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A-Step 1 > Neuro > Flashcards

Neuro Flashcards

1
Q

Why is there so much variability in the expression of mitochondrial diseases?

A

normal and abnormal diseases are randomly distributed between daughter cells in mitosis (heteroplasmy)

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2
Q

Name three mitochondrial syndromes

A
  1. Leber hereditary optic neuropathy
  2. Myoclonic epilepsy with ragged-red fibers
  3. mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes (MELAS)
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3
Q 
child
cognitive impairment
behavioral problems
mild anemia
lives in old house
A

lead poisioning

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4
Q

Enzymes most sensitive to lead inhibition

A

delta-aminolevulinate dyhydrase and ferrochelatase (zinc containing)
Causes delta-ALA and proroporphyrin IX to accumulate, decreased production of heme

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5
Q

Pathway that N. meningitidis uses to colonize CNS:

A

Nasopharynx-> mucosal epithelium -> bloodstream -> choroid plexus ->CNS via BBB

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6
Q

Location of Foramen of Monro

A

between lateral ventricles and third ventricle

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7
Q

Location of cerebral aqueduct

A

between third ventricle and fourth ventricle

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8
Q

Openings between fourth ventricle and subarachnoid space:

A

lateral foramina of Luschka and medial foramen of Magendie

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9
Q

How does CSF return to venous circulation

A

arachnoid villi

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10
Q

Noncommunicating hydrocephalus

A

caused by obstruction of CSF flow

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11
Q

Communicating hydrocephalus

A

blockage of subarachnoid space, not within brain or brainstem
from fibrosis following inflammation

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12
Q

Normal pressure hydrocephalus

A

CSF is not absorbed by arachnoid villi

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13
Q

Hydrocephalus ex vacuo

A

Increased CSF volume occurring in conditions of brain atrohpy

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14
Q

Psuedotumor cerebri

A

increased intracranial pressure in overweight young women. Related to decreased CSF outflow at arachnoid villi

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15
Q

Causes of progressively weakening diaphragmatic contractions (intact phrenic nerve stimulation)

A

NMJ pathology (myasthenia gravis) or rapid diaphragmatic muscle fatigue (restrictive lung disease)

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16
Q

fever and muscle rigidity after surgery under general anathesia
tachycardia, hypertension, hyperkalemia, myoglobinemia

A

Malignant hyperthermia. Caused by excess free calcium in the cytoplasm of muscle fibers (defect in ryanodine receptor). Ramps of action of sarcoplasmic reuptake of Ca (ATP dependent-> generates heat)

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17
Q

What drug is used to reverse malignant hyperthermia?

A

Dantrolene- muscle relaxant that acts of ryanodine receptors preventing further release of Ca into the cytoplasm.

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18
Q

Tetrodotoxin

A

neurotoxin in pufferfish. Binds to voltage gate sodium channels in nerve and cardiac tissue, preventing depolarization

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19
Q

Most common symptoms of MS

A

optic neuritis
internuclear opthalmoplegia
sensory deficits
Worsen with heat exposure

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20
Q

Enzyme deficiency in Niemann-Pick

A

sphingomyelinase

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21
Q

Urinary incontinence
ataxic gait
dementia

A

Normal pressure hydrocephalus (wacky, wobbly, wet)

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22
Q

CT findings in normal pressure hydrocephalus

A

symmetric dilitation of ventriculi

23
Q

Light reflex findings when one optic nerve is damaged

A

Light in effected eye will not cause contstriction of either pupil (optic nerve can’t carry info away)
Light in unaffected eye will cause constriction of both pupils (occulomotor nerves still function)

24
Q

What happens at sites of irreversible neuron injury?

A

Astrocytosis (glial cells that form scar that compensates for volume loss)

25
Q

Which vitamins are fat soluble?

A

ADEK

26
Q

Which vitamin deficiency causes neuromuscular disease and hemolytic anemia?

A

Vitamin E (functions to protect fatty acids from oxidation. Deficiency allows damage to neurons and RBCs)

27
Q

Cerebellar ataxia
Telangectasia
increased risk of sinopulmonary infections

A

ataxia telangiectasia:
AR
defect in ATM (DNA repair) gene
Causes IgA deficiency

28
Q

Function of kinesin

A

transport of neurotransmitter containing secretory vesicles to synaptic terminals

29
Q

Spastic rigidity
hyperreflexia
paresis

A

Upper motor neuron lesions. Can be located in corticospinal tract, internal capsule (posterior limb), and primary motor cortex.

30
Q

hemiballism

A

wild, flinging movements of the extremities on one side (contralateral to lesion) of the body. Occurs due to damage of the subthalamic nucleus (part of basal ganglia)

31
Q

What comprises the wall of a cerebral cystic cavity?

A

astrocytes (gliosis)

32
Q

Cause of neonatal tetanus

A

infected umbilical stump

33
Q

When can a child get their first tetanus vaccine?

A

Two months

34
Q

What molecules are derived from POMC?

A

beta-endorphins
ACTH
MSH

35
Q

How does tetanospasmin (tetanus toxin) travel through the body?

A

motor neuron (retrograde transport) -> spinal cord-> inhibition of inhibitory interneurons -> unregulated firing on primary motor neurons

36
Q

Two brain tumors that arise in the cerebellum

A

pilocytic astrocytomas and medullobastomas

37
Q

tumor composed of sheets of small cells with basophilic nuclei and scant cytoplasm (small, round, blue). Abundant mitoses

A

Primitive neuroendocrine tumors. Include Medullobastomas

38
Q

Amniocentesis findings in neural tube defect

A

High Alpha-fetoprotein and acetylcholinesterase.

39
Q

Confusion/apathy/inattentiveness
ataxia
opthalmoplegia (horizontal nystagmus)
damage to mamillary bodies

A

Wernicke encephalopathy

Caused by glucose infusion in a patient with a thiamine deficiency

40
Q 
flu-like prodrome
agitation
disorientation
pharyngospasm
photophobia
followed by flaccid paralysis
Exposure to wild mammals
A

Rabies encephalitis

41
Q

How do rabies viruses bind to cells?

A

Bind to nicotinic acetylcholine receptors

42
Q

Signs of acute Vitamin A toxicity

A

nausea
vomiting
vertigo
blurred vision

43
Q

Signs of chronic Vitamin A toxicity

A 
alopecia
dry skin
hyperlipidemia
hepatotoxicity
HSM
visual difficulties
intracranial hypertension
44
Q

Signs of niacin deficiency

A

3D’s of pellagra: dementia, dermatitis, diarrhea

45
Q

Schizophrenic symptoms lasting 1-6 months

A

Schizophreniform disorder

46
Q

Substance that causes the most overdose deaths in the US

A

Opioid analgesics

47
Q

Vertical diploplia

A

Double vision when looking towards the nose. USually caused by palsy of trochlear nerve (CNIV)

48
Q

Total sensory loss on one side of the body (no motor dysfunction)

A

Contralateral thalamic syndrome

49
Q

Cause of lacunar infarcts

A

small vessel lipohyalinosis and atherosclerosis involving penetrating vessels supplying deep brain structures

50
Q

Risk factors for lacunar infarcts

A

DM and uncontrolled hypertension

51
Q

Stimulus for release of acetylcholine into NMJ

A

Calcium influx into nerve terminal

52
Q

Blotchy red fiber on Gomori trichrome stain

A

mitochondrial myopathies. Because abnormal mitochondria accumulate under the sarcolemma

53
Q

Tumor at cerebellopontine angle
S-100 positive
around cranial nerve (especially VIII)

A

Schannoma

54
Q

What two types of cancers are S-100 positive

A

Schwannoma and Melanoma

A-Step 1 (15 decks)

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