Pretest

Question 1

cranial nerve palsy in sarcoid?

Answer 1

Cr VII

-same in Lyme disease

Question 2

mcc of fungal meningitis

Answer 2

cryptococcus
(encapsulated fungus)

-acquired from lungs and spreads to CNS hematogenously

Question 3

brain abscesses:

• most common organism?
• most common site?
• most common mechanism of spreading?

Answer 3

• Streptococcus
• gray-white matter junction
• hematogenous spread of infection

-Staph post-neurosurgery

Question 4

most common organism of brain abscess in AIDs pt?

Answer 4

toxoplasma gondii

Question 5

most common fungal brain abscess?

Answer 5

aspergillus

Question 6

rabies invasion of CNS?

Answer 6

• retrograde axoplasmic flow
• from periphery extending centripetally along peripheral nerves

-only supportive tx available

Question 7

source of CJD?

Answer 7

growth hormone treatment from cadaver pituitary gland

Question 8

cysticercosis

Answer 8

• taenia solium
• pork tapeworm
• hatch in GI –> spread to CNS –> multiple cysts in cerebrum

-feco-oral spread; S. America, SE Asia, Africa

Question 9

von Hippel Lindau syndrome

Answer 9

• autosomal dominant
• multiple tumors & cysts in different organs (CNS, kidneys, eye)
• hemangioblastoma* –> lethal if bleeds into brain
• clear cell renal carcinoma, pheochromocytoma, pancreatic tumors/ cysts

Question 10

brain metastases with highest mortality?

Answer 10

malignant melanoma

Question 11

common maligancies in AIDS pts?

Answer 11

• lymphoma (primary CNS tumor)

- Kaposi sarcoma (rarely mets to brain)

Question 12

gelastic seizure

Answer 12

uncontrollable laughter

-seen in hypothalamic hamartomas

Question 13

hyperCa –> dec membrane excitability –> what symptoms?

Answer 13

• lethargy
• weakness
• areflexia

Question 14

deficient enzymes of:

• Tay Sachs
• Gaucher
• metachromatic leukodystrophy
• Neimann-Pick

Answer 14

• hexaosaminidase A (Tay-Sax lacks hex)
• glucocerebrosidase
• arylsulfatase A
• sphingomyelinase (No man picks his nose with his sphinger)

Question 15

`things that precipitate subacute combined degeneration?

Answer 15

• giving folate before B12 (when both deficient)
• atrophic gastritis –> intrinsic factor deficiency
• nitrous oxide

Question 16

visual defect with B12 deficiency?

Answer 16

centrocecal scotoma

Question 17

chlordiazepoxide

Answer 17

• benzodiazepine

- tx alcohol withdrawal, anxiety

Question 18

alcohol vs benzodiazepine withdrawal symptoms

Answer 18

• alcohol: within 72 hours

- benzodiazepines: 7-10 days post abstinence

Question 19

pellagra

Answer 19

• B3 (nicotinic acid) deficiency

- dermatitis, diarrhea, dementia, death

Question 20

kwashiorkor

Answer 20

• protein-energy malnutrition

- symptoms: edema, irritability, anorexia, dematoses, enlarged liver

Question 21

Pickwickian syndrome = obesity hypoventilation syndrome

Answer 21

obesity + hypersomnia + hypoxemia + pulmonary HTN

Question 22

transcortical sensory aphasia

seen in what disease?

Answer 22

decreased ability to understand complex linguistic structures

-seen in Alzheimer’s; along with aphasia (dec fluency, dysnomia, etc)

Question 23

MPTP addiction causes what to brain?

Answer 23

• damage to substantia nigra –> Parkinson-like symptoms which develops over a few months (vs years in regular Parkinson’s disease)
• MPTP is similar to heroin

Question 24

chorea gravidarum

Answer 24

• chorea in a pregnant woman
• asymmetric, forceful movements
• caused by dramatic change of hormonal environment of brain

-can happen with estrogen replacement, as well

Question 25

Sydenham's chorea px

Answer 25

- after Group A beta-hemolytic Streptococcus infection - chorea - dysarthria, behavior changes, gait disturbance, slowed cognition, hypotonia - kids, girls - no genetic or MRI abnormalities

Question 26

substantia nigra in Parkinson's disease show what?

Answer 26

Lewy bodies | = intracytoplasmic inclusion bodies = eosinophilic inclusions

Question 27

flexion of neck causes electrical sensation to radiate down spine

Answer 27

Lhermitte's sign -seen in MS

Question 28

Canavan's disease px

Answer 28

- occurs in infants by 6 months - extensor posture - rigidity - myoclonic seizures - spongiform change to white matter

Question 29

abetalipoproteinemia px, cause

Answer 29

- dec LDL, VLDL, chylomicrons - acanthocytes - ataxia - posterior column & spinocerebellar degeneration - retinitis pigmentosa -MTP mutation --> impaired VLDL formation --> dec vit E delivery & dec fat absorption

Question 30

Sturge-Weber syndrome px

Answer 30

- port wine stain* - glaucoma - seizures - mental retardation - abnormal blood vessels* - ipsilateral leptomeningeal angioma - tram track calcifications*

Question 31

phenylketonuria

Answer 31

- autosomal recessive - phenylalanine hydroxylase deficiency --> lack of tyrosine & accumulation of phenyl-alanine & -ketones - some may restrict dietary phenylalanine intake - px: seizures, intellectual delay, musty body odor

Question 32

Hartnup's disease

Answer 32

- tryptophan malabsorption --> nicotinamide (vit B3) deficiency - autosomal recessive - ataxia - scaly erythematous rash

Question 33

telangiectasias in fundi on retinal exam - what disease?

Answer 33

von Hippel Lindau -numerous tumors and cysts

Question 34

first trimester viral infection can lead to?

Answer 34

aqueductal stenosis --> hydrocephalus

Question 35

tuberous sclerosis

Answer 35

- ash leaf spots - shagreen patches - adenoma sebaceum (malar distribution) - CNS calcifications (calcified subependymal glial nodules --> hydrocephalus) - renal tumors - cardiac rhabdomyomas - seizures - retinal phakomas (no tx needed) - autsomal dominant

Question 36

fetal alcohol syndrome px

Answer 36

- intra- & post-uterine growth retardation - dysmorphic facies - CNS: brainstem/cerebellum malformation, learning disability, microcephaly, hetrotopia (cortical neurons in abnormal locations)

Question 37

dermatomyositis px

Answer 37

- proximal muscle weakness - heliotrope rash - erythema over knuckles -paraneoplastic syndrome

Question 38

DMD carrier will have elevations of what in serum?

Answer 38

creatinine phosphokinase (CPK)

Question 39

DMD can be seen in females with what genetic disorder?

Answer 39

Turner syndrome (XO)

Question 40

ALS

Answer 40

- UMN & LMN symptoms --anterior horn cells, corticospinal tract, brainstem motor nuclei, etc - fibrillations - denervation atrophy of muscles -bad prognosis: if cranial nerves affected --fasciculations of tongue (Cr XII)

Question 41

Parinaud syndrome

Answer 41

- paralysis of upward gaze - -> downward gaze primary position -pinealoma association