresis, dysarthria w/hand clumsiness. Seen in "step-wise" progression.

12. Various visual field loses and their causes (centrally)*

Monocular: lesion anterior to chiasm

Bitemporal hemianopia: chiasmal

Homonymous hemianopia: lesion posterior to chiasm

Temporal: Contralateral homonymous hemianopsia
Parietal: Contralateral homonymous hemianopsia
Occipital: crossed homonymous hemianopsia or partial field deficit if left sided or bilateral: visual agnosia for objects/colors
 with irritative lesions: unformed visual hallucinations
Bilateral: cortical blindness
Pituitary: bitemporal hemianopsia (most common cause)

Craniopharyngioma: bitemporal hemianopsia

13a. S/S and imaging findings of Astrocytoma*

Presents similar to glioblastoma mulitforme (nonspecific complaints & increased ICP: HA, N/V, malaise) but course is more protracted, often over several years. Cerebellar lesions may have a more benign course (intellecutal fxning is preserved). Total removal usu not possible unless it is located in the cerebellum. Tumor not sensitive to radiation. 

Seen most frequently in kids; generally arises from roof of 4th ventricle & leads to increased ICP accompanied by brainstem & cerebellar signs. May seed subarachnoid space.  Surgery plus chemo & radiation needed.

Slow-growing from myelin creating cells of CNS; usu arises in cerebral hemisphere in adults. Calcification may be visible on skull xray.  Surgical txt is usu successful b/c tumor is well circumscribed. Radiation & chemo may be used if tumor has malignant features. 

Presents during childhood w/cranial nerve palsies & then w/long tract signs in the limbs (spastic paralysis). Sxs of increased ICP occur late (so N/V do too!) Tumor is inoperable - txt is by irradiation & shunting. May present as spinal cord lesion.

14. Clinical definition of a cerebral concussion* 

pg 918 - bruising on side of impact (coup injury) or contralaterally (contrecoup injury).

Sxs:transient loc w/bradycardia, hypotension, respiratory arrest for a few seconds followed by retrograde 7 posttraumatic amnesia. Occasionally followed by transient neuro deficit. 

15a. S/S of MCA stroke (ischemic and hemorrhagic)*

mca: contralateral hemiplegia, hemiparesis, hemisensory loss, homonymous hemianopia, eyes look twrd lesion. Broca's Aphasia (if on L), drowsiness, stupor, coma d/t swelling. Intracranial hemorrhage should be suspected if neuro status deteriorate qhr. 

aka: Endolymphatic hydrops is distension of the endolympatic compartment of the inner ear caused by syphilis, head trauma or autoimmune dx. SXS: Recurrent attacks of vertigo (30 min-24 hr), fluctuating low freq SN hearing loss, tinnitus (often low tone and "blowing") and aural fullness. Caloric testing reveal loss or impairment of thermally induced nystagmus on the involved side. Sxs associated w/ increased stress. NOTE: these symptoms with out hearing loss suggests migraine associated dizziness

• S/S common to all include HA, fever, sensorial disturbances, neck and back stiffness
• +Kernig (pain in hamstrings when legs extended) and Brudzinski (passive flexion of neck causes spontaneous flexion of lower limbs) signs and CSF abnormalities
• Classic triad: fever, stiff neck and altered mental status (44% sensitivity for bacterial meningitis)
• Nearly all pts with bacterial meningitis have at least 2 of the following symptoms: fever, HA, stiff neck, and altered mental status

• causes ocular histoplasmosis syndorme: due to fungal infection of the retina 
• Can lead to severe, irreversible vision loss
• 1° cause of blindness in adults who have lived in areas where this mold is found in the soil (mississippi-ohio river valley and mid-atlantic states)

16. Causes of recurrent vertigo*

(From HEENT spreadsheet on vertigo)

Central vertigo causes gradual or episodic bouts d/t: Vascular compromise (hemorrhagic or ischemic insults to cerebellum, vestubular nuclei, other connections), MS (episodic vertigo w/unilateral, rapid hearing loss), CNS infection, CNS tumors (unremitting, disabling vertigo & non-fatigable, vertical nystagmus unsuppressed w/visual fixation), CNS trauma (several days but may last <1 mth). 

-Meneire's dx: recurrent attacks of vertigo (30-24hrs)

-Labyrinthitis: acute onset of incapacitating vertigo (days-weeks)

-Benign Paroxysmal positioning vertigo (BVVP):recurrent spells of vertigo (under several min per spell) - ***Most common Dx in patients presenting with vertigo 

-Vestibular Schwannoma (Acoustic  Neuroma): almost never acute vertigo

-Vestibular neuritis - single attack of vertigo (several days-week)  

-Acute peripheral vestubulopathy: Single incapacitating episode of vertigo w/gradual onset (peak in several hours, resolve within 48hrs)

-Migranous vertigo

-Ischemic vertigo syndromes (i.e. Wallenberg syndrome):long lasting vertigo

-Cervical vertigo: cervical proprioceptive dysfxn triggered by neck movements or injury(hyperextendion) or cervical degenerative dx

-

17a. S/S of Perilymph fistula*

(Benign Paroxysmal positioning vertigo) D/t free floating calcium carbonate cyrstals within semicircular canals. SXS: recurrent spells of vertigo (under several min per spell), assoc w/changes in head position, gaze evoked, dix hallpike induced nystagmus that is fatiguable. Brief latency period (10-15 sec) before symptoms start, Acute vertigo subsides within 10-60 sec, pt may remain imbalance for several hours. ***Most common Dx in patients presenting with vertigo

18a. S/S of Muscular dystrophy*

Symptoms vary with the different types of muscular dystrophy. All of the muscles may be affected. Or, only specific groups of muscles may be affected, such as those around the pelvis, shoulder, or face. Sxs include:

-Mental retardation (only present in some types of the condition)

-muscle weakness that slowly gets worse

-Delayed development of muscle motor skills

-Difficulty using one or more muscle groups

-Drooling

-Eyelid drooping (ptosis)

-Frequent falls

-Loss of strength in a muscle or group of muscles as an adult

-Loss in muscle size

-Problems walking (delayed walking)

Duchene's type: psydohypertrophy of muscles, intellectual retardation, skeletal deformaties, muscle contractions, cardiac abnormalities.  The distribution is pelvic, then shoulder girdle; later limb & respiratory. Rapid progression to death w/in 15 years. 

-In both Becker & Duchene serum creatinine is increased.  

18b. S/S of Myasthenia gravis*

Key indicators: fluctuating weakness & muscle fatigue.

-Pt tires w/repeated activites (brushing teeth, combing hair).

-Sustained muscle contraction is hard (holding arms above head).

-Facial weakness: no expression on face, straight smile.  

-Face is symmetric.

-Weakness of ocular muscles: ptosis.

-Transient diplopia, blurred vision, dysconjugate gaze (eye deviation).

-Dysphonia (nasal, slow, labored, soft or indistinct speech).

-Dysphagia (coughs during eating).

-Dypsnea.

-Muscle fatigue decrease and ability to move muscles freely returns after rest period.

-Senstation is intact it is the motor fibers of Cn nerves that are affected. 

18c. S/S of Botulism*

• Begin within 72 hours (usually 12-36 hrs) following ingestion of the toxin and may progress for several days
• Typically: diplopia, ptosis, facial weakness, dysphagia and nasal speech. Followed by: respiratory difficulty and finally by weakness that appears last in limbs. 
• Visual disturbances particularly diplopia, loss of accommodation (causing blurring) ptosis, cranial nerve palsies with impairment of extraocular muscles and fixed dilated pupils are characteristic
• There may be dryness of the mouth, dysphagia, constipation (paralytic ileus) and postural hypotension
• nausea and vomiting may be present (particularly with type E)
• Sensation is preserved, and DTRs are not affected unless involved muscles are very weak
• Temperature remains normal

18d. S/S of Eaton-Lambert syndrome*

Lambert-Eaton myasthenic syndrome (LEMS) is a rare disorder of neuromuscular transmission. It is a presynaptic disorder of neuromuscular transmission in which quantal release of acetylcholine (ACh) is impaired, causing a unique set of clinical characteristics, which include proximal muscle weakness, depressed tendon reflexes, posttetanic potentiation, and autonomic changes. The initial presentation can be similar to that of MG but the progressions of the two diseases have some important differences.

Sxs: (usu insidious over mnths or yrs prior to diagnosis)

-Cancer is present or subsequently discovered in 50-70% of pts 

-The main symptom of LEMS is muscle weakness.LE proximal muscles are affected predominantly. Therefore, patients typically have difficulty rising from a chair, climbing stairs, and walking.

-The oropharyngeal and ocular muscles are affected in about 1/4 of pts (ptosis, diplopia, & dysarthria - but usu not to the same extent/severity as MG).

-Strength improvement after exercise is common in LEMS.

-Many pts have some degree of autonomic dysfunction: dry mouth (usu 1st presenting sxs),

dysphagia, constipation, urinary retention, pupillary constriction, sweating, lacrimation and salivation abnormalities, postural hypotension).

-While respiratory muscle fxn is involved, it is not usu as severe as w/MG

-Symptoms of the underlying cancer, as well as the "B" symptoms of cancer, may be present.

-Reflexes usu reduced or absent. An increase in reflex after contraction is a hallmark of LEMS.

-no fasciculations

PATHO: Lambert-Eaton myasthenic syndrome (LEMS) is the result of an autoimmune process in which antibodies develop to the VGCCs and impair the release of ACh from the presynaptic terminal. The same calcium channels in cell lines are found in SCLC, and they are also inhibited by LEMS-IgG, in both tumor and nontumor cases. In tumor cases, a protein of the calcium channel particles may trigger the autoantibody response, but the stimulus in the nontumor cases is unknown.

18e. S/S of ALS*

Steadily progressive dx (w/o remission) lasts 2-4yrs w/respiratory complications typical cause of death. This dx presents w/both UMN (spacticity, hyperreflexia, Babinski, clonus) & LMN (weakness of legs, hands, proximal area of arms, slurred speech, difficulty swallowing, muscle cramps/wasting, weight loss, paresis of intercostal muscles, foot drop & fasiculating atrophic tongue).

-Sensations remain intact - NO paresthesias.

-Bladder fxn/eye movements not affected, intellctual function preserved.

-Late in dx: claw-hand deformity.

19a. Pathophysiology (including genetics) of Duchenne muscular dystrophy*

Pg.935: A genetic defect on the short arm of the X chromosome (Xp21) has been identified. It is a X-linked recessive dx (primarily affects boys, who inherit the dx through their mothers. Women can be carriers of DMD but usually exhibit no symptoms.) in which the affected gene codes for protein dysptrophin, which is markedly reduced or absent from the muscle of pts. 

Normally the closure of the neural tube occurs around the 23rd and 27th  day after fertilization. However, if something interferes and the tube fails to close properly, a neural tube defect will occur. Medications such as some anticonvulsants,diabetes, having a relative with spina bifida, obesity, and an increased body temperature from fever or external sources such as hot tubs and electric blankets may increase the chances of conception of a baby with a spina bifida. However, most women who give birth to babies with spina bifida have none of these risk factors, and so in spite of much research, it is still unknown what causes the majority of cases.

Extensive evidence from mouse strains with spina bifida indicates that there is sometimes a genetic basis for the condition. In human spina bifida, as with other human diseases such as cancer, hypertension and atherosclerosis (coronary artery disease), spina bifida likely results from the interaction of multiple genes and environmental factors.

Research has shown that lack of folic acid (folate) is a contributing factor in the pathogenesis of neural tube defects, including spina bifida. Supplementation of the mother's diet with folate can reduce the incidence of neural tube defects by about 70 percent, and can also decrease the severity of these defects when they occur. It is unknown how or why folic acid has this effect.

Spina bifida does not follow direct patterns of heredity like muscular dystrophy or haemophilia. Studies show that a woman who has had one child with a neural tube defect such as spina bifida, has about a three percent risk of having another child with a neural tube defect. This risk can be reduced to about 1% if the woman takes high doses (4 mg/day) of folic acid before & during pregnancy. For the general population, low-dose folic acid supplements are advised (0.4 mg/day).

• a paralytic  caused by botulinum toxin
• The toxin of clostridium botulinum prevents the release of acetylcholine at neuromuscular junctions and autonomic synapses
• Naturally occurring botulism occurs in 1 of 3 forms: food-borne, infant (in honey) or wound (typically associated with injection drug use)
• occurs most commonly following the ingestion of contaminated home-canned food
• nothing found about genetics

Autoimmune disorder affecting the neuromuscular jxn where autoantibodies (immunoglobulin G [IgG]) develop against ACh nicotinic postsynaptic receptors. causing an antibody-mediated attack on Ach receptors at NM jxn. This disrupts the transmission & decreases the Ach receptor numbers. Pt have 1/3 as many Ach receptors compared to NL population.

It is thought that there is a viral trigger that causes the body to produce antibodies by the plasma cells, lymph system & thymus gland. These anitbodies have the same peptide sequence in an area as the Ach receptor so in essence, the body gets confused. A large thymus is often present in MG pts.  Cholinergic nerve conduction to striated muscle is impaired by a mechanical blockage of the binding site by antibodies and, ultimately, by destruction of the postsynaptic receptor. Patients become symptomatic once the number of ACh receptors is reduced to approximately 30% of normal

There is a genetic disposition w/higher incidence in people w/other autoimmune dxs (SLE,RA, thyroid).The clinically typical form of autoimmune MG with thymus hyperplasia shows the most reproducible genetic associations, especially with the A1-B8-DR3 (8.1) haplotype of the major histocompatibility complex (MHC). 

Caused by infection with the poliovirus, an enterovirus. The virus spreads by direct person-to-person contact, by contact with infected mucus or phlegm from the nose or mouth, or by contact with infected feces.

The virus enters through the mouth and nose, multiplies in the throat and intestinal tract, and then is absorbed and spread through the blood and lymph system. The time from being infected with the virus to developing symptoms of disease (incubation) ranges from 5 - 35 days (average 7 - 14 days).

Polio virus is a fairly simple virus of the virus family Picornavirdae. The capsid surrounding this virus have surface receptors that help the virus recongnize and bind to target motor neurons in the host's body. 

20. Clinical description of presyncope*

Typically cardiovascular - lack of blood to brain (drop in systemic arterial pressure, failure of cardiac output, diffuse cerebral vasoconstriction). SXS: lightheadedness, generalized weakness, visual blurring, blackout, diaphoresis, SOB, palpitations, pallor, nausea; reproducable sxs w/hyperventilation. 2nd category of dizziness vertigo d/t poor bldflw to the brainstem vestibular nuclei.

21. S/S of post-polio syndrome*

Post-polio syndrome (PPS) is a condition that affects polio survivors years after recovery from an initial acute attack of the poliomyelitis virus. Most often, polio survivors start to experience gradual new weakening in muscles that were previously affected by the polio infection and also in muscles that seemingly were unaffected by the virus.  The most common symptoms include slowly progressive muscle weakness, fatigue (both generalized and muscular), and,a gradual decrease in the size of muscles (muscle atrophy).   Pain from joint degeneration and increasing skeletal deformities such as scoliosis are common may precede the weakness and muscle atrophy. Some individuals experience only minor symptoms while others develop visible muscle weakness and atrophy. Taken from NIH website.

22. Know Glasgow coma scale intimately*

Provides a score from 3-15 to estimate and categorize the outcomes of brain injury on the basis of overall social capability or dependence on others. Lower # = more severe injury & poorer prognosis. Measures motor response, verbal response and eye opening response (add all together to get score): 

Mild (13-15)

Mod disability (9-12): loss of consciousness >30min; impairments which may or may not resolve; rehab 

Severe disability (3-8): Coma, unconscious state; no meaningful response, no voluntary activities

Vegetative state (>3): sleep wake cycles; arousal but no interaction with environ; no localized response to pain

23. Treatment of Wernicke-Korsakoff syndrome*

A lack of vitamin B1 is common in people with alcoholism. It is also common in persons whose bodies do not absorb food properly (malabsorption), such as sometimes occurs after obesity surgery.Korsakoff syndrome, or Korsakoff psychosis, tends to develop as Wernicke's symptoms go away. Wernicke's encephalopathy causes brain damage in lower parts of the brain called the thalamus and hypothalamus. Korsakoff psychosis results from damage to areas of the brain involved with memory.

txt:The goals of treatment are to control symptoms as much as possible and to prevent the disorder from getting worse. Some people may need to stay in the hospital early in the condition to help control symptoms:

- Thiamine  (IM/PO): improves confusion/delirium, difficulties w/vision & eye movement, lack of muscle coordination. It usu doesn't improve loss of memory & intellect. 

-Stop EtOH Eating a well-balanced

-nourishing diet can help 

24b. S/S of Brown-Sequard syndrome*

Pg. 919: A unilateral cord lesion that leads to an ipsilateral motor disturbance w/accompanying impariment of proprioception and contralateral loss of pain & temp appreciation below the level of the lesion.

From Neuro made simple: All sxs are ipsilateral in this syndrome except for the contralateral pain-temp loss below the level of the lesion.  

Brown-Sequard, one of the founders of clinical neurology and neurophysiology, noted in the latter half of the XIXth century, that extramedullary tumors that compress the spinal cord from its lateral or antero-lateral aspect, cause, in the early stages, impairment of pain and temperature sensation on the side CONTRALATERAL to the tumor, and weakness and spasticity on the side IPSILATERAL to the lesion. Since that observation it has been known that damage of the anterolateral system (spinothalamic tract; ALS) in the spinal cord causes impairment or loss of pain and temperature sensation CONTRALATERAL to the lesion, and that damage of the corticospinal tract in the spinal cord (lateral corticospinal tract; LCST) results in upper motor neuron syndrome IPSILATERAL to the lesion. 

24c. S/S of Cauda equine syndrome*

SXS: low back pain (local tenderness to palpation/percussion may be present), pain in the legs (or radiating to the legs) h/o incontinence of urine/stool, saddle paresthesias. Specific historical points to elicit from the patient include the following: unilateral or b/L LE motor and/or sensory abnormality, difficulty starting or stopping a stream of urine that may be followed by frank incontinence first of urine then of stool. poor anal sphincter tone is characteristic of CES

Causes: compression of the cauda d/t: Traumatic injury, Lumbar disk herniation, Abscess, Spinal anesthesia, Spinal epidural hematoma, Tumor, (either primary or metastatic), late-stage ankylosing spondylitis, idiopathic, lymphoma, sarcoid

24d. S/S of central cord syndrome*

CCS is an acute cervical spinal cord injury, It is marked by a disproportionately greater impairment of motor fxn in the UE than in the LE, as well as by bladder dysfunction & a variable amt of sensory loss below the level of injury.
Although CCS has been reported to occur w/particular frequency among older persons w/ cervical spondylosis who sustain hyperextension injury, it can be found in persons of any age and can be assoc w/various etiologies, injury mechanisms, and predisposing factors. CCS, the patho is the most common incomplete SCI syndrome.

25. Gait abnormalities in Parkinson’s, including the pathophysiology thereof*

Gait w/stooped posture in a slow, short, shuffling manor w/narrow-based steps & loss of arm swing is characteristic of Parkinson's pts. Dysfxn in the pars compacta of the substantia nigra & other pigmented nuclei (located in the basal ganglia that is located in the midbrain), results in death of dopaminergic neurons, thus the brain loses the ability to continuosly produce, store & release DA.  In addition the basal ganglia conects to cortical projections to form the extrapyramidial tract, the area of the CNS responsible for coordination of fluid movements. 

26a. S/S (*case study) of MCA stroke*

mca: contralateral hemiplegia, hemiparesis, hemisensory loss, homonymous hemianopia, eyes look twrd lesion. Broca's Aphasia (if on L), drowsiness, stupor, coma d/t swelling. 

26b. S/S (*case study) of PCA stroke*

pca: hemianopia/quadrantanopia w/macular sparing; contralateral hemiplegia w/dysmetria, mild/temp hemiparesis, cereberllar ataxia, trermor, cn3 palsy. Thalamic syndrome, 

26c. S/S (*case study) of ACA stroke*

ACA: weakness & cortical sensory loss in contralateral leg, mild proximal arm weakness, clumsy hand syndrome, contralateral grasp reflex, paratonic rigidity, lack of initiative, frank confusion, incontinence. b/l:  behavioral changes & memory loss.

27. Analyze causes of vertigo and come up with a working diagnosis based on constellation of symptoms*

S/S of herpes zoster:

• Unilateral Lesions: grouped, tense, deep-seated vesicles distributed unilaterally along a dermatome – usu on face or trunk 

• pain preceeds eruption by 48hrs or more & ↑ in intenstiy after the lesions have gone (Eruption lasts 2-3 wks)

• Regional lymphadenopathy  

• direct fluorescent antibody positive

28b. Treatment of herpes zoster in a trigeminal distribution*

• Acyclovir (800mg/5d), famciclovir(500mg tid), Valacyclovir(1g tid):7d 
• Initial severe pain:nerve blocks 
• Prednisone: 60 mg/d:↓acute pain:tapering 3wk course (non-immuno pts only)   
• Local txt: calamine lotion
• postherpetic neuralgia:prevent w/early,aggressive anti-virals. Lesions:capsaicin ointment 0.025-0.075% or Lidoderm topical patches     
• Chronic neuralgia: regional blocks; Amitriptyline 25-75mg qhs; Gabapentin up to 3600mg (start 300tid) adjunct pain relief prevention: vaccine(pts>60) NOTE: Immuno pts should continue antiviral txt until lesions crust(up to 2wks)-IV antivirals if dx progresses

29a. Neuro symptoms in pregnancy*

-seizures of eclampsia (triad of hypertension, proteinuria, and seizures)

-reversible posterior leukoencephalopathy (the cerebral autoregulation of blood pressure is overwhelmed, usually because of a rapid rise in blood pressure) presenting as he 2 most common clinical presentations in pregnant women are seizures and postpartum blindness.

-cerebral venous thrombosis (CVT) is as common a cause of stroke as cerebral ischemia or cerebral hemorrhage. It is often encountered after delivery

-back pain or posterior pelvic pain during pregnancy

-Compression or stretch neuropathies occur rarely as postdelivery complications

-new onset headache should be thoroughly investigated b/c certain tumor types grow more quickly during pregnancy & AV malformations are found more readily during this time too.

29b. Neuro symptoms in pituitary tumors*

The pituitary gland is located btwn the two cavernous sinuses. Here the nerves that control eye movement  & the major arteries that feed the brain (carotid arteries, the veins that drain the eyes and other nerve related structures) reside. Therefore, tumors at this location compress on these structures causing dbl vision, pituitary apoplexy (severe sudden HA, loss of and/or double vision, protruding eyeball). In addition, b/c the gland is directly beneath where nerves cross (eyes to brain, aka optic chiasm), loss of peripheral vision can be found. Non-Secreting Tumors almost always have problems w/vision, as the hormonally quiet tumor grows to oversized proportions, actually growing to the point of lifting up and stretching the optic nerves (especially where the nerves from both eyes cross as they travel to the brain).

29c. Neuro symptoms in MCA strokes*

contralateral hemiplegia, hemiparesis, hemisensory loss, homonymous hemianopia, eyes look twrd lesion. Broca's Aphasia (if on L), drowsiness, stupor, coma d/t swelling. 

29d. Neuro symptoms in Cushing’s syndrome*

B/c of progressive proximal muscle weakness, pts may have difficulty climbing stairs, getting out of a low chair, and raising their arms. Psychological problems such as depression, cognitive dysfunction, and emotional lability may develop. Pts w/ACTH-producing pituitary tumor (Cushing disease) may develop headaches, polyuria and nocturia, visual problems,or galactorrhea, visual-field defects, often bitemporal, and blurred vision may occur in individuals with large ACTH-producing pituitary tumors that impinge on the optic chiasma.

30. Pain distribution of trigeminal neuralgia*

31a. Pathophysiology of ALS*

AD is characterised by loss of neurons & synapses in the cerebral cortex & certain subcortical areas causing gross atrophy of the temporal & parietal lobes (predominately) and parts of the frontal cortex & cingulate gyrus.  AD is also a protein misfolding dx caused by accumulation of abnormally folded A-beta & tau proteins in the brain which cause the neurofibrillary tangles & amyloid plaques that act as the central event triggering neuron degeneration. There IS a inherited susceptibility (genes for B-amyloind precurser protein & apolipoprotein E). 

Mononeuropathy is damage to a single nerve or nerve group, which results in loss of movement, sensation, or other function of that nerve which is outside the CNS.  It is most often caused by injury, although body-wide (systemic) disorders may cause isolated nerve damage. 

33. Steps necessary to pronounce brain death*

Pg. 917: In order to establish brain death the irreversibly comatose pt must be shown to have lost all brain-stem reflex responses, including the pupillary, corneal, oculovestibular, oculocephalix, oropharyngeal, & respiratory reflexes & should have been in this condition for at least 6 hours. The apnea test (presence or absence of spontaneous respiratory activity at a PaCO2 of at least 60mmHg) serves to determine whether the pt is capable of resiratory activity. Reversible coma simulating brain death may be seen in pts w/hypothermia (T<32C) & overdosage w/CNS depressant drugs, and these conditions must be excluded.  Also readhttp://www.braindeath.org/clinical.htm

34. Describe “sundowning” in an Alzheimer’s patient*

Sundowning is widely used to describe a group of behaviors occurring in some older pts w/ or w/o dementia at the time of nightfall/sunset: confusion, fearfulness, anxiety, agitation, aggressiveness, inc motor activity (pacing, wandering, resistance to redirection) & inc verbal activity (yelling). The circadian abnormalities in AD pts (sleeplessness d/t day/night revesal) progress concomitantly w/their behavioral & cognitive dysfxn. (clinicalgeriatrics.com)

35. frontal lobe tumor sxs*

Frontal: intellectual decline (progressive), slowing of mental activity, personality changes, contralateral grasp reflex come back, expressive aphasia if lesion is in Broca's area (inferior frontal gyrus), anosmia (d/t pressure on olfactory nerve), if located centrally in the lobe focal MOTOR seizures or contralateral pyramidal deficits.

36a. S/S of Wernicke’s aphasia*

Intro ppt - pg18:

-d/t damage in left temporal lobe

-verbal expression of language ok

-understanding of spoken or written word impaired

-aka "receptive dysphasia"

-pt makes word salads:language productino is good but no one makes out what they are trying to say...even pts themselves.

36b. S/S of Broca’s aphasia*

Intro ppt pg18:

- understands language ok

-ability to meaningfully express words in speech or writing is impaired 

-pt "can't get words out

-aka "Expressive dysphasia"

- d/t damage in frontal lobe

36c. S/S of Apraxic aphasia*

36e. S/S of Cranial nerve XI degeneration*

 Injury to the SAN results in varying degrees of shoulder dysfunction. SXS: 

-Shoulder pain (the most common presenting symptom) can radiate to the neck and upper back and occasionally to the ipsilateral arm, it, may worsen when the weight of the involved shoulder is not supported, placing strain upon the shoulder joint. 

-Diminished strength when performing regular daily activities (eg, placing dishes in overhead shelves, exercises that involve bearing weight on the shoulders).

-Limited or loss of sustained abduction of the shoulder is the most common sign. -A full passive range of motion may eventually progress to decreased passive range of motion due to adhesive capsulitis 

-The ipsilateral shoulder may droop.

-Scapular winging or prominence of the medial border of the scapula and protraction may be found.

-Internal rotation of the humeral head may be found.

-Atrophy of trapezius muscle may be found.

37. Causes of intermittent migraine to progress to daily (chronic) migraine*

#1 cause: overusing analgesics (should be used no more than 2x per week, book: or 15x month or 10x month if combination analgesic)

Need to r/o enlarging mass with CT or MRI : tumor, subdural hematoma

38a. S/S of Migraine HA*

Episodic HA with neurologic, GI, and autonomic changes (7 types)

- autonomic sxs include: hyper/ hypotension, nasal stuffiness, peripheral vasoconstriction, tachy/ bradycardia

Common (without Arua):

Must have 2 of:

- Unilateral HA (but can become generalized), Throbbing or - pulsating pain, Moderate to severe pain that may inhibit or restrict ability to fxn, pain aggravated by routine physical activity

Plus 1 of: 

- Nausea and/or vomiting

- Photophobia and phonophobia (intolerance of light and sound)

Classic (With Aura): same dx criteria as common but has an associated aura (that precedes or accompanies the HA)

- Visual, sensory, motor, or language symptoms (ex: scintillating scotomas, numbness, tingling, disequilibrium, smell hallucinations)

Menstrual: due to estrogen withdrawal in late luteal phase = trigger for severe migraine at ONSET of menses (between day -2 & day+3)

- without aura

Basilar artery: uncommon, most frequent in children

Brainstem symptoms: ataxia, diplopia, vertigo, tinnitus, dysarthria, blindness or visual disturbances throughout both eyes, b/l motor and sensory disturbances, depressed level of awareness

Retinal: Rare: preceded or accompanied by monocular visual disturbances (even blindness)

Opthalmoplegic: RARE: HA with paresis of the extraocular muscles, lateralized pain often about the eye, with N/V, diplopia, mydriasis, difficulty moving the eyes in any direction except laterally

- opthalmoplecia d/t 3rd and often 6th nerve palsy

Familial hemiplegic: migraine with aura, including weakness when at least one 1st or 2nd degree relative has same disorder

Sporadic hemiplegic: has genetic mutations, but no other family members are affected

38b. S/S of Cluster HA*

[image]

- Severe, excruciating unilateral, periorbital pain.

- Generally remains on same side of head

- More common in men

- Lasts from 15-180 minutes

- Associated autonomic sxs: Conjunctival injection, lacrimation, nasal congestion, rhinorrhea

- Frohead and facial sweating, Miosis, Ptosis, Eyelid edema: Horner's syndrome

- Frequency: 1 every other day to 8/day (for weeks-mths)

- Attacks occur in clusters

- No Aura or N/V, photophobia, phonophobia

- pt up during attack and active

- Paroxysmal Hemicrania: variant with same sxs, attacks more frequent and shorter

38c. S/S of Tension type HA *

- daily HA that is often vise-like (pressure) or tight in quality (but not pulsatile) of mild-moderate intensity
- Pericranial tenderness, poor concentration & other vague nonspecific symptoms

- Exacerbated by emotional stress, fatigue, noise or glare

- Does not interfere with activity
- Usually generalized, but may be most intense about neck or back of head. 

- Can be episodic or chronic

- Episodic = most common type of HA

- due to abnormal neural sensitivity and pain facilitation, not muscle contraction

38d. S/S of Compressive myelopathy*

The symptomatology (symptoms and signs) of spinal cord compression consists of sensory (pain, numbness and paresthesia), motor and autonomic disturbances, the nature and extend of which is related to: 

*  the level that is compressed - high or low cervical, high and low thoracic, lumbosacral 

*  the direction from which the compression originates; from without or from within the spinal cord, posterior, lateral or anterior 

*  the speed with which the compression is accomplished 

Two good websites:

http://www.neuroanatomy.wisc.edu/SClinic/Myelo/Myelopathy.htm

http://www.slideshare.net/smcmedicinedept/compressive-myelopathy

38e. S/S of PN of axonal type* ???

Classic presentation: decreased sensation, motor abnormalities in a "stocking-glove" distribution. Pts more likely to complain of positive sxs (tripping, new pain, paresthesias, dyesthesias, difficulty walking in dark, loss of balance). Foot drop, decreased reflexes, ataxia, romberg????

Patho:Primary site: neuronal cell body: unable to provide nurtrients that maintain the long axon in the peripheray.  The longest axons are most vulnerable. Degeneration goes slowly occuring first distally & then progressing centrally. DM, HIV and Alcoholism are most common causes; Vit b12 deficiency can too. 

39. Location of brain tumors causing ataxia*

40b. S/S of Temporal arteritis*

• Major symptom is headache 
• often associated with or preceded by myalgia, malaise, anorexia, wt loss, and other nonspecific complaints
• Vision loss is common and often feared
• Clinical exam reveals tenderness of scalp and over temporal arteries

40e. S/S of Wallenberg syndrome*

Ischemic vertigo syndromes (i.e. Wallenberg syndrome) located most commonly in VA (vertebral artery) & PICA (posterior inferior cerebellar artery) it is d/t stroke or ischemic attack. SXS: long lasting vertigo, dysarthria (poor control over speech muscles), dysphagia (difficulty swallowing), hoarseness, hiccups Horner's sign (miotic pupil, ptosis), diplopia, gait limb ataxia, vertical nystagmus. Rule: any episode of vertigo lasting longer than 30 min in a pt with risk factors for stroke should be seen as TIA until ruled out.

41a. Pathophysiology of Epidural hemorrhage*

41b. Pathophysiology of Subdural hematoma*

41c. Pathophysiology of CSF fistula*

Any tear or hole in the membrane that surrounds the brain and spinal cord (dura) can allow the fluid that surrounds those organs to leak (this is known as a CSF fistula). This causes the P around the brain and spinal cord to drop, causing HA that worsens when pt sits up and improves when lie down (other sxs: light sensitivity, nausea, neck stiffness). Drainage of CSF from ear of nose may also occur

Causes include: 

- Head, brain or spinal surgeries

- Head injuries

- placement of tubes for epidural anesthesia or pain meds

- LP

41e. Pathophysiology of Subarachnoid hemorrhage*

42a. Pathophysiology of Meningioma*

Originates from the dura mater or arachnoid; compresses rather than invades adnacent neural structures. Increasingly common w/age. Tumor size varies greatly. Sxs vary w/tumor site (unilateral ptosis=spenoidal ridge, anosmia & optic nerve compression=olfactory groove). Tumor is usu benign & readily detected by CT scanning; may lead to calcification & bone erosion visible on skull xray. 

42b. Pathophysiology of Glioblastoma multiforme*

A malignant, rapidly growing, puply, cystic tumor that spreads w/speudo-like projections. It is a mixture of monocytes, pyrifomr (pear-shaped) cells, astrocytes & fibrous processes. Pt complaints are nonspecific, there is focal deficits. Characteristic "butterfly" shape on scan.

42c. Pathophysiology of Medulloblastoma*

42d. Pathophysiology of Oligodendroglioma*

42e. Pathophysiology of Acoustic neuroma*

-aka vestibular schwannoma: overgrowth of schwann cells on cn8 that grows to involve  the cerebelloponitne angle eventually compressing the pons.  

-usu in internal auditory canal, can expand into postrior fossa (secondary effects on  other CNs & brain stem)

-among the most common intracranial tumors

-SXS:unilateral hearing loss w/ deterioration of speech discrimination, eventual  hydrocephalus, continued dysequilibrium If brain stem compressed: ataxia, gait  disturbances, spasticity, and weakness from long tract effects may result

43a. Pathophysiology of Guillian-Barre syndrome*

- acute, inflammatory demyelinating polyneruopathy (AIPN)

- demyelination of many nerves peripheral and cranial: PNS

Abnormal inflammatory response to a precipitating infection or other immune stimulus

- Activates T-Cells

- Sensitized to P-2 (major peripheral nerve myelin antigen)

- Early antibody attack on myelin

some cases: an inflammatory process

- Infiltrate of lymphocytes, monocytes or macrophages

- destroys myelin

- inability to transmit nerve impulses and renders them nonfxnal

- 60-70% of pts had a mild respiratory or GI infection 5 ays to 3 weeks before onset of sxs

- Triggers: mono, viral hepatitis, HIV, Anti-rabies vaccine, swine flu, surgical procedures, malignant dz, inoculations

- association with preceding campylobacter jejuni enteritis

43b. S/S of Polio*

1.Abortive polio (minor illness): HA, vomitting, diarrhea, constipation, fever, sore throat (2-3d).

2.Nonparalyitc polio:  in addition to sxs of abortive meningeal irritation ans muscle spasm. This variant is indistinguishable from aspetic meningitis.

3.Paralytic polio (.1% pts): paralysis during fever, tremor,  muscle weakness, constipation & ileus. It has two forms: spinal polio (paralysis of shoulder girdle  & bulbar polio (weakness of muscles supplied by Cn - esp. IX, X - and of the respiratory & vasomotor centers.

LMN lesion (flaccid paralysis) w/decreased deep tendon reflexes & muschle wasting. CSF shows excess leukocytes, w/lymphocytic predominance. 

43c. S/S and pathophysiology of Tertiary syphilis*

Patho: may occur at any time after 2° syphilis, even after years of latency (rarely seen today d/t use of antibiotics)

- probably represent a delayed hypersensitivity reactivation of the tissue to the organism 

- 2 types: 1.) localized gummatous reaction (relatively rapid onset and generally prompt response to therapy)

2.) diffuse inflammation of a more insidious onset that characteristically involves the CNS and large arteries (fatal if untreated and at best arrested by tmt)

S/S: any tissue and organ may be involved, most common:

- Gumma formation in  skin, bones, mucous membranes, eyes, lungs, larynx trachea, liver, stomach, cardiovascular system and CNS

Skin: cutaneous lesions: multiple nodular lesions that eventually ulcerate or form pigmented scars; solitary gummas start as painless sc nodules, enlarge, attach to overlying skin and ulcerate

Mucous membranes: leukoplakia

Skeletal system: destructive bone lesions with little or no associated rendess or swelling but often myalgia & myositis of neighboring muscles (esp at night)

Eyes: gummatous iritis, chorioretinitis, optic atrophy, CN palsies

GI: epigastric pan, early satiety, regurgitation, belching and wt loss

CV: aortitis, aneurysms, aortic regurgitation

neurosyshilis 

44a. S/S  of Simple partial seizure*

• General s/s:
  • pts remain conscious and aware of surroundings
  • able to respond appropriately
  • clinical manifestations depend on discrete area of cerebral cortex involved (see ? #67)
  • may have Jacksonian march
  • EEG: focal spies or spike and wave discharges; get while awake and asleep; confirms event and localizes for surgery
  • MRI/CT: if new pathological process, may show: tumor, abscess, stroke, focal encephalitis, head trauma
  • Lab testing: hyperglycemia

44b. Treatment of Complex partial seizures*

• Get EEG and MRI with special attention on temporal lobe. 
• Tmt is same as with generalized tonic-clonic except Lamotrigine can be used for adults and children

44c. S/S  of Absence seizures*

• Benign 1° generalized epilepsy
• Tent to remit in adulthood (by 20yo)
• Main feature: pt's brief lapse of consciousness
• may have mild clonic, tonic or atonic components (ie ↓ or loss of postural tone), autonomic components (ie enuresis/bedwetting), or accompanying automatisms
• Start as a generalized event without aura
• No postictal symptoms
• Hyperventilation may precede seizure
• simple and brief automatic movements may be present
• not focal in beginnings
• Neuro exam: normal
• EEG: brief generalized b/l synchronous 3 Hz spike and wavy discharges: confirmatory

44d. S/S of Generalized “tonic-clonic” seizures*

• Generalized: spreads immediately to both hemispheres
• Usually preceded by nonspecific, vaguely defined prodromes, which can last up to hours
• if Specific aura: focal origin with 2° generalization
• Tonic: Start with loss of consciousness, generalized tonic muscle contraction, a cry and a fall
• Autonomic signs present during tonic phase: tachycardia, hypertension, cyanosis (respiration stops), salivation, sweating, incontinence
• Tonic phase becomes interrupted quickly
• Followed by the clonic phase: jerking of body musculature
• Relaxation phase progressively lengthen and seizure eventually ends
• stage of flaccid coma: pts often stuporous for a moment
• Eventually awaked confused with postictal disorientation, HA, lethargy & myalgia that can last days

45a. Pathophyz, S/S and CSF findings in Asceptic meningitis*

Acute inflammation of the meninges with no evidence of bacteria, fungi, spirochetes, or parasites (onset is hours to 1 day)

- 1° cause: viral infection, esp HSV and enterovirus group 

- Infectious mono may be accompanied by aseptic meningitis

- also occurs with 2° syphilis and disseminated lyme dz. 

- prior to MMR vaccinations mumps was most common cause

S/S: HA, fever, sensorial disturbances, neck and back stiffness, + Kernig and Brudzinski signs, and CSF abnormalities

CSF:

Cells: 25-2000, mostly lymphocytes

Glucose (mg/dl): normal or low

Protein (mg/dl): High (>50)

Opening Pressure: slightly elevated

45b. Pathophyz, S/S and CSF findings in Brain abscess*

Patho: Arise as a sequela of dz of the ear or nose, may be ca complication of infection else where in the body or a result from infection introduced intracranially by trauma or surgical procedures. Bacteriology is usually polymicrobial and includes S aureus, gram neg bacilli, streptococci and anaerobes (including anaerobic streptococci and Prevotella species)

S/S: HA, drowsiness, inattention, confusion, seizures followed by sigs of increasing ICP then a focal neurologic deficit vomiting, fever may be absent, change of mental status or focal neurological manifestations 

- Present as space occupying lesions, when suspected CT should always be preformed

- If CT + for brain abscess: LP should NOT be performed (clinically useful information rarely obtained, and may cause herniation)

45c. Pathophyz and CSF findings in Early bacterial meningitis*

- Acute inflammation of the meninges caused by bacterial infection

- commonly acquired bacterial meningitis: Heamophilus influenzae, Streptococcus pneumoniae, Neisseria meningitidis

- These encapsulated organisms can invade host defenses, and invade blood vessles

- cause bacteremia then enter CNS via choroid plexus

- may be preceded by: head trauma with CSF leaks, sinusitis, pneumonia

S/S: can present abruptly and progress quickly.

- Severe HA, worsened by movement of head and flexion of neck

- Fever up to 40C (105F) in adults. no fever in infants or elderly

- alterations in mental status

- petechial or purpuric rash with meningicoccal infection

Nuchal rigidity

- +Kernigs and Brudzinski sign

- other variable signs: CN palsies (esp with pneumococcal), alteration of sensorium, seizures, papilledema

CSF: >1000 wbcs/ul; 60% PMNs

- Glucose: low: <40 mg/dl (<50% serum glucose)

- Protein >150 mg/dl

- Gram stain: + cocci 80-90% of time

- opening P: markedly elevated

45d. Pathophyz, S/S and CSF findings in Viral encephalitis

• Patho: usually herpesviruses, arboriviruses, rabies virus, flaviviruses, etc 
• s/s: produces disturbances of the sensorium, seizures, and many other manifestations including low grade fever, mild HA, low energy,  clumsiness, unsteady gait, confusion, drowsiness, irritability Pts are more ill than those with aseptic meningitis 
• CSF: may be normal or show some lymphocytes and in some instances  (eg herpes simplex) RBCs

46a. S/S of Postherpetic neuralgia*

• Develops in 15% of pts with shingles, particularly in the elderly with severe rash and when the 1st division of the trigeminal nerve is affected
• Burning, sharp and jabbing or deep and aching pain. 
• Affected skin may be sensitive to light touch (ex: touch of clothes on skin)
• Generally limited to the area of skin where shingles outbreak 1st occurred

• most common in middle aged to elderly women
• Momentary episodes of sudden lancinating facial pain occur and commonly arise near one side of the mouth and shoot toward the ear, eye, or nostril on that side. 
• May be triggered or precipitated by touch, movement, drafts, and eating. (many pts will try to hold the face still while talking to avoid triggering further attacks)
• Spontaneous remissions for several months at first
• progression leads to more frequent attacks, with shorter remissions and a dull ache that may persist between episodes

• Idiopathic facial paresis of LMN type attributed to an inflammatory reaction involving the facial nerve near the stylomastoid foramen or in the bony facial canal
• may be due to reactivation of herpes simples virus
• more common in pregnant women or with DM
• Abrupt facial paresis, that worsens over the next few days
• Pain about the ear that lasts a few days
• Face feels stiff and pulled to one side
• May be an ipsilateral restriction of eye closure, difficulty with eating, fine facial movements, hyperacusis
• disturbance of taste is common 

• most common in middle aged women, esp depressed
• constant, often burning pain
• may have a restricted distribution at onset but soon spreads to the rest of the face on the affected side and sometimes involves the other side, neck, or the back of the head 

47. S/S of status epilepticus*

Recurrent seizures w/o full recovery of consciousness btwn them; a fixed & enduring epileptic condition >30mins. Mortality rate 30%, Neuro defict: 30%Usu d/t: anticonvulsant w/drawl, EtOH detox, Acute encephalopathy, hypoglycemia, systemic infection.  Medical er!! Initial mgmt: secure airway, give O2, watch for aspiration. If they continue: iv diazepam/lorazepam (to break it) & iv pheytoin to maintain, also give glucose, mag to replenish pt. Monitor admin w/ecg to note arrythmias.  If all of the precededing fail then general anesthesia w/vent & neuromuscular jxn blk.  

48. Pathophysiology of traumatic brain injury, including consciousness patterns

49a. Description of vegetative state*

Pg. 917 - persistent vegitative state: pts w/severe b/L hemispheric dx may show improvement from an initially comatose state, so that, after a variable interval, they appear to be awake but lie motionless & without evidence of awareness or higher mental activity. Most pts in this state will die in mths or years, but partial recovery has occurred. Sharon describes this as cerebral death: an irrevsible loss of fxning of cerebral hemisphere BUT fxn of brainstem & cerebellum w/persons unresponsive to environment (loc). Respiration, cv control, body temp, brainstem relflexes (yawning, grasping, sucking) intact as well as sleep-wake cycle. No conscious perception. 

49b. Description of brain death*

In order to establish brain death, the irreveribly comatose pt must be shown to have lost all brainstem reflex responses, including the pupillary, corneal, oculovestibular, oculocephalic, oropharyngeal & respiratory relflexes, & should have been in this condition for at least 6 hours. Sharon states it is the irreversible loss of cerebral, brainstem & cerebellar fxns resulting in loc, respiration, cv and temp control fxn, no sleep-wake cycle, flat eeg

49c. Description of locked-in syndrome*

aka De-efferneted state

Acute destructive lesions (i.e. infarction, hemorrhage, demyelination, encephalitis) involvine the ventral pons & sparing the tegmentum may lead to a mute, quadriparetic but conscious state in which the pt is capable of blinking & voluntary eye movement in the vertical plane, w/preserved pupillary responses to light. Such a pt might be mistakenly be regarded as comatose. The pt is fully aware of their surroundings.

50a. Location of lesions seen with monocular visual loss*

50b. Location of lesions seen with bitemporal hemianopia*

50c. Location of lesions seen w/homonymous hemianopia*

posterior to chiasm

50d. Location of lesions seen with cortical blindness*

 B/l lesions of the primary visual cortex may cause cortical blindness. SXS:  visual hallucinations, denial of visual loss (Anton–Babinski syndrome), and the ability to perceive moving but not static objects. (Riddoch phenomenon).

50e. Location of lesions seen with central field deficit*

51. S/S of Alzheimer’s disease*

Insidious onset -> decline in multiple cognitive abilites over years. 1st sxs: inability to learn new material (rapid forgetting or loss of short term memory). There are 3 stages of AD:

I. memory impairment, personality & affect (mood) changes, problems w/IADLs, ADL difficulties absent or minimal, neuro findings & motor skills unaffected. 

II. Aphasia, apraxia, agnosia, suppervision w/IADLs, impaired fine motor coordination, may need help w/ADLs,

III. severe memory impairment, help req'd w/ADLs, hyperrelexia, gait aprazia, frontal release signs (primitive grasp/snout reflexes), Parkinsonian sxs, possible myoclonus, imparied fxn, marked rigidity, diffuse hyperrelexia, spasticity, progressive inability to swallow

52a. Distribution of sensory abnormalities in cerebral hemisphere stroke

52b. Distribution of sensory abnormalities in brain stem lesion

52c. Distribution of sensory abnormalities in spinal cord lesion

52d. Distribution of sensory abnormalities in peripheral neuropathy*

Most neuropathies: symmetric w/distal loss.

Large nerve fibers affect position sense, vibratory perception, tendon reflexes. Three types: 

1.Axonal: "stocking-glove" distribution (distal to proximal)

2.Wallerian degeneration: immediate paralysis of the muscle or loss of sensation in area supplied by axon

3.Segmental demyelination (inflammatory/autoimmune): fairly symmetric w/motor involvement > sensory. Weakness is present in both proximal and distal muscles, and this pattern is a hallmark of acquired demyelinating polyneuropathy

53a. S/S of Bacterial meningitis*

can present abruptly and progress quickly.

- Severe HA, worsened by movement of head and flexion of neck

- Fever up to 40C (105F) in adults. no fever in infants or elderly

- alterations in mental status

- petechial or purpuric rash with meningicoccal infection

- Nuchal rigidity

- +Kernigs and Brudzinski sign

- other variable signs: CN palsies (esp with pneumococcal), alteration of sensorium, seizures, papilledema

There are 4 clinical types: 

1. asymptomatic: CSF abnormalities without symptoms 

2. Meningovascular: meningeal involvement or changes in vascular structures of the brain, producing symptoms of chronic meningitis (HA, irritability), CN palsies (basilar meningitis), unequal reflexes, irregular pupils with poor light and accommodation reflexes and cerebrovascular accidents if large vessels are involved

3. Tabes doralis: progressive degeneration of posterior columns, causing impairment of proprioception and vibration sense, Argyll Robertson pupils (poorly reacting to light, but well to accommodation), muscular hypotonia and hyporeflexia, wide based gait, inability to walk in dark. Paresthesias, analgesia or lancinating pain in leg muscles. Crises are common including: gastric (sharp pain, N/V), laryngeal (paroxysmal cough and dyspnea), urethral (painful bladder spasm), rectal & anal. Painless trophic ulcers on feet and joint damage also occur

4. General paresis: generalized involvement of cerebral cortex leading to ↓ in concentration power, memory loss, dysarthria, tremor of fingers and lips, irritability and mild HAs. Change in personality: pt becomes slovenly, irresponsible, confused and psychotic.

Classic presentation: tremor, "cogwheel" rigidity, bradykinesia, postural instability

2ndary features: micorgraphia, masked facies, sialorrhea, freexing, soft speech, reading problems, orthostatic hypotension, constipation, excessive perspiration, impotence, urinary dysfxn, sleep disturbance

• a rare, but life-threatening, idiosyncratic reaction to a neuroleptic/antipsychotic medication. The syndrome is characterized by fever (up to 41C), muscular rigidity, altered mental status, and autonomic dysfunction
• Although potent neuroleptics (eg, haloperidol, fluphenazine) are more frequently associated with NMS, all antipsychotic agents, typical or atypical, may precipitate the syndrome (clozapine and many others)
• Autonomic instability: BP extremes, tachycardia, tachypnea, diaphoresis, incontinence, tremulosness, 
• Tremors: coarse, cogwhelling, lead-pipe rigidity
• EPS symptoms: Sialorrhea (excess salivation), Opisthotonus (arched back and lying position of body with feet and hands on bed; caused by spasm), oculogyric crisis (rapid eye movements)
• Agitation: restlessness, aversion to being still (akathisia)
• Exam: ↑ leukocytes and CPK, non-focal slowing on EEG

The sxs are the same as w/Parkinson's but this condition is caused by other dxs, toxins (CO) or meds that, once removed, will improve or eliminate the issues.  Some of the worst meds are: cholpromazine (thorazine), haloperidol (haldol), thiordasine (mellaril).

57.   Makeup of a normal CSF (lab findings)*

Color: clear and colorless

P: < 20 cm H₂O

RBCs: none

WBCs: adults 0-5 cells/ul; 6-18 yo: 0-10; 1-5 yo 0-20; neonate 0-30 

Protein: 15-45 mg/dl (up to 70 in elderly and children)

Glucose: 50-75 mg/dl (60-70% of blood glucose level)

Chloride: 700-750 mg/dl

LDH: < or = 40 unitl/L adults

Lactic acid: 10-25 mg/dl

Glutamine: 6-15 mg/dl

58b.  S/S & important historical findings in Brain abscess*

An intracranial space-occupying lesion arising as a sequela of dx of the ear or nose, as a complication of infection elsewhere in the body, or from infection introduced intracrainally by trauma or surgical procedures. The most common infective organisma are strept, staph & anaerobes - congenital heart dx may be present. SXS: HA, drowsiness, inattention, confusion & seizure early followed by signs of increasing ICP & focal neuro deficit. There may be little to no systmeic evidence of infection. CT typically shows an area of contrast enhancement surrounding a low-density core. 

58c.  S/S and important historical findings in Viral encephalitis

59.   S/S and etiology of normal pressure hydrocephalus*

• Occur sporadically or on a familial basis with autosomal dominant inheritance
• 2 Types: 
  • Type 1: Recklinghausen dz; characterized by multiple hyperpigmented macules and neurofibromas. Associated cutaneous lesions: axillary freckling and cafe au lait spots (patches of cutaneous pigmentation)
  • Type 2: 8th CN tumors; is often accompanied by other intracranial or intraspinal tumors
• neurologic presentation is usually with s/s of tumor
• palpable mobile nodules on superficial cutaneous nerves 
• Plexiform neuromas may develop: marked overgrowth of subcutaneous tissue sometimes with an underlying bony abnormality
• occasionally become malignant and lead to peripheral sarcomas 
• meningiomas, gliomas (esp optic nerve), bone cysts, phenochromocytomas, scoliosis, and obstructive hydrocephalus may also occur

Early sxs: delayed milestones i.e. controlling head, rolling over, reaching with one hand, sitting without support, crawling or walking. Persistence of primitive reflexes

Sxs can be mild to severe depending on severity of brain damage & may include:

- Abnormal muscle tone: spastic or unusually relaxed an floppy. limbs may be held in unusual positions

- abnormal movements: may be jerky or abrupt or slow and writhing. May appear uncontrolled or without purpose

- skeletal deformities: if unilateral may have shortened limbs on affected side

- Joint contractures: severe stiffining of joints b/c of unequal P on them

- Mental retardation: around 44%

- Seizures: 33%; may appear early in life or years after the brain damage that caused CP. physical signs may be masked d/t abnormal movements of CP

- Speech and swallowing problems: inability to control muscles of tongue, mouth and throat

- Hearing loss: partial hearing loss

- Vision problems: 3/4 have strabisus (turning in or out of one eye) and are often nearsightedness

- Bowel and bladder problems d/t lack of muscle control

Many persons with a Type I CM do not have symptoms and may not know they have the condition. Individuals with other CM types 2-4 may complain of neck pain, balance problems, muscle weakness, numbness or other abnormal feelings in the arms or legs, dizziness, vision problems, difficulty swallowing, ringing or buzzing in the ears, hearing loss, vomiting, insomnia, depression, or headache made worse by coughing or straining. Hand coordination and fine motor skills may be affected. Sxs may change for some individuals, depending on the buildup of CSF and resulting pressure on the tissues and nerves. Adolescents and adults who have CM but no symptoms initially may, later in life, develop signs of the disorder. Infants may have symptoms from any type of CM and may have difficulty swallowing, irritability when being fed, excessive drooling, a weak cry, gagging or vomiting, arm weakness, a stiff neck, breathing problems, developmental delays, and an inability to gain weight.

http://www.columbianeurosurgery.org/conditions/chiari-malformation/

61.  Pathophysiology and etiology of intracranial aneurysms

62c.  Pathophysiology of Berry aneurysm*

62d.  Pathophysiology of Malaria*

• Malaria is caused by a parasite that is transmitted from one human to another by the bite of infected Anopheles mosquitoes. In humans, the parasites (called sporozoites) travel to the liver, where they mature and release another form, the merozoites. These enter the bloodstream and infect the red blood cells.
• The parasites multiply inside the red blood cells, which then rupture within 48 to 72 hours, infecting more red blood cells. The first symptoms usually occur 10 days to 4 weeks after infection, though they can appear as early as 8 days or as long as a year after infection. Then the symptoms occur in cycles of 48 to 72 hours.
• The majority of symptoms are caused by the massive release of merozoites into the bloodstream, the anemia resulting from the destruction of the red blood cells, and the problems caused by large amounts of free hemoglobin released into circulation after red blood cells rupture. (chills, coma, convulsion, fever, HA, jaundice, muscle pain, n/v, bloody stools, sweating)
• Malaria can also be transmitted from a mother to her unborn baby (congenitally) and by blood transfusions. Malaria can be carried by mosquitoes in temperate climates, but the parasite disappears over the winter.

62e.  Pathophysiology of Febrile seizure*

• NIH definition: "An event in infancy or childhood usually occurring between three months and five years of age, associated with fever, but without evidence of intracranial infection or defined cause." (do LP to rule out meningitis, esp for those under 1 yo, do only when suspected if over 18 mnts)
• Febrile seizures occur in young children at a time in their development when the seizure threshold is low. This is a time when young children are susceptible to frequent childhood infections such as upper respiratory infection,otitis media, viral syndrome, and they respond with comparably higher temperatures.
• Exact patho is unknown, but believed that the cytokine activation is believed to play a role
• does not necessarily occur when the fever is at its highest, but usually occurs during a sudden rise in temperature (39C/102F) as opposed to one that has been present for a prolonged time
• Usually benign and self limiting
• Recurrent febrile seizures occur in 40% 
• Subsequent epilepsy develops in 2-4%

63. Sensory tracts and their cross overs*

Crosses in medulla: corticospinal, post. column (proprioceptive-vibration)

Crosses immediately: spinothalamic

Spinocerebral does not cross

64. S/S of Wernicke’s disease*

Thiamine (Vit B1 deficiency) can result in Wernicke encephalopathy, a serious neurologic disorder that is part of a triad of acute mental confusion, ataxia, and ophthalmoplegia. Korsakoff amneotic syndrome is a late neuropsychiatric manifestation of Wernicke encephalopathy w/memory loss & confabulation; sometimes, the condition is referred to as Wernicke-Korsakoff syndrome or psychosis. Wernicke encephalopathy may develop in nonalcoholic conditions, as in prolonged starvation, hyperemesis gravidarum, bariatric surgery, HIV-AIDS, and even in healthy infants given the wrong formulas.Frequently unrecognized, Wernicke encephalopathy is more prevalent than commonly supposed.

Sxs: h/o long-term alcohol abuse/malnutrition & any of the following: acute confusion, ataxia, ophthalmoplegia, memory disturbance, hypothermia with hypotension, and delerium tremens.

-Ocular abnlities are the hallmarks of Wernicke encephalopathy: nystagmus, b/l rectus palsies, & conjugate gaze palsies reflecting cranial nerve involvement of the oculomotor, abducens, and vestibular nuclei. Less frequently noted are pupillary abnormalities such as sluggishly reactive pupils, ptosis, scotomata, and aniscoria. 

-Encephalopathy is characterized by a global confusional state, disinterest, inattentiveness, or agitation. The most constant symptoms of Wernicke encephalopathy are the mental status changes. Stupor and coma are rare.

-Gait ataxia is often a presenting symptom. Ataxia is likely to be a combination of polyneuropathy, cerebellar damage, and vestibular paresis.

-Vestibular dysfxn, usu w/out hearing loss, is universally impaired in the acute stages of Wernicke encephalopathy. In less severe cases, pts walk slowly w/a broad-based gait. However, gait and stance may be so impaired as to make walking impossible. 

-80% of adults will have some degree of peripheral neuropathy, which may include weakness, foot drop, and decreased proprioception.

-Thiamine deficiency can possibly cause a GI syndrome of nausea, vomiting, abdominal pain, and lactic acidosis.

-Other symptoms that may occur in addition to, or in place of, the classic triad include vestibular dysfunction, hypothermia, hypotension, and coma. Thiamine deficiency often affects the temperature-regulating center in the brainstem, which can result in hypothermia.

-Hypotension can be secondary to thiamine deficiency either through cardiovascular beriberi or thiamine deficiency–induced autonomic dysfunction.

-Of patients surviving Wernicke encephalopathy, many have Korsakoff psychosis: retrograde amnesia (inability to recall information), anterograde amnesia (inability to assimilate new information), decreased spontaneity and initiative, and confabulation. 

-Other manifestations of thiamine deficiency involve the cardiovascular system (wet beriberi) and peripheral nervous system (nutritional polyneuropathy).

65a. S/S of myoclonic seizures*

• Generalized seizure
• consist of single or multiple myoclonic jerks
• can occur on either side of body
• tend to occur in am
• can have unexplained falls
• no lapse of consciousness 
• typically begin during teenage years, sometimes remits spontaneously in later years
• may have occasional tonic-clonic seizures
• occur in kids with various epilepsy syndromes: Lennox-Gastaut syndrome, infantile spasms (West syndrome), Early myoclonic encephalopathy
• in adults: CNS storage dz or prion dx
• neuro exam: normal
• EEG: b/l synchronous irregular spike and wave at variable repetition rates at 4-6 Hz, but no focal epileptic discharges

Focal seizures are partial seizures, they are restricted to a part of one cerebral hemisphere and the S/S depend on the area of the brain involved. 

- there may be focal motor symptoms, somatosensory symptoms, special sensory symptoms or autonomic symptoms

- they can be simple or complex depending on whether or not the pt has altered consciousness

66. Muscle findings of only fasiculations and the workup of such*

Muscle twitching is caused by minor muscle contractions in the area, or uncontrollable twitching of a muscle group that is served by a single motor nerve fiber. These are often visible under the skin arising from spontaneous discharge of a bundle of skeletal muscle fibers

Can be caused by: diet deficiency (inadequate magnesium), drug OD (caffeine), drug side effects, exercise, dehydration, fatigue or by dz processes: LMN lesion, ALS, nerve damage, muscular dystorphy, spinal muscular atrophy, or myopathy

Work up should include: blood tests to look for problems with electorlytes, CBC, thyroid gland fxn, glucose, creatinine, blood chem; EMG, nerve conduction studies, MRI or spine or brain

67.  Manifestations of simple partial seizures*

Brain area involved determines clinical symptoms: may be manifested by focal motor, sensory or autonomic symptoms

• Frontal lobe: focal motor (convulsive jerking)
• Parietal: Somatosensory (paresthesias or tingling)
• Either of above can spread to different parts of the limb or body depending on cortical representation
• Occipital lobe: visual (light flashes)
• auditory, olfactory or gustatory regions of brain cause additional special sensory symptoms (ie buzzing)
• Autonomic symptoms: abnormal epigastric sensations, sweating, flushing, pupillary dilation

68.  S/S of orthostatic hypotension and treatment in the elderly*

The most common symptom of orthostatic hypotension is feeling lightheaded or dizzy when you stand up after sitting or lying down. This feeling, and other symptoms, usually happens shortly after standing up and lasts a few seconds or minutes. Orthostatic hypotension signs and symptoms include: 
- Feeling lightheaded or dizzy after standing up, blurry vision, weakness, fainting (syncope), confusion, nausea, HA

Tmt: change medications that could cause vasodilation or hypovolemia (ie diuretics), elevate head of bed 30 degrees, elastic stockings, sodium tabs, Midodrine (alpha agonist)

69. Treatment migraine headaches*

Migraine: Abortive meds

- excedrine + caffein= 1st go to drug

-5-HT1 receptor agonists: Triptans: standard of care. Vasoconstrictors

Ex: Sumatriptan (Imatrex), Zolmitriptan (Zomig), Rizatriptan (Maxalt); Frovatriptan (frova) for prolonged attacks

- no ergot drugs for 24hr pre and post taking these: prolonged vasoconstriction can lead to MI

- no MAOIs within 2 weeks, caution when taking SSRIs

Ergotamine Tartrate: alpha-adrenergic blocker

-contraindications: pregnancy, sepsis, CAD, Cerebral or peripheral vascular dz, liver or renal dz, uncontrolled HTN

Dihydorergotamine (DHE): potent venoconstictor, no physical dependence or problem with rebound, sam contraindications as with ergots

- give antiemetic with it for nausea

- repeated injections for status migrainous and transformed migraine

Meperidine (demerol): IV in ER only, with antiemetic

Other abortive meds: acetaminophen or aspirin 1000 mg PO; Fiorinal 2 tabs PO; stadol 1mg IN; caffeine adjuvant 60 mg PO; codeine 30 mg PO; Ibuprofen 800 mg PO; Indomethacin 50 mg PO; Butalbital: short acting barbiturate with anticonvulsant and sedative properties (ex: Fioricet, Midrin, Phrenilin, etc)

Migraine + seizures: use anticonvulsants instead of TCAs (induce seizures)

Rebound: Ketorolace (Toradol) nonopoid; combo drugs: Midrin: (isometheptene mucate + dichloralphenazone + acetaminophen) contraindicated in glaucoma, renal dz, HTN, heart or liver dz

Menstrual migraine: 

Abortives: same as all migraines

Refractory menstrual migraines: GHRH agonists, estradiol implants, danazol

70.  Pathophysiology of concussion* 

71.   Migraine headache prophylaxis drugs*

Recommended when HA > 2 times per month or when HA results in extended disability of > 3 days

- let pts help with med selection

- use HA log

- may not need if pts are overusing analgesics (have them stop 1st)

- try to withdraw from these if free of HA for 1 year

B-Blockers: Propranolol bid works best  (start low, go slow)

TCAs: most useful for pts with mixed HA

- Amitroptyline (Elavil) Start 10-25 mg at bedtime increase as tolerated 50-100 is max dose

- Nortiptyline (pamelor) works well, fewer anticholinergic side effects; same dosing

- (AEs: sleepiness, dry mouth, constipation, wt gain, Precautions with: urinary retention, angle closure glaucoma, cardiovascular abnormalities)

Anticonvulsants: for those with bad ups and downs

- Divalproex sodium (depakote)

- side effects: tremor, sig wt gain, alopecia, hepatitis, pancreatitis

Combo meds if single agent fails: ex B-Blocker + TCA

Methysergide: an ergot; use if other agents fail; give for 6 months then a 1 month holiday to prevent fibrotic complications (in lungs); many contraindications

MAOIs: for HA refractory to other methods: Phenelzine sulfate (Nardil); huge list of meds and dietary restrictions (including sympathomimetic meds, red wine and aged cheese)

Menstrual migraines: 

Short term Prophylaxis:

- NSAIDs (Naproxen 550mg bid day 15-menses)

- Hormones: estradiol 0.1mg patch day -4 to +4

- Suplements: magnesium 360mg qd day 15 to start of menses

- Triptans: given around menses; Frovatriptan 2.5 mg bid does not increase post tmt HA

Zolmitriptan 50% reduction in HA freq; Naratriptan effective but ↑ post tmt HA

Continuous prophylaxis: same as for regular migranes, increase dose before menses

- extended dosage OCPs: 168 day cycles

72.   Muscles inervated by abducens nerve*

CN VI is a motor nerve that innervates the lateral rectus muscle (responsible for moving eye laterally) of the ipsilateral orbit

73.  Location of lesions that produce flaccidity and steppage gait*

steppage gait results from lesions on the deep fibular/peroneal nerve.

flaccidity????

74d. Symptoms of facial palsy*

isolated cases may occur in pts with HIV seropositivity, sarcoidosis or lyme dz, but most often it is idiopathic (Bells palsy)

same s/s as with Bell's palsy (see 46c)

75a. Diagnostic criteria for TIA*

75b. Diagnostic criteria for RIND*

A cerebral infarct that lasts > 24 hours, but < 72 hours is termed a reversible ischemic neurologic deficit or RIND.

According to Sharon's notes the diagnostic approach for stroke is to answer these 5 questons:

1. Is it a vascular event (i.e.acute)?

2. Is hopsitalization needed?

3. Is the event hemorrhagic or ischemic?

4. Does the event localize to anter or post location?

5. What is the mechanism (vasculitis, blocked carotid, etc...)?

http://www.cuore.iss.it/eurociss/reg_ictus/pdf/ictus_criteri-diagnostici.pdf

77. Confirmatory lesions of Alzheimer’s (at autopsy)*

79.  Pathophysiology of Herpes Zoster*

- Caused by infection of the nervous system the varicella-zoster virus, after previous chicken pox infection

- usually in adults

- usually only single attack

- generally occurs when immune system is compromised

- HIV+: 20x more likely to develop  (test all pts <55 yo who develop shingles for HIV)

- Pain along course of nerve followed by acute eruptions of grouped vesicles. Pain preceeds eruption by 48hr+ & ↑ in intensity after lesions have gone.

- Unilateral; follows dermatome

- lesions on face or trunk 

80. S/S of L4 compression/ radiculopathy*

81.  Part of brain stem that mediates papillary reflexes & eye movements*

82. Pathophysiology of Huntington’s disease*

It is an autosomal dominant dx that presents w/a characteristic expanded & unstable CAG trinucleotide repeat in the huntinigton gene at 4p16.3; repeat length may affect rate of progression. This abnormality leads to a decrease in GABA (an inhibitory neuroregulator that controls the outflow of DA.  Low GABA = High DA.  In addition there is an increase in somatostatin (a transmittler that enhances release & action of DA) so high somatostatin = High DA.  This is a chronic, progressive degenerative dx involving the caudate nucleus & cerebral cortex.  Autopsy will reveal severe neuronal loss w/decrease in brain weight of up to 30%. 

• At least 10 previous HA episodes
• Fewer than 180 per year or 15 per month
• Duration from 30 minutes to 7 days
• no N/V, + anorexia
• No photo/phonophobia

Eye opening:

4: Spontaneous at baseline 

3: Opens to verbal command, speech, or shout

2: Opens to pain 

1: none

Verbal Response:

5: Alert and Oriented

4: Confused, yet coherent conversation

3: Inappropriate responses, word salads

2: Incomprehensible speech

1: none

Motor:

6: obeys commands for movement

5: purposeful movement to pain (localizes to stimuli)

4: Withdraws from pain

3: abnormal (spastic) flexion, decorticate posture

2: extensor (rigid) response, decerebrate posture

1: none

Hydrocephalus:Presenting sxs are related to the age of the patient: 

- young, nonverbal patient presents with behavioral change, increasing head circumference often is the only presenting sxs in infants.

-young kids: listlessness, irritability, vomiting, & decreased social interactions.

-older kids & adults: HA, especially upon awakening in the morning. Vomiting w/out nausea is more common in the morning, since being recumbent (eg, sleeping) increases ICP.

-Pts may develop double vision (Cn6 stretched from the hydrocephalus).

-Visual disturbances more commonly are a result of papilledema

Temporal: seizures w/hallucinations of smells & tastes, motor abnormalities (licking/smacking lips), impariment of external awareness w/o LOC, depersonalization, emotional changes, behavioral disturbances, de ja vu/jamais vu, visual field defects (homonomous hemianopsia), auditory hallucingations, receptive aphasia (if on left side), problems w/perception of musical notes/songs (if on R)

Parietal: contralateral disturbances of sensation, sensory seizures or loss, lose sense of posture/tactile discrimination (like graphesthesia or sterognosis), if tumor is extensive it can produce thalamic syndrome:spontansoue pain!!!), involvement of optic raditation: contralateral hymonymous field defect, denial neglect & constructional aprazia (can't copy drawings) if tumor is on R, rejection of paralyzed limb (if on L).

- crossed homonymous hemianopsia (or a partial field defect) if tumor is very big or small

- L-sided or b/L lesions cause visual agnose for objects/colors

- Irritative lesions on either side (d/t bld from tumor) cause unformed visual hallucinations "sees    ghosts"

- inability to identify a familiar face

-coritcal blindness if tumor is b/l

-Cn nerve palsies

-nystagmus (usu vertical d/t gaze ctr is knocked out)

-pyrimidal & sensory deficits in limbs (uni if cerebellar or b/l if brainstem involved) 

-rise in ICP comes late IF brainstem tumors are contained w/in

-cerebellar tumors: marked ataxia of trunk or limbs 

(Nl: pinel body lies in the corpus callosum & produces melatonin)

- increased ICP

-impaired upward gaze

-midbrain lesion

Looks like a pituitary adenoma w/o hormonal changes.  It originates above sella turcica, deprepresses optic chiasm, usu in kids, can lead to endocrine dysfxn (d/t mass effect), bitemporal field defects

• Valpronic Acid (depakane/kote):  Drug of choice for idiopathic generalized seizures (including absence) 20-60mg/kg. Adult 750-1500 mg qd.( t1/2 = 8 hrs)
• Phenytoin (Dilantin or cerebyx) common in adults (not usually children). 5-8mg/kg/qd, 300-400 mg/qd (t1/2=24hr)
• Carbamazepine (Tegretol or caratrol)15-30 mg/kg/qd adult 1000-1400 mg/qd
• Phenobarbital: usually used in combination. Drug of choice for neonates. QD or BID dosing. 5 mg/kg/qd. Adult 90-180mg qd (t1/2= 24hr)
• Lamotrigine (lamictral): newer drug, used as adjunct for generalized seizures in children. May develop a rash + interactions with ohter antiepileptic drugs
• Adjuncts: Gabapentine (Neruotonin), Topiramate (Topamax), Levetriracetam (Keppra), Tiagabine (Gabitril), Zonisamide (Zonegran)
• Other tmts: ketogenic diet, vagal nerve stimulator, resection of identified site or corpus callosum section or disconnective hemispherecctomy

• Ethosuximide (Zarontin) 15-40 mg/kg qd (adult: 750-1000 mg qd) Drug of choice
• Valpronic Acid (depakane/kote): 20-60mg/kg. Adult 750-1500 mg qd.( t1/2 = 8 hrs)
• Clonazepam

• Tmt is same as with generalized tonic-clonic except Lamotrigine can be used as an adjunct for adults and children

symptoms: acute progressive polyradiculoneuropathy, weakness more severe than sensory, acute dysautonomia (life threatening)

- develop over hours to days, peak in an average of 12 days

- rapidly progressive and severe ascending symmetric weakness

- initial complaint of overall leg weakness affecting stair climbing and walking

- weakness starts in the thighs and ascends to arms in days

- Pain in 30%

- bilateral sciatica

- cramping like discomfort

- b/l weakness of facial muscles (33%)

- swallowing difficulty (10%)

- severe: respiratory (vent required in 33%), eye movements. 

- autonomic disturbances: tachycardia, cardiac irregularities, hypo/ hypertension, facial flushing, sweating, pulmonary dysfnx

Mild cases: self-limiting, minimal weakness

Worst: quadriplegia, opthalmoplegic, requires ventilation for 1 yr 

PE: Proximal to distal weakness with no atrophy

- ↓ in joint position perception, vibratory sense (only sensory nerves that are myelinated)

- pain/temp ↓ in stocking glove distribution late finding (severe)

- sensory ataxia

 1. Premonitory phase: 40-60% of pts

- Behavioral, emotional, autonomic and constitutional disturbances that happen 1-2 days before HA. Include sleep disruption, food cravings, fatigue, yawning, depression and euphoria

2. Aura: minority of migraineurs

- usually precedes the HA, can also accompany it

- dx criteria (3/4): 1 or> reversibel symptoms; aura sxs develop over 4 minutes; last no longer than 60 min; HA onset with in 60 min after aura ends

3. Headache: usually unilateral but can be b/l (40% of pts)

4. Headache resolution: criteria: varies form 4-72 hrs without tmt

5. Postdromal Phase: Often includes sxs of fatique and mental

confusion. Lasts 1-2 days

2 major theories: 

1. vascular theory: vasoconstriction causes the aura; vasodilation causes the pain; not supported by research

2. Current theory: pain generated by serotonergic and adrenergic pain-modulating systems in trigeminal system

- anything from C3 and up can trigger trigeminal system (ie superior sagittal sinus, meninges, meningeal vessels, cerebral vessels, trigeminal ganglion, trigeminal nerve)

Regular exercise, stretching, relaxation techniques, simple analgesics.

- If no response consider prophylasis: TCAs or SSRIs, esp with comorbid depression; Local Botox injections may help

- Do not overuse meds: cause chronic daily HA. 

Cluster:

Symptomatic: 

- O₂ by face mask at 7L/min until attack aborted (dont exceed 20 min). If doesn't work, wait 5 min & repeat

- Sumatriptan, 6 mg SC

- DHE 0.5-1 mg SC, IM, IV, IN

- Ergotamine 1 inhalation; repeat in 5 minutes

Prophylactic: 

- ↓ length of cluster period & avoid overuse of abortives

- maintain tmt at least 2 wks after cluster, gradually taper

- CCBs: verapamil (Calan) 1st choice

- Lithium: 2nd choice

- Indomethacin (avoid if possible)

- Ergotamines

- Methysergide (only if all others fail)

- Cortosteroids (as last resort)

- Anticonvulsants: Divalproex sodium (depakote), Valproic acid (Depakene), Topamax (sulfamate)

- MAOIs: phenelzine (Nardil) (although better for migraine)

Paroxysmal Hemicrania: variant of cluster

- responds well to indomethacin

- If migraine lasts longer than 72 hours

- Repeated DHE injections give relief in 90% of pts

- give IV fluids and O2

- may add steroids PRN

Phenothiazines: Prochlorperazine (Comprazine), Chlorpromazine (Thorazien): iv injections to abort migraines in ER

- For adverse reactions to these: benztropine mesylate (cogentin) or benadryl and keep pt supine for 4 hrs (orthostatic hypotension)