question

Todds paralysis

answer

a brief period of transient paralysis following a seizure

question

acknowledged sources of cardiac embolism to the brain

answer

A fib, mechanical prosthetic heart valves, acute MI, low left ventricular ejection fraction < 30%, patent foramen ovale, and endocarditis.

question

lacunar strokes

answer

small vessel strokes that are characterized by pure motor stroke or pure sensory stroke. Associated with HTN and/or DM

question

risk factors for strokes

answer

age, HTN, smoking, DM, hyperlipidemia, heart disease, hyperhomocysteinemia, and fam Hx

question

Cortical symptoms

answer

aphasia, neglect, agnosia, apraxia. indicate a lesion in the anterior circulation of the brain

question

posterior circulation lesion sxs

answer

diplopia, vertigo, crossed neuro findings, homonymous hemianopia.

question

how to distinguish ischemic vs hemorrhagic stroke

answer

only way is via imaging. however, headache, depressed consciousness, or extreme elevation in BP increase suspicion of hemorrhagic stroke

question

tx stroke

answer

within 3 hrs and no contraindications-> tPA

question

contraindications for tPA

answer

active bleeding, recent stroke, hx intracerebral hemorrhage

question

2ndary stroke prevention (ischemic)

answer

implemented right away: antiplatelet aspirin, clopidogrel, or combination aspirin and extended release dipyridamole (mech unknown but inhibits platelet adhesion and dilates coronary arteries). If high risk for cardioembolic events (afib), give long term anticoag with warfarin risk factor management: control HTN (start tx 1 week after initial stroke. tx hyperlipidemia (LDL <100), carotid endarterectomy or stenting,

question

suspicion for MS

answer

in a young perion with acute onset of symptoms localized to the CNS that are separated by time (acute and past) and space (optic nerve and brain/cord) MS is dx until proven otherwise

question

what is MS

answer

A chronic disease that usually begins in young adulthood that is characterized by relapsing, remitting, or progressive neuro deficits. The lesions are in scattered areas of the CNS.

question

MS subtypes

answer

1. Benign (20%)- few mild attacks with complete clearing 2. Relapsing/remitting (25%)- frequent early attacks and less complete clearing with long periods of stability. 3. 2ndary chronic progressive (40%)-> increasing attacks with fewer and less complete remission after each. worsens for many years then levels off with mod to severe disability 4. primary progressive (15%) -> most disabling. onset is severe and course is slowly progressive.

question

MS presentation

answer

-onset over several days. Initial sxs are often motor related (30-40% of initial attacks are in the corticospinal tract) -50-70% pts have sensory complaints at some point -50% have cerebellar signs at some point (tremor, ataxia, scanning speech, nystagmus) -optic neuritis and retrobulbar neuritis-> blurred vision, scotomas, and decreased color perception **affective disorders in 1/3-1/2. Can have sudden and transient neurologic deterioration if body temp is elevated**

question

Lhermitte sign

answer

sensations of electricity running down the spine that is aggravated by flexing the cervical spine-> seen in MS

question

dx MS

answer

-MRI imaging of brain and spinal cord. Lesions are bright on T2, especially in the corpus collosum and periventricular regions. linear or oviod -MRI spect-> look at N-acetylaspartate. levels decreased in MS plaques -CSF analysis: elevated IgG, oligoclonal bands, and increased myelin basic protien (MBP). -visual evoked responses

question

Tx MS

answer

-Primary tx: steroids -immunomodulating agents to modify course of MS: IFN-B (1a and 1b), glatimer acetate (synthetic MBP), -in pts with worsening dz: immunosuppressive agents: mitoxantrone, cyclophosphamide, methotrexate, and cyclosporine. -antidepressants

question

Subarachnoid Hemorrhage: risk, consequences

answer

risk factors: age (mean age is 50), female, HTN, tobacco use, sentinel bleed. Fibromuscular dysplasia(25%), PKD (3%), liver disease, Marfans, OCP. -60% occur during physical or emotional strain -often result in hyponatremia due to elevation of atrial natriuretic factor, cerebral salt wasting, and syndrome of inappropriate antidiuretic hemorrhage. -ECG changes especially QT prolongation and T-wave inversion, and arrhythmias can result -vasospasm in 4 to 14 days -acute communicating hydrocephalous due to blockage of granulations by subarachnoid blood -intraventricular hemorrhage= worst prognosis -60% die in first 30 days

question

neurogenic pulmonary edema

answer

a rare complication of SAH

question

aneurysm location with CNIII compression

answer

posterior communicating artery

question

most common cause/location of SAH

answer

-ruptured saccular or berry aneurysms account for 80% of nontraumatic SAH and have the worst prognosis. Most frequent side is the anterior communicating artery > bifurcation of the internal carotid with posterior communicating > bifurcation of the internal carotid with middle cerebral artery -in PKD, most common location is anterior comm artery

question

what do if CT is negative but still suspect SAH

answer

-do lumbar puncture and look for xanthrochromia and increased RBCs -most sensitive after 12 hours.

question

Tx of SAH

answer

Grade 1 and 2 (headache and nuchal rigidity without confusion)-> observed after diagnositic measures. -emergent conventional angiography if ruptured aneurysm is suspected -endovascular coiling-> reduces rebleeding in low-grade cases -clipping-> in first 48 hours or after 2 weeks -Triple H therapy to reduce vasospasm (hypertensive hypervolemic hemodilution) and nimodipine

question

first step in dx of migraine

answer

get a head MRI

question

migraine prodrome

answer

Can occur days but usually hours before head pain. More mental such as depression, euphoria, and irritability or increased urination, defecation, anorexia or fluid retention

question

Aura of migraine

answer

aura is associated with neurologic dysfunction prior to onset of pain. have in 25% of attacks. Can last from 5 min to 1 hour. Visual scotomas, teichopsias, fortification spectra, photopias, distortion of migraine. Sensory- numbness, tingling, aphasia, hemiparesis.

question

Pain of migraine

answer

unilateral presentation in 65%, usually periorbital and can extend to cheek and ear (can also occur anywhere else in head/neck). Usually lasts 4-8 hours. usually has pulsating, throbbing quality. -often associated with N/V, photophobia, and phonophobia

question

evaluation of Migraine pt

answer

1)Routine blood studies: chem panel, cbc, HIV testing, vasculitis screen, TFTs, serum protein electrophoresis 2)sed rate: (if older than 60 can be temporal arteritis) 3) spinal fluid examination: (if associated with stiff neck or altered mental status) 4) imaging study: MRI

question

Tx post spinal headache

answer

lay down, if dont improve-> epidural blood patch = inject a small amount of blood into the epidural space at site original spinal tap

question

tx postcoital cephalgia

answer

analgesic

question

sxs and tx of carotid dissection

answer

orbital or neck pain with Horner syndrome on ipsilateral side. Often after trauma to the neck or vigorous movements -can cause ischemia in ipsilateral side of brain-> contralateral signs Tx: many recanalize on own, so stenting only recommended for pts with no vessel recanalization after 3-6 months

question

abortive tx of migraines

answer

1)Triptans (sumatriptan) 2)ergotamine 3) dihydroergotamine 4)isometheptene mucate, dichloralphenazone, acetaminophen

question

Migraine characteristics

answer

onset: teen-40 yrs location: half of face, frontal, usually about eye or cheek precipitating factors:fatigue, stress, hypoglycemia, diet, sunight, hormone changes freq: normally 2-4x month sex: 70% female,30% male duration: 4 hours, postdrome 24-36 hrs

question

prophylactic tx of migraine

answer

used when there are at least 3 attacks/month or if acute tx not effective -anticonvulsants* (topiramate**, divalproex, gabapentin) -beta-blockers (propranolol) - Calcium channel blockers (verapamil) -antidepressants (duloxetine, amitriptyline, nortriptyline)

question

chronic migraine

answer

headaches more than 15 days/month for at least 4 hours/day with history of episodic migranes.

question

Tension headache

answer

May get nausea without vomiting. Pain is in the temporal regions, described as a pressing or tightening, which is of mild to moderate severity often in a hatband distribution. Can also have pain in occipital area and in posterior strap muscles of the neck.

question

non-medical tx of chronic headache

answer

biofeedback, stress management, psychological interventions, and lifestyle changes.

question

medical tx of chronic headache

answer

Removal of OTC medications (can cause rebound headaches) Preventative medications: anticonvulsants, antidepressants, etc. Initial tx is sodium valproate ER. Topiramate, amitriptyline, nortriptyline can also be helpful

question

pediatric migranie

answer

headache lasting 1-72 hours, has at least two of these: either bilateral or unilateral(frontal/temporal) location, pulsating, mod to severe intensity, aggrevated by physical actibitys. Has at least one of these: N/V, photophobia or phonophobia. Dx requires 5 or more attacks TX: abortive: ibuprofen and acetaminophen. If ineffective, try triptans (sumatriptan nasal spray) Prophylaxis: beta-blockers, TCAs, antihistamines, ca channel blockers, anticonvulsants. **Cyproheptadine, amitriptyline, topiramate. **with migraine 2x more likely to have epilepsy and vice versa**

question

cluster headache

answer

unilaterat, repetitive brief headaches with periorbital pain with ipsilateral lacrimation, rhinorrhea, Horners syndrome. More common in males, has circadian pattern. Tx: sumatriptan

question

classic vs common migraine

answer

classic has aura, common does not

question

trigeminal neuralgia

answer

Sxs: paroxysmal, lancinating pain, triggered by cold TX: carbamazepine, phenytoin, gabapentin -MS is often associated with trigeminal neuralgia, caused by a plaque within the brainstem -other causes: basilar artery aneurysms, acoustic schwannomas, posterior fossa meningiomas.

question

atypical facial pain

answer

feel a constant deep pain. Often bilateral but can me unilateral, usually affecting the cheek, nose, or http://quizlet.com/6030222/edit/zygomatic regions. -idiopathic -Tx: antidepressants

question

pseudotumor cerebri

answer

Unknown cause, occurs predominantly in obese women of childbearing age Sxs: headache, transient visual obscurations, progressive visual loss, pulsatile tinnitus, diplopia, and shoulder/arm pain. usually imaging studies are normal, but may see enlarged optic nerve sheaths, small slit-like ventricles, or empty sella. neuroexam-> papilledema, optic atrophy, occasional 6th nerve palsy (horizontal diplopia) increased ICP Tx: high-volume lumbar puncture, acetazolamide, ventriculoperitoneal shunting, optic nerve sheath fenestration

question

Brown-sequard syndrome

answer

hemisection of spinal cord resulting in ipsilateral weakness and loss of fine touch and vibration and contralateral pain and temp loss.

question

Anterior cord syndrome

answer

traumatic or vascular injurt to the anterior 2/3 of the spinal cord due to loss of blood flow from the anterior spinal artery.--> spares dorsal columns. Only retain light touch and position sense. bilateral paralysis and loss of pain/temp sense.

question

Central cord syndrome

answer

injury to structures around spinal canal due to drauma, neoplasms, or dilation of the central canal (syringomyelia). Bilateral loss of pain and temp as well as some weakness (where spinothalamic tracts decussate.

question

spinal shock

answer

acute loss of descending sympathetic tone--> decreased systemic vascular resistance-> hypoTN Tx is aggressive fluid resuscitation -resolves over 1-6 weeks

question

Tx of acute spinal cord injuries

answer

ABCs, immobilize, maintain spinal cord perfusion,etc -surgery-> immediate if significant compromise of spinal canal or very unstable spine. -give corticosteroids (methylprednisone)-> decrease inflammatory response and further damage (this is controversial)

question

Wallenbergs (lateral medullary) syndrome

answer

damage to PICA-> knocks out spinothalamic, descending SNS, nuc ambig, solitary nuclus, CNX nuclea, and vestib nucleus--> decreased contralateral pain, ipsilateral dysphagia, hoarsenss, vertigo, nystagmus, ipsilateral horners, ipsilateral ataxia

question

Epidural hematoma

answer

trauma->loss of consciousness-> lucent period-> seizure/cog deficits/neurologic deficits -with posterior fossa epidural hematoma-> can have dramatic delayed deterioration (can be talking one minute and comatosed/dead the next) *accumulation of blood b/w the inner table of the skull and the outer dural membrane. In 85-95% of patients there is a fracture of the bone. 58% are arterial (acute), 31% are subacute, and 11% are chonic from venous bleeding. -2/3 from tear of the middle meningeal artery -biconvex shaped, high density on CT ***pupil reactivity is a good predictor of outcome

question

physical signs of expanding intracranial mass

answer

-Cushing response (increased ICP->HTN, bradycardia, bradypnea) -decreased or fluctuating consciousness/GCS -dilated, sluggish, or fixed pupils -coma -decerebrate posturing -hemiplegia contralateral to injury

question

Causalgia

answer

caused by trauma to nerves in extremities-> hypesthesia (decrease in accurate perception of stimuli), Dysesthesia (persistent discomfort), and allodynia (perception of pain with application of nonpainful stimuli)

question

ulnar nerve damage

answer

ulnar nerve runs in ulnar groove-> under aponeurosis of flexor carpi ulnaris in the cubital tunnel -damage-> weakness of the interosseous and ulnar lumbricals. Lumbrical weakness-> claw deformity with impaired extension of the ulnar 2 digits= benedicition sign

question

cerebral contusion

answer

traumatic alteration in cognitive function with or without loss of consciousness (mild TBI). -the ascending reticular-activating system (ARAS) mediates wakefulness, transient interruption of fxn-> temporary loss of consciousness -grade 1 (no LOC and sxs resolve in 15 min), grade 2 (no LOC, sxs last >15 minutes), and grade 3 (LOC) TX: if any suspicion of damage, immobilize c-spine (only a health care provider should take off helmet), noncontrast CT if persistent headache, >60 YO, intoxication, emesis, persistent anterograde amnesia, evidence of soft-tissue or bony injury above the clavicles, or a seizure -if grade 1 or 2 with normal CT and neuroexam->home after 2 hours.

question

postconcussion syndrome

answer

up to 90% will have headaches and dizziness for at least 1 month. Often report irritability, depression, insomnia, and subjective intellecutal dysfunction -peak intensity ~1wk post Grade 1-> removed from game for at least 15 minutes and assessed every 5, if at 15 minutes, all symptoms resolved->play Grade 2->return to play in 1 week Grade 3-> go to ER for evaluation. If exam normal and LOC brief-> return in 1 week, if LOC prolonged-> 2 weeks. 2nd concussion-> 1 wk grade 1, 2 weeks grade 2, 1 month grade 3T

question

vertbrobasilar territories

answer

cerebellum, occipital lobes, and thalami -PICA is off the vertebral -PCA, SCA, AICA off the basilar (carotids supply the frontal, parietal, and most of the temporal lobes and basal ganglia)

question

stroke work up

answer

CT (or MRI), ECG, CBC, glucose, PT, PTT, fasting lipid panel, b12, folate, homocysteine, HgA1c, ESR, RPR, HIV and tox screens -ischemic: carotid u/s, echo, MRA, and/or arteriogram.

question

stroke and BP

answer

-acute BP elevation is normal, in general, - BP should not be lowered in the first few days of an ischemic stroke unless extremely elevated

question

signs suggestive of anterior vs posterior circulation lesions

answer

anterior circulation: -aphasia, neglect, agnosia, apraxia etc posterior circulation: -diplopia, vertigo, crossed neuro findings, homonymous hemianopia.

question

Tx of ischemic stroke

answer

IV tPA must be administered within 3 hours. -Contraindications: active bleeding, recent stroke, hx of intracerebral hemorrhage -if not candidates, tx with aspirin, clopidogrel, or combo asprin and ER dipyridamole. -risk factor management-> start meds 1 week after stroke -when afib present-> warfarin therapy used

question

tx hemorrhagic stroke

answer

supportive and involves control of hypertension. Monitor ICP, if elevated-> hyperventilation and osmotic therapy or surgical decompression when appropriate.

question

Causes of carpal tunnel

answer

small carpal tunnel, trauma or injury, overactive pituitary (eg acromegaly), hypothyroidism, diabetes, rheumatoids arthritis, work stress, repeated use of vibrating hand tools, fluid retention, cysts or tumors in the canal, amyloidosis, sarcoidosis, multiple myeloma, and leukemia

question

Acute disseminated encephalomyelitis (ADEM)

answer

Usually follows an infection or vaccination, probably caused by immune-mediated inflammatory demyelination. It is a monophasic illness with multiple neurologic signs reflecting compromise of the brainstem, spinal cord, cerebrum, optic nerves, and cerebellum. -sxs appear suddenly 1-3 weeks after infection -> headache, nausea, vomiting, confusion, and can progress to obtundation and coma. -Tcell mediated, high ESR -perivenular inflammatory myelinopathy with engorgement of veins in the white matter -MRI: hyperintense white matter T2-weighted signals and enhancement on T1. -CSF: increased pressure, elevated RBC and WBC and protein -1/4 will develop MS TX: consider IV corticosteroids, plasmapheresis, and IVIG (controversial) ****things that help distinguish from MS: viral prodrome or recent vaccination, lesions in the thalamus, mental confusion, early onset ataxia, high lesion load on MRI, absence of oligoclonal bands****

question

Viral vs bacterial meningitis

answer

bacterial- higher fevers, petechie, more acute. CSF: elevated opening pressure, elevated protein, decreased glucose, pleocytosis with <60% PMNs. Viral: CSF: elevated opening pressure, normal glucose, elevated cell counts with <50% PMNs (predominance of lymphocytes)

question

Kernig sign

answer

inability to completely extend the leg when the hip is flexed in the supine position. Due to severe stiffness in the hamstring muscles from inflammation of the lumbrosacral roots

question

Brudzinski sign

answer

Flexion of the neck causes involuntary flexion of the thighs and legs

question

latex particle agglutination

answer

antibody or antigen coats the surface of latex particles-> agglutination. used to detect H. influenza B, S.pneumo, and N.meningitidis.

question

empirical tx of meningitis

answer

ceftriaxone or cefotaxime plus vancomycin plus IV dexamethasone plus IV acyclovir If skin rash present, use penicillin or ampicillin

question

most common bacteria in meningitis by age

answer

neonatal: E.coli, Group B strep, Listeria later: S.pneumonia, N.meningitidis, HIB

question

most common viral meningitis

answer

most common= enteroviruses esp coxsackie and echovirus Others= HSV (see bilateral epileptiform discharges in temporal region)

question

infantile botulism

answer

symmetric descending weakness, autonomic dysfunction with hypotension and constipation, respiratory difficulties, CN abnormalities, hyporeflexia. Dx: stool sample culture and serum sample. EMG: repetitive nerve stimulation-> decreased amplitude, tetanic and post-tetanic facilitation, absence of post-tetanic exhaustion Tx: NG feeding and ventilatory support, give antitoxin IV.

question

HIV-associated dementia

answer

classic findings: behavioral changes (apathy, social withdrawal, quiteness), difficulty with coordination (poor balance/coordination), and mild impaired intellect (probs with concentration, attn, mentation, forgetfulness. Later apraxia, frontal release signs) -later, may develop: myoclonic jerks, tremor, bowel/bladder dysfunction -no focal neurodeficits MRI: diffuse cortical atrophy, some have white matter changes and abnl in thalamus and basal ganglia. histo: atrophy of frontotemp are with diffuse myelin pallor, frequent activated glial cells

question

CJD

answer

Rare, degenerative, rapidly progressive, and fatal brain disorder. First probs with muscular coordination, personality changes, impaired memory, thinking, and impaired vision. develop myoclonus, and eventually lose ability to move and speak and enter a coma.--> death. Dx: r/o treatable forms of dementia and chronic meningitis. EEG->periodic sharp waves. Only way to confirm is by brain biopsy or autopsy. test on CSF to detect 14-3-3 protien tx: supportive

question

Tabes dorsalis

answer

Normally 10-20 yrs after infection (HIV positive pts can present much sooner) sxs: lancinating pain with sensory ataxia, CN abnormalities, and impotence or bowel and bladder dysfunction. argyll Robertson pupils, hyporeflexia Dx: RPR and VDRL. CSF study-> elevated protein, lymphocytes, CSF CDRL positive, elevated IgG. Serum studies for treponemal ab (FTA-ABS, TPHA, MHA-TP) -cause-> endarteritis of terminal arteriole-> inflammatory and necrotic changes - demyelination in the posterior columns and inflammatory changes in the posterior roots. -EMG: normal but absent H reflexes due to damage of DRG Tx: high dose IV penicillin G. If allergic doxycycline and ceftriaxone

question

4 forms of neurosyphilis

answer

-asymptomatic -meningovascular (5-7 yrs) -> ischemia, particularly along the MCA and meningial inflammation -tabes dorsalis (10-20 yrs) -general paresis (10 years)

question

Toxoplasmosis

answer

Leading cause of focal CNS disease in AIDS. transmitted though ingestion of cysts or oocytes presentation: headache, wt loss, tonic-clonic seizures, focal neuro signs.. Dx: serum IgM and IgG for toxo, PCR of CSF. CT or MRI-> single but usually multiple hypodense ring enhancing lesions in white matter and occasionally basal ganglia Tx: anticonvulsants and tx of pyrimethane, sulfadiazine, and folinic acid. *in preg-> hydrocephalous, intracerebral calcification, retardation, chorioretinitis, hearing loss, death *prophylaxis with TMP/SMX

question

encephalitis lethargica

answer

transient fevers, lethargy, headache, disturbed eye movements, movement disorder (chorea, athetosis, dystonia, myoclonus, etc) -sequela= severe, unremitting parkinsonism

question

sarcoidosis associated CN prob

answer

CN VII involvement-> facial paresis (weakness without change in sensation)

question

most common fungal meningitis

answer

cryptococcus. occurs in people with defects in immune system.

question

Schistosoma mansoni

answer

can infect the brain and cause a subacutely evolving paraparesis. Deposits effs in the veins of Batson-> lumbrosacral spinal cord. Pt develops granulomas around the ova that lodge in the spinal cord.

question

Echinococcosis

answer

from ingesting feces of sheep or dog->encephalic hydatidosis->dev a major cyste with multiple compartments in which smaller cysts are evident.--> behaves like a tumor

question

Most common rim-enhancing lesions in AIDS

answer

Primary CNS lymphoma (CSF EBV PCR is highly sensitive and specific for it) -Toxo (usually multiple lesions)

question

brain abscesses-> location and mechanism of spread

answer

brain abscesses mostly from hemotogenous spread. usually start from focus of infection at the gray-white junction. aspergillus= most common cause of fungal abscess streptococcal bacteria=most common bacterial abscesses

question

listeria meningitis

answer

often rhombencephalitis. CSF shows pleocytosis but no organism. All blood and and CSF cultures are negative ampicillin +gentimycin

question

PML

answer

see inclusion bodies in the oligodendrocyte nuclei. Primary infection is universal and asymptomatic. When get immunocompromised-> reactivation. dx: MRI-> multiple focal white matter lesions that dont enhance or have mass effect. CSF -PCR, normal except for slight increase in protein. EEG-> slowing of affected area -extensive demyelination and giant astrocytes

question

subacute sclerosing panencephalitis (SSPE)

answer

usually develops in children and is rare after age 18. Most have had measles before the age of 2, but sxs of sspe dont show up for 6-8 years. Produces a CSF pattern similar to MS with increase in gamma globulin fraction and oligoclonal bands.. -demyelinating disease, eosinophilic inclusions in cytoplasm of glial cells and neurons -decreased concentration, emotiona lability, cognitive impairment-> seizures, chorioretinitis, ataxia, hyperactive reflexes, bilateral babinski. -EEG shows bursts of high-voltage slow waves followed by low-voltage stretches.

question

auditory brainstem response

answer

electrical evoked hearing test. Electrodes are placed on each ear lobe and on the forehead. a sound is delivered at a specified loudness, an attached computer captures the electrical brain activity.

question

epidermoid tumor

answer

benign, composed of squamous epithelial elements that arise from congentital nests

question

metastatic brain Ca

answer

-brain metastases are 10x more common than primary brain tumor (most common= lung>breast>melanoma) -metastatic tumors most commonly supratentorial and located at the gray-white junction and arterial border zones -give corticosterids to reduce edema -give anticonvulsants if seizures -melanoma is hyperintense on T1 and hypointense on T2

question

Glioblastoma multiforme

answer

Grade 4 astrocytoma. Most common primary brain tumor in adults. <1 year life expectancy. Can cross corpus callosum. Stains with GFAP -pseudopalisading pleomorphic tumor cells that border central necrosis/hemorrhage

question

Glomus tumor

answer

highly vascular tumor arising from neuroepithelial cells. 10% produce catecholamines, 10% bilateral, 10% familial, 10% malignant

question

meningioma

answer

2nd most common primary brain tumor in adults. Benign tumors of mesodermic origin (arachnoid cells) attached to the dura. Commonly along sagital sinus, over cerebral convexitites, and in the cerebellar-pontine angle. -may cause hyperostosis (thickening of bone) -gray, sharply demarcated, firm -histo: uniform cells with round, elongated nuclie, and whorled pattern. Psommoma bodies -affect women more -slow onset of neurologic deficits or a focal seizure Tx: surgical therapy is curative

question

Schwannoma

answer

3nd most common brain tumor. Often localize to CN VIII at cerebellopontine angle-> acoustic schwannoma. S-100+, Resectable. Bilateral is associated with NF2

question

oligodendroglioma

answer

rare, slow growing. Often in frontal lobes. Chicken-wire capillary pattern. Oligodendrocytes have a "fried egg" appearance

question

Pilocytic astrocytoma

answer

common tumor in kids. well circumscribed brain tumor in kids, GFAP +, benign, good prognosis. eosinophilic corkscrew fibers

question

Medulloblastoma

answer

most common tumor in kids. Highly malignant cerebellar tumor in children, from of primitive neuroectodermal tumor (PNET). Generally controllable with chemo. -see rosettes or perivascular pseudorosettes, small blue cells

question

ependymoma

answer

ependymal cell tumors, often in the 4th ventricle, can cause hydrocephalous, poor prognosis. have perivascular pseudorossetes, rod-shaped blepharoplasts near nucleus

question

hemangioblastoma

answer

associated with vHL when found to retinal angiomas. can produce EPO-> polycythemia. -often multiple an become symptomatic by bleeding into themselves. -foamy cells and high vascularity Tx: surgical resection

question

craniopharyngioma

answer

benign childhood epithelial tumor that can cause bitemporal hemianopia and hypopituitarism. Most common supratentorial tumor in childhood. Calcification is common

question

brain tumors that are calcified

answer

astrocytoma, meningioma, oligodendroglioma, or metastatic tumors.

question

pineal tumors

answer

1/3 are germcell tumors. pineocytomas-> Parinaud syndrome: loss of vertical gaze, loss of pupillary light reflex, lid retraction, and convergence-retraction nystagmus (eye appears to jerk back into orbit on attempted up gaze). -> due to lesions in the dorsal brainstem near superior colliculus

question

hypothalamic hamartomas

answer

nonneoplastic malformations with neurons and glia in the hypothalamus -may cause neuroendocrine probs-> precocious puberty or acromegaly. -may experience paroxysms of laughter, known as gelastic seizure.

question

paraneoplastic cerebellar degeneration (PCD)

answer

relentlessly progressive ataxia, dysarthria, and nystagmus and cerebellar atrophy due to loss of Purkinje cells. Most commonly associated with small cell ca of lung, ovarian ca, and lymphoma.

question

Lambert-eaton myasthenic syndrome (LEMS)

answer

Poximal muscle weakness that develops subacutely and spares the bulbar muscles and eyes -loss of function of voltage-sensitive Ca channels in the presynaptic terminal and NMJ -increased strength with repeated muscle activation -assoc with small cell ca of lung or other malignancy tx: immune-modulating therapy, 3,4-diaminopyridine.

question

Vitamin B12 deficiency

answer

-B12 impt in conversion of methylmalonyl Coa to succinyl coA and homocysteine to methionine. Without B12, homocystiene and methylmalonic acid accumulate. -demyelination/gliosis/vacuolar degeneration of dorsal column, lateral corticospinal tract, and somethimes the lateral spinothalamic tract -presents with parasthesias of hands and feet -> loss of vibratory and joint position sense, weakness, spasticity, +babinski-> optic atrophy, mental changes, etc -macrocytic anemia, premature whitening of hair, beefy, red, smooth, sore tongue -centrocecal scotoma -increased intensity on T2 of dorsal column and lateral tracts TX: IM B12

question

Essential tremor

answer

ET is the most common movement disorder. Cogwheel effect without increased tone. - often begins gradually starting in teens -tremor of 6-12Hz during movement -bilateral onset, can affect arms>head, legs, larynx, trunk. -tremors disappear at rest or asleep. -alcohol dramatically decreases tremor -increased activity in the thalamus Tx: beta-blockers and primidone. In severe cases deep brain stimulation

question

Huntingtons disease tx

answer

-chorea-> haloperidol (can cause tardive dyskinesia) or tetrabenazine (dopamine-depleting agent, not associated with tardive dyskinesia) -depression/anxiety-> SSRI -PEG if trouble swallowing

question

primary generalized dystonia

answer

syndrome with sustained muscle contractions that provoke twisting and repetitive movements or abnormal postures -most often due to a GAG delection in DYT-1 gene -begins with a focal action dystonia before mid 20s that commonly generalize-> severe gait and posture abnormalities -results from abnormally low or abnormal activity of basal ganglia output structures: GPi and substantia nigra pars reticulata. Tx: levodopa, central muscarinic cholinergic blockers (trihexyphenidyl), benzos, baclofen, botox, deep brain stimulation of globus pallidus, pars interna (most effective)

question

Parkinsons tx

answer

-DA agonists: pramipexole, ropinirole, bromocriptine-dopaminergic agents: levodopa +carbidopa(blocks peripheral breakdown) -COMT inhibitors: entacapone and tolcapone -MAO-B inhibitors: selegiline and rasagiline -Amantidine: blocks NMDA receptors, decreases tremor and dystonia and alleviates levodopa induced dyskinesias -DBS for dyskinesias ***influenza and MPTP have led to parkinsomism, neuroleptics, metoclopramide **clarity of volume and speech decline, patient gets micrographia** IntraCYTOPLASMIC inclusions in lewy bodies ***on-off effect seen in pts with dz for many years-> abrupt worsening and remitting sxs due to veariability in responsiveness of CNS to meds

question

chorea gravidarum

answer

movement disorder during pregnancy or with estrogen use. With termination of pregnancy and withdrawal of estrogen-> stop of movements

question

Meige syndrome

answer

focal dystonia characterized by blepharospasm, forceful jaw opening, lip retraction, neck contractions, and tongue thrusting. Tx: botulinum toxin injection

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Wilson disease

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hepatolenticular degeneration. Copper deposits. renal tubular acidosis, hepatic fibrosis, heart and lung damage, dementia. -chelating agent is penicillamine.

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locked-in syndrome

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consciousness is preserved but the patient is paralyzed from the eyes down. Survival is usually limited to days or weeks. -Due to ischemic or hemorrhagic damage to the pons, often with basilar artery occlusion

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Spinocerebellar ataxia (SCA)

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AD disease usually due to DNA trinucleotide expansions with > that 25 subtypes. Avg age of onset is 20-30, except SCA6 which is in 40s and 50s. SCA-2 dementia, slow eye movements SCA-3: Most common, pure cerebellar ataxia->ataxia, dysarthria, spasticity, parkinsonism, amyotrophy. Lid retraction, nystagmus, saccade dysmetria, ophthalmoparesis, square-wave jerks.***impaired temp discrimination, bulging eyes SCA-7: ataxia + retinopathy (visual loss) DX: MRI/CT->atrophy of cerebellum, genetic testing Tx: no cure and no tx, only supportive

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tx of tardive dyskinesia

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benzodiazepines, baclofen and vitamin E---> only useful in the most mild cases **drugs that deplete dopamine do not cause this and can be useful in tx. a-methyl-p-tyrosine inhibits formation of catecholamines by blockade of tyrosine hydroxylase, reserpine depletes catecholamines **tetrabenazine-> catecholamine depleter: more effective with few side effects. not available in US **anticholinergics make TD worse!!

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MS symptoms

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-visual compromise, stiffness, and weakness are presenting sxs onset is over several days, initial sxs are often motor. weakness and stiffness-> corticospinal tract Charcot's triad: intention tremor, dysarthria (scanning speech), nystagmus. -optic neuritis, trigeminal neuralgia, facial myokymia (worm-like movements of face) -spastic bladder. -Lhermitte sign: flexion of cervical spine-> sensation of electricity running down spine -exacerbated sxs with heat ***earlier age of onset-> benin or relapsing remitting form

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MS diagnosis

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In a young, presumably healthy person, acut onset of sxs in CNS that are separated by time and space-> MS until proven otherwise -1 in 1000 ppl have in US -peak incidence of 24 yrs old. -MRI: bright lesions on T2 in corpus collosum and periventricular regions and cerebellar peduncle -CSF: ilevated IgG index, presence of oligoclonal bands, increased myelin basic protein. -Visual evoked responses (VER)-> view stimuli and measure response in occipital lobe-> reflects compromise in path b/w optic nerve and brain.

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MS treatment

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Primary treatment is IV steroids for acute attack. Immunomodulating agents for prevention: -IFNB-1a, IFNB-1b, and glatiramer acetate. others. can give baclofen for spasticity

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Optic neuritis

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idiopathic, inflammatory, infectious, or demyelinating optic neuropathy. -usually associated with acute, unilateral loss of visual acuity, visual fields, or both -90% have ocular pain, esp with eye movement -afferent pupillary defect-> dilated pupil in involved eye (CNII messed up) **strong association with MS, also with Sjogren, Guillain barre, and HIV TX: IV corticosteroids

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Chronic inflammatory demyelinating polyneuropathy (CIDP)

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essentially a chronic form of Guillan barre. acquired peripheral neuropathy. At onset have pattern of numbness and weakness in upper and lower extremities -> pain develops gradually over several weeks -symmetrical pattern. -often initially diagnosed with Guillain Barre or AIDP, but changed to CIDP when persist beyond 8 weeks. *predominantly affects spinal roots, major plexuses, and proximal nerve trunks **progressive symmetric or asymmetric polyneuropathy that is relapsing and remitting or progresses for >2 months -increased CSF protein, cytoalbuminologic dissociation TX: corticosteroids, IVIG, plasma exchange, immunosuppressive drugs

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Guillain-Barre syndrome aka AIDP

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Most pts experience proximal lower extremity weakness and ascending paralysis, usually following GI infection. arreflexia. Can rapidly worsen to respiratory failure and cardiac arrhythmia and autonomic instability -85% recover completely TX: IVIG and plasma exchange

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adrenoleukodystrophy

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adrenal dysfunction and progressive degenerative disease of white matter.. -limb ataxia, nystagmus, MR, low serum cortisol. X-linked type: ATP-binding transporter problem in the system for long-chain fatty acid metabolism.

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canavan disease

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AR spongy degeneration of infancy. Demyelinating disease that produces developmental regression at about 6 months and retardation in infants, results in macrocephaly (increase in brain volume and weight) -infant has extensor poturing and rigidity. N-acetlyaspartic acid metabolism is the prob and is detectable in urine.

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Dx of polymyositis and dermat omyositis

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CK-> dramatically elevated elevated aldolase, LDH, AST, ALT -anti-Jo-1 and other abs. -EMG/NCS->irritative or inflammatory myopathy -muscle bx-> chronic inflamm, fibrosis, net loss of myofibrils -in dermatomyocytis, lymphs are perivascular/perifascicular -in polymyocitis, lymphs are within fascicles

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polymyositis

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more common than dermatomyositis. More gradual onset, generally begins in teens. -difficulty swallowing, muscle tenderness, decreased muscle function -progressive, painless symmetrical proximal muscle weakness. -can get pharyngeal involvement. -fever, fatigue wt loss. -10% get intersititial pneumonitis -cardiac involvement-> congestive heart failure. -increased risk malignancy Tx: immunosuppressants (prednisone, methotrexate, azathioprine), IVIG -PT, speech therapy

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Dermatomyositis

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15% of cases are paraneoplastic with tumors such as lung, ovaries, GI, breasts, etc. Thus adults with dermatomyositis should get Ca workup, especially men onset 5-25 and 40-60 -elevated CPK -proximal muscle weakness and pain and a heliotrope rash (lilac colored periorbital rash) around both eyes, trouble swallowing -calcinosis cutis-> dystrophic calcifications in soft tissues and muscles->skin ulceration -flat-topped raised nonpruritic erythematous rash across knuckes and base of nails -anti-Jo-1 antibody -reynaud phenomenon Tx: immunosuppressants (prednisone, methotrexate, azathioprine), IVIG -PT, speech therapy

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Duchenne dystrophy

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X-linked, elevated CPK, calf pseudohypertrophy, Growers sign. Absence of dystrophin protein (defective in Beckers) -proximal muscle weakness-> gait instability (toe walking) -rarely survive past adolescence -slightly below average intelligence -cardiac muscle commonly affected dx: muscle bx and/or genetic testing. EMG/NCS will show myopathy (abnl muscle fxn) Tx: control sxs, supportive. Corticosteroids slow muscle degeneration, anticonvulsants control some muscle activity, immunosuppressants delay damage to dying muscle cells.

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Amyotropic Lateral sclerosis

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PURE MOTOR sxs with insidious onset dx requires upper and lower MN signs, in at least 3 regions. do EMG/NCS, MRI to r/o cervical myelopathy -loss of anterior horn cells (lower motor neurons) in spinal cord and motor nuclei in brainstem (upper motor neuron) -loss of large motor neurons in frontal cortex -degeneration of corticospinal tract. -muscle wasting, weakness, spasticity, muscle fasiculations **signs of brainstem disease such as early involvement of muscles supplied by CN--> bad prognosis ~1yr -10% hereditary (SOD1), 90% sporadic. Gain of toxicity rather than loss of function Tx: riluzole-> decreases release of glutamate (decreased excitotoxicity) supportive care

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Sarcoidosis

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may cause neuropathy and myopathy with noncaseating granulomas

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ataxia telangiectasia

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telangiectasias over malar eminences, conjuctivae, and ears

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Myotonic dystrophy

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problems with relaxing grip, hypersomnolence, premature baldness, testicular atrophy, and cataracts. -cardiac defects-> require pacemaker implantation -EMG shows repetitive discharge with minor stimulation

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polymyalgia rheumatica

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-arthritis of elderly (improbable in someone <60). -markedly elevated sedimentation rate, anemia, weight loss, malaise, -normal CPK

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EtOH effects on brain

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loss of Purkinje cells in the superior vermis of the cerebellum and atrophy

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Triorhocresyl phosphate

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Organophosphate found in rat poisons and roach powder.. Damages upper and lower motor neurons sxs are severe and may lead to death due to bronchospasm and diaphragmatic paralysis -early sxs= headache, vomiting, abd cramps, excessive sweating, wheezing, twitching tx: atropine + pralidoxime

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Lead poisoning

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-ataxia and tremor in children at low doses -chronic exposure-> impairs psychomotor dev and may lead to retardation -causes Brain edema in infancy that can lead to herniation. In adults: bilateral neuropathies, especially radial nerves.-> wrist and finger drops + sensory abnormalities -abd pain, constipation, anemia, basophilic stippling of erythrocyte precursors, lead lines on ginfiva. Tx: penicillamine

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Mercury poisoning

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-in dyes used to cure felt, paper/pulp plants sx: **personality changes, tremor, ataxia extensive dmg to granular cells of cerebellum.. Calcarine cortex of occipital lobe-> constriction of visual feilds

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Arsenic poisoning

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Tonic-clonic seizures, encephalopathy. -hemolysis, mucosal irritation -polyneuropathy -white lines across fingernails -drowsiness -death

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Ergot

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vasoconstricting agent from rye fungus. Get contamination of bread in less developed countries-> degeneration of the posterior columns and dorsal roots. Peripheral neuropathy.

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Manganese poisoning

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Manganese, inhaled by miners. Similar picture to Wilsons disease. **Parkinsonism -shuffling gait, tremor, drooling, cog-wheel rigidity -neuronal loss in the globus pallidus, putamen, caudate, hypothalamus, and cerebellum

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Ciguatoxin

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toxin in fish. Increased permeability of voltage-gated Na channels-> increased excitabiltiy/ sxs: abd discomfort, N/V, diarrhea, parasthesias, headache, fatigue, ataxia, myalgias. **temperature reversal (cold things feel hot) tx: Iv mannitol

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tick paralysis

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affects young children. -rapid ascending paralysis. toxin=holocyclotoxin-> interferes with presynaptic release at NMJ

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myasthenia gravis

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proximal muscle weakness, ptosis -fatigue with repetitive stimulation, improvement with rest -skeletal muscle weakness affecting ocular, facial, bulbar, respiratory, and proximal limb muscles dx: antiacetylcholine receptor antibodies, EMG **TFTs should always be done as concomittant thyroid disease is often seen **CT and MRI of the mediastinum-> r/o thymoma Tx: -Immunosuppressive agents: corticosteroids, cyclosporine, azathioprine, IVIG, plasmaphoresis -anti-cholinesterase (pyridostigmine)-> treats sxs but not disease.

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Inclusion body myositis

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-Mot common acquired muscle disease in people>50 More gradual onset of weakness over years. Mostly proximal, asymmetric. Atrophy of deltoids and quads -can have peripheral neuropathy with loss of DTRs. -CK levels elevated, but not as strikingly as poly/dermatomyositis - muscle cells have eosinophilic cytoplasmic inclusions, vacuoles rimmed with basophilic granules, and foci that stain + for congo red

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Lissencephaly

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Lissencephaly=smooth brain Severe mental retardation, recurrent seizures, infantile spasms, microcephaly -not capable of progressing beyond the 3- to 6-month level of milestones. Due to abnormal neuronal migration during early neural development CHROMOSOME 17p13.3 DELETION -craniofacial dysmorphologies: high forehead with vertical wrinkling, bitemporal hollowing, wide spaced eyes, flattened ears, short nose with upturned nares -hypotonia, contractures,cryptorchidism, omphaloceles. -often hs of polyhydramnios, intrauterine growth restriction. Tx: supportive. Prednisone + ACTH for spasms, anticonvulsants, feeding tube, PT

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Sturge-Weber syndrome

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mental retardation, homonymous hemianopsia, and hemiparesis. Infantile spasms, epilepsy, calcifications of the cerebral cortex in a railroad track appearance -port wine spot over face. -cataracts

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von Hippel -Lindau

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AD -erythrocytosis+ cerebellar signs= hemangioblastoma - microscopic hematuria (PCKD), renal carcinoma -hepato-splenomegaly -retinal angiomas (telangiectasias)

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Abetalipoproteinemia

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cant absorb cholesterol-> no chylomicrons or VLDL.-> cant make myelin. -abnl shaped RBCs, very low cholesterol and TGs. -posterior column and spinocerebellar tract degeneration. -loss of position sense, +Babinski. -retinitis pigmentosa Tx: Vitamin E may slow progression

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tuberous sclerosis

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AD with variable penetrance. -adenoma sebaceum- over malar eminences, may evolve to angiofibromas of the skin. -shagreen patch -ash-leaf spot -retinal phakomas= gliomatous tumors -periventricular tubers (subependymal glial nodules) -CNS calcifications -renal tumors -cardiac rhabdomyomas -seizures (infantile spasms- tx with ACTH) -65% have MR

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ataxia telangiectasia

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facial telangiectasia

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Dandy Walker malformation

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cerebellar elements not fused midline

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Holmes-Adie pupil

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solitary unreactive pupil in otherwise healthy person with decreased deep tendon reflexes dx: pupillary constriction with application of pilocarpine

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red glass test

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the eye is not moving properly in the direction to red image appears to be. -the outside image is always the false image

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menieres

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affects low tones -episodes= hours - days tx salt restriction and diuretics

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