Neuro X Flashcards by Ned Jones

deficient enzyme in Krabbe disease

galactocerbosidase

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what builds up in krabbe’s?

1) galactocebroside

2) psychosine

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Krabbe pathophys

galactocerboside + psychosine destroy myelin sheath.

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findings in Krabbe’s

1) peripheral neuropathy
2) developmental delay
3) optic atrophy
4) globoid cells

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Metachromatic leukodystrophy pathophys

Buildup of sulfatides leads to impaired production and destruction of myelin sheath.

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Findings in metachromatic leukodystrophy

1) central and peripheral demyelination with ataxia and dementia

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PML epidemiology

2-4% of AIDS patients

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What drugs confer increased risk for PML?

1) natalizumab

2) rituximab

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adrenoleukodystrophy complications

Long-term coma/death

- adrenal gland crisis

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adrenoleukodystrophy pathophys

buildup of very-long-chain fatty acids in nervous system + adrenal gland + testes

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General characteristics of seizures

Synchronized, high-frequency neuronal firing

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Most common origin of partial seizures

medial temporal lobe

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partial seizure common scenario

preceded by seizure aura.

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Simple partial vs. complex partial focal seizures

consciousness intact with simple, impaired in complex

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status epilepticus cutoff

5 minutes

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Most common causes of seizures in children…

1) genetic
2) infection (febrile)
3) trauma
4) congenital
5) metabolic

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Most common causes of seizures in adults…

1) tumor
2) trauma
3) stroke
4) infection

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Most common causes of seizures in elderly…

1) stroke
2) tumor
3) trauma
4) metabolic
5) infection

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generalized seizures

1) absence
2) myoclonic
3) tonic-clonic
4) tonic
5) atonic

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absence seizure characteristics

1) 3 Hz
2) no postictal confusion
3) blank stare

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tonic clonic (grand mal) presentation

alternating stiffening and movement

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only headache that’s more common in men?

cluster

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cluster duration

15 min-3 hr; repetitive

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cluster associated with?

horner syndrome

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acute cluster treatment

sumatriptan + 100% O2

cluster prophylaxis

verapamil

typical tension headache duration

Greater than 30 minutes, typically 4-6 hrs. constant.

treatment for chronic pain associated with tension headaches?

amitriptyline

migraine duration

4-72 hours

migraine pathophys

Due to irritation of CN V, meninges, or blood vessels.

Molecules related to migraines

Release of substance P, calcitonin gene-related peptide, vasoactive peptides.

acute migraine treatment

1) NSAIDs 2) triptans 3) dihydroergotamine

migrane prophylaxis

1) lifestyle changes (sleep, exercise, diet) 2) beta blockers 3) calcium channel blockers 4) amitriptyline 5) topiramate 6) valproate

trigeminal neuralgia duration

less than 1 minute usually

more common type of vertigo?

peripheral vertigo

peripheral vertigo pathophys

inner ear etiology (semicircular canal debris, vestibular nerve infection, Meniere disease).

How do you test for peripheral vertigo?

positional testing leading to delayed horizontal nystagmus.

central vertigo pathophys

Brain stem or cerebellar lesion (eg stroke affecting vestibular nuclei or posterior fossa tumor).

Findings in central vertigo

1) directional change of nystagmus 2) skew deviation 3) diplopia 4) dysmetria

How do you test for central vertigo?

Positional testing --> immediate nystagmus in any direction; may change directions. Focal neurologic findings.

Other name for Sturge-Weber syndrome

Encephalotrigeminal angiomatosis

Sturge-Weber pathophys

Congenital, *non-inherited (somatic) developmental anomaly of neural crest derivates due to activating mutation of GNAQ gene. **sporadic

blood vessels affected in storage-weber

small (capillary-sized) blood vessels.

nevus flammeus

non-neoplastic "birthmark" in CN V1/V2 distribution

other impt findings in sturge-weber

1) ipsilateral leptomeningeal angioma --> leading to seizures/epilepsy 2) early onset glaucoma

pathophys of early onset glaucoma in spurge-weber?

episcleral hemangioma --> increased IOP --> early-onset glaucoma.

What are tram track calcifications?

bright spots on imaging on opposing gyri.

Increased incidence of what in tuberous sclerosis?

1) subepndymal astrocytomas | 2) ungual fibromas

cardiac complication of tuberous sclerosis?

Mitral regurgitation

other name for NF1?

von Recklinghausen disease

origin of neurofibromas

neural crest cells

mutated tumor suppressor gene in NF1?

neurofibromin

neurofibromin action

negative regulator of RAS

hemangioblastoma description

high vascularity tumors with hyperchromatic nuclei.

where do hemangioblastomas occur in VHL?

1) retina 2) *brain stem 3) cerebellum 4) *spine

other finding in VHL...

angiomatosis

angiomatosis?

cavernous hemangioma in skin, mucosa, organs

GBM tumor type?

grade IV astrocytoma

GBM staining

GFAP (astrocytes)

GBM prognosis

1-year median survival

meningioma origin

arachnoid cells

meningioma characteristics

1) extra-axial (external to brain parenchyma) | 2) may have dural attachment ("tail")

meningioma management

resection and/or radiosurgery

meningioma presentation

1) often asymptomatic | 2) seizures or focal neurologic signs

meningioma pathology

1) spindle cells concentrically arranged in a whorled pattern 2) psammoma bodies