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Flashcards in Neuro Deck (74)
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1

Sturge-Weber Syndrome

Somatic activating mutation in the GNAQ gene that causes developmental anomaly of neural crest derivatives.
Port Wine stain (bv derangement) V1/V2 distribution-->Ipsilateral leptomeningial angioma--> seizures/epilepsy, intellectual disability
Episclearl hemangioma -->Increase IOP --> early onset glaucoma
STURGE (Sporadic; Tram track calcifications; Unilateral port wine stain; Retardation; Glaucoma/GNAQ gene; Epilepsy)

2

Tuberous sclerosis

HAMARTOMAS:
Hamartomas in the CNS and skin
Angiofibromas
Mitral regurg
Ash leaf spots
Cardiac Rhabdomyomas
Tubserous sclerosis
Autosomal dOminant
Mental retardation
renal Angiomyolipoma
Seizsures/Shagreen patches

- Increased risk of subependymal astrocytomas
- ungual fibroms (nail growth)

3

Neurofibromatosis type 1 (von Recklinghausen disease)

Cafe au lait spots
Lisch nodules
Cutaneous neurofibromas (derived from neural crest cells)
Optic gliomas
Pheochromacytomas
NF1 mutation, chromasome 17, neurofibrim (negative regulator of Ras)

4

Von Hippel Lindau disease

Chromosome 3, VHL gene,
Hyperchromatic nuclei
Hemangioblastomas of cerebellum, spinal cord, brainstem and retina
Angiomatosis (cavernous hemangiomas of the skin, mucosa and organs)
Pheochromacytomas
Bilateral renal cell carcinoma

5

Charcot Marie Tooth Disease

Hereditary motor and sensory neuropathy
AD
Defect in production of proteins involve in the structure of peripheral nerves and the myelin sheath
Associated with scoliosis and foot deformities

6

Metachromatic leukodystrophy

AR Lysosomal storage disease
Arylsulfatase A deficiency that inhibits ability to degrade myelin and thus turn over myelin
Sulfatides build up
Central and peripheral demyelination with ataxia and dementia

7

Krabbe disease

AR lysosomal storage disease
Deficiency is galatocerebrosidase
Build up of galatocerebroside and psychosine destroys myelin sheath
Peripheral neuropathy, developmental delay, optic atrophy, and globoid cells

8

Adrenoleukodystrophy

X linked
Impaired addition of coenzyme A to long chain fatty acids and thus disruption of metabolism
Long chain fatty acids build up in the nervous system, the adrenal glands, and the testes
Long term coma and death, adrenal crisis

9

Aphasia

inability to speak (cortical)

10

Dysarthria

loss of motor function to speak

11

Where is Broca's area?

Inferior front gyrus of the frontal lobe

12

Where is Weirnke's area?

Superior temporal gyrus of the temporal lobe

13

Conduction aphasia

Damage to arcuate fasiculus, can't repeate
"no ifs, ands or buts"

14

What is the difference between Broca's and transcortical motor aphasia?

Broca's has impaired repetitive with nonfluent speach and intact repitition whereas transcortical motor has nonfluent speach with preserved repitition and comprehension (same pattern for Wernicke's)

15

Kluver Busy syndrome:

Damage to the amygdala associated with HSV1 producing inhibited behavior like hyperphagia, hyperorality, hypersexuality

16

Gerstman syndrome

Damaged to the dominant parietal-temporal cortex (angular gyrus near the parietal-temporal lobe junction) resulting in dysgraphia, dyscalcula, finger agnosia, left right disorientation

17

Ramsay hunt syndrome

VZV in the geniculate gyrus of the facial nerve (facial nerve paralysis, pain in the ear, loss of taste to the anterior 2/3 of the tongue and a rash on the ear canal, tongue, and hard palate

18

Ddx for a facial nerve palsy/Bells

Lyme disease
HSV1
VZV / Ramsay Hunt syndrome
Sarcoidosis
Diabetes
Tumors
Hermes eats Lymes at the Zoo and Stares Directly at the Teradactyls

19

What levels does the Artery of Adamkewitz help perfuse?

T8-L4 (collateral circulation)
Makes mid thoracics T4-T8 ish suceptible to infarct

20

Meninere's disease

idiopathic disorder affecting people 30-60 years old; presents with the triad of sensorineural hearing loss, vertigo lasting hrs with nystagmus, and tinitus. Hearing loss often progresses to low frequency permanent loss in both ears. Results of an increase in the volume of endolymph possibly due to malfunction of the endolymphatic sac (responsible for the filtration and excretion of endolymph) 

21

Guillian Barre syndrome/Acute inflamatory demyelinating polyradiculopathy

Autoimmune destruction of Schwann cells resulting in symmetric ascending muscles weakness and paralysis with facial paralysis in 50% of the cases. Can see concomitant sensory and autonomic (hypo/hypertension, cardiac issues). Most resolve spontaneously in weeks to months. Labs will show CSF with a normal cell count but elevated protein (albuminocytologic dissociation) which may cause papilledema. Assciated with Campy, HSV, and Orthomixovirus infections. Possible molecular mimcry pathway. Treat with respiratory support and plasmapheresis/IVIG

22

How to amino acids cross the BBB?

carrier facilitated transport

23

How do small molecules like CO2 and O2 cross the BBB?

simple diffusion

24

How does glucose cross the BBB?

carrier mediated transport

25

How do non polar/lipid soluble molecules cross the BBB?

diffusion

26

What areas in the brain have fenestrated capillaries?

Area postrema: toxin vomiting
Vascular organ of lamina terminalis: OVLT (third ventricle, sense osmolarity and transmits to pituitary for ADH release)
Neurohypothesis

27

Neurons

Permanent (don't divide in adulthood)
Nissle stain --> RER , can see cell bodies and dendrites, but not axons

28

Wallerian degeneration

Trauma to axon
Distal degeneration
Proximal retraction
PNS axons can regenerate

29

Astrocytes function

Physical support
K metabolism
Removal of excess neurotransmitter
BBB
Glycogen fuel reserve
Reactive gliosis in response to nerve injury (main component of glial scar)

30

What is the histological appearance of HIV encephalitis?

HIV infected microglia fuse to form multinucleated giant cells