From Questions- Nervous system Flashcards by Katherine E Nordstrom

What is the immune disorder characterized by inflammation of a specific segment of the spinal cord?

transverse myelitis

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How can you differentiate transverse myelitis from guianne barre?

GBS is associated with LMN symptoms - flacid paralysis

transverse myelitis can present with UMN symptoms

BOTH commonly come after an infection

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First-line treatment & prophylaxis cluster headache?

treatment: 100% oxygen / sumatriptan
prophylaxis: verapamil

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Treatmetn & prophylaxis migraines?

treatment: triptan
prophylaxis: amitriptyline / valproate

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What braincancer is associated with VHL? It secretes what cytokine?

hemangioblastoma (in cerebellum)

secreting erythropoietin - leading ot polycythemia

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How can you differentiate bell’s palsy from AICA stroke?

AICA stroke - loss of pain & temp on one side of the face & opposite side of the body, vertigo, nystagmus, ataxia, ipsilateral Horner syndrome

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anti-NMDA antibody syndrome is most commonly associated with what neoplasm?

What other non-malignant condition?

ovarian teratoma

HSV encephalitis

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increased ammonia leads to what problems?

increased release of inhibitory neurotransmitters (GABA)

decreased release of excitatory neurotransmitters

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MOA of triptans?

5-HT1B & 1D agonists - decreases the release of CRGP & substance P - therefore causing vasoconstriction and diminished transmission of pain

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What are the 3 anatomic areas affected in neurosyphilis?

dorsal columns - loss vibratory & proprioception (ataxia)

cortex - dementia & behavioral changes

pretechtal area - argyll robertson pupils

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What is the most common site of ependyoma?

fourth ventricle

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What is the most common malignant primary bran tumor in children?

Histological findings?

medulloblastoma

rosettes - small blue tumor cells that surround a fibrotic core

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Histological findings of pilocytic astrocytoma?

eosinophilic, corkscrew fibers

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Glioblastoma multiorme arises from what cell type?

meningiomas?

glioblastoma: astrocyte
meningioma: arachnoid cells in the meninges

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What are the dopamine agonists that are used in the treatment of parkinsons?

ergot: bromocriptine

non-ergot: pramipexole & ropinirole

both are used to treat motor symptoms in patients with parkinsons

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Pattern of inheritance & chromosome affected in NFT1?

Cutaneous features?

Neurologic features?

Other features?

Inheritance
- 100% penetrance
- chromosome 17
- variable expresivity
Cutaneous
- cafe au lait
- cutaneous neurofibromas
- lisch nodules (pigmented hamartomas)
neurologic features
- optic gliomas
- meningiomas
- intellectual disability
other
- pheo
- bone lesions

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Pattern of inheritance & chromosome affected in NFT2?

Cutaneous features?

Neurologic features?

Other features?

Inheritance
- chromosome 22
cutaneous
- none
neurologic
- bilateral acoustic neuromas
- meningiomas
- gliomas
- neurofibromas
other
- cataracts
- scoliosis

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Describe the flow of sympathetic nerve fibers fromthe spinal cord to exiting the sympathetic column

intermediolateral columns of spinal cord

exit via the ventral nerve root

enter sympathetic chain via white ramus communicans

synapse or travel up/down

exit via grey ramus communicans

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Location of injury for damage to the common peroneal nerve vs tibial nerve? Symptoms?

Common peroneal - fibular head; problems w/ dorsiflexion & loss of sensation on dorsal portion of foot and lateral leg

Tibial - poplieal fossa; problems with flexing toes, inverting and plantar flexing foot + sensorly loss over sole of foot

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Clinical features & diagnostic criteria CJD?

onset 50-70, rapidly progressive dementia & myoclonus

elevated 14-3-3 protein titers & periodic sharp wave EEG

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What is bruxism & what stage of sleep does it occur?

Feature of this stage of sleep?

teeth grinding

K-complexes & sleep spindles (N2)

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What is the network of neurons that controls arousal & consciousness? What is its course?

reticular activatign system (RAS)

projects from midbrain, through thalamus & into cerebellum

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What is the best test to confirm prior infection in a patient suspected of tertiary syphilis?

fluorescent treponemal antibody absorption (FTA-ABs)

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Difference between simple & complex seizure?

complex - loss of consciousness

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Laboratory finding seen in the diagnosis of Alzheimer's disease?

diffuse atrophy of the cortex B-amyloid plaques (apple-green birefringence on polarized light) neurofibrillary tangles granulovacuolar degeneration & Hirano bodies

Difference in fundoscopic examination of a patient with central retinal arteyr occlusion & retinal vein occlusion?

* CRAO: * cloudy, pale retina * cherry-red spot on the fovea * RVO: * optic disc swelling * dilated veins * retinal hemorrhages * cotton wool spots

Typical presentation of a patient with giant cell (temporal) arteritis?

elderly female, presenting with new-onset and severe unilateral headache, fever, malaise, temporal tenderness & jaw claudication elevated ESR & normal CK

Metachromatic dystrophy is due to a deficiency in what enzyme? Symptoms?

arylsulfatase A discrete plaques of demyelination - sparing subcoritcal (U) fibers worsening motor fibers & intellectual disabillity

What should you give first: glucose or thiamine?

THIAMINE FIRST necessary for proper glucose metabolism, so if give glucose first, you can precipitate symptoms of Wernicke encephalopathy

What are the neurotransmitters affected in Huntington's? In what direction are they affected?

decreased GAMA & ACh Increased Dopamine

What are the neurotransmitters affected in Parkinson's? In what direction are they affected?

Increased ACh Decreased Dopamine & Serotonin

What are the neurotransmitters affected in Alzheimer's? In what direction are they affected?

decreased ACh

What are the neurotransmitters affected in Depression? In what direction are they affected?

decreased dopamine, serotonin, NE

What are the neurotransmitters affected in anxiety? In what direction are they affected?

decreased GABA & serotonin, increased NE

Lesions in the the following lobes result in what type of visual defecits bilaterally? parietal temporal occipital

parietal: interior quadrant temporal: superior quadrant occipital: macular sparing

What do you see in the CSF of a patient with MS?

Oligoclonal IgG bands elevated levels myelin basic protein

Key diagnostic features of MS

worsens w/ rising body temperatuer (working out) swelling of the optic disk w/c papilledema eye pain worsens w/ movement

What nerve passes anteriorly to the bifurcation of the common carotid? Posteriorly?

anterior - hypoglossal posterior - recurrent laryngeal nerve (branch of CNX)

What is the cause of internuclear ophthalmoplegia seen in MS? Symptoms?

d/t damage to the MLF conjugate gaze palsy & impaired adduction of the eye ipsilateral to the site of damage

Common presentation of a patient with damage to their inferior gluteal nerve?

trouble with hip extension, climbing stairs, or rising from a seated position MC after a fall/trauma that causes a posterior hip dislocation

What are the respective positions of the facial, vestibular & cochlear nerves within the fundus of the internal auditory meatus?

2 thin crests of bones to form 3 separate canals facial: superior cochlear: inferior vestibular: posterior

How can you assess thiamine (vit B1) deficiency?

erythrocyte transketolase activity

What is Foster Kennedy Syndrome?

meningiomas arising from the olfactory groove compression/atrophy ipsilateral optic nerve intracranial hypertension (optic disc swelling) olfactory degeneration

What are the 4 tumors associated with psomma bodies?

PSaMM papillary thyroid carcinoma serous ovarian carcinoma mesothelioma meningioma

Mutation in classical vs. vascular Ehlers Danlos Syndrome?

Classical - collagen type V (COL5A1, COL5A2) vascular - collagen type III (COL3A1)

Hearing loss secondary to recurrant otitis media is what type of hearing loss?

conductive hearing loss

Spinal level of inferior glueal nerve?

L5-S2

Where is the hypoglossal canal located?

posterior fossa superior & medial aspect of the occipital condyles

Genetic cause of Friedrich Ataxia? Causes what mutation?

GAA repeat on chromosome 9 oxidative damage d/t mitochondrial accumulation of iron

What are the tumors seen with VHL disease?

pheochromocytoma hemangioblastoma (retina, brain stem, cerebellum, spine) angiostomatosis bilateral renal cell carcinoma

Cardiac rhabdomyomas are seen in what genetic condition?

Tuberous sclerosis chromosome 16

major complication of acyclovir?

crystaline nephropathy | (AKI)

To prevent ODS, what rate shoudl serum sodium be corrected?

not excede 0.5mEq/L/hr

Mannitol is CI in patients with what condition?

congestive heart failure b/c it can lead to pulmonary edema b/c it causes water to move out of cells & into extracellular compartment

Why can pinealomas result in vertical gaze palsy?

they can compress the superior colliculus

What do you need to test a patient for before starting them on rituximab therapy?

JC virus - can precipitate PML

Histopathology seen in patients with frontotemporal lobe dementia? D/t what mutation?

hyperphosphorylated tau proteins (tau cytoplasmic inclusion bodies) mutation MAPT gene on chromosome 17

List the correct order of penetration by a needle for a lumbar puncture

skin subcutaneous tissue ligamentum flavum epidural space dura mater subdural space arachnoid space subarachnoid space

What lesions are associated with first neuron Horner syndrome?

pontine hemorrhage lateral medullary syndromes spinal cord lesion above T1

What lesions are associated with second neuron Horner syndrome?

compression of the stellage ganglion by a pancoast tumor (anterior to C7 transverse process)

What lesions are associated with third neuron Horner syndrome?

carotid dissection

What is the other name for Sturge-Weber synrome? It is caused by what genetic mutation?

encephalotrigeminal angiomatosis GNAQ- affects cappilary sized blood vessels in CNV1 & CNV2 distributions

Huntingtons has what type of anticipation? What is the repeat? On what chromosome?

paternal - sequence is less stable during spermatogenesis CAG repeat on HTT gene on chromosome 4