Movement

Question 1

Papez

Answer 1

MATCH mamillary, ant thal, cingulate, hippocampus

Question 2

Ach

Answer 2

Meynert

Question 3

Norad

Answer 3

Ceroulus

Question 4

Serot

Answer 4

Raphe

Question 5

Dopa

Answer 5

nigra pars compacta

Question 6

GABA

Answer 6

accumbens, nigra pars reticulata

Question 7

Friederichs

Answer 7

AR GAA repeat in frataxin ch9, neuropathy + UMN + cerebellar, high arch feet, spine deformity, cardiac (coQ10 analogue idebenone treats)

Question 8

SCA

Answer 8

AD most, CAG repeat expansion, progressive truncal and limb ataxia, UMN/spasticity, MRI atrophy, SCA3 (+, ataxin 3 CAG, bulbar sx, dopa resp park), SCA7 (vision loss)

Question 9

Fragile X-tremor-ataxia sx

Answer 9

CGG repeat FMR1, ataxia, parkinsonism (like MSA-C), retard grandson, T2 hyper inf cerebellar peduncles

Question 10

Entacapone

Answer 10

COMT inh (inc dopa half life) [liver]

Question 11

ropinirole, pramipexole

Answer 11

Dopa agonists (D2, D3), [impulse control, sedation]

Question 12

rasagiline, selegiline

Answer 12

MAOB inh (dec dopa metab), insomnia

Question 13

trihexyphenidyl, benztropine

Answer 13

Anticholinergics (for tremor only), cognitive

Question 14

presenilin-1 and 2

Answer 14

(ch14 early aggressive) ch1 early

Question 15

AD path

Answer 15

amyloid plaque (extracellular), neurofib tangles (intraneuronal tau), granulovacuolar degeneration, Hirano bodies (actin cytoplasm inclusion)

Question 16

AD CSF

Answer 16

low amyloid, high tau and p-tau

Question 17

Huntington ch

Answer 17

ch4

Question 18

Dentatorubral-pallidoluysian atrophy

Answer 18

AD CAG repeat ch12, myoclonus, choreoathetosis

Question 19

Lesch-Nyhan

Answer 19

X HGPRT, hyperuricemia, kidney stones, psych, choreatetosis, self mutilation

Question 20

Dopa-responsive dystonia

Answer 20

GCH1 or tyrosine hydroxylase def

Question 21

Paroxysmal dyskinesias

Answer 21

kinesigenic (worse startle/hypervent, AED carbamazepine), nonkinesigenic (worse etoh/caf/fatigue), exertional (exercise, GLUT-1 mut high CSF gluc, keto)

Question 22

EA1

Answer 22

KCNA1 facial twitch, myokimia, AEDs

Question 23

EA2

Answer 23

CACN1A4 same as hemiplegic migraine, brainstem sx, acetazolomide, 4-aminopyridine

Question 24

Kluver Bucy

Answer 24

b/l ant temp/amygdala, hyperoral + sensitive to environment + blunted + hypersex + visual agnosia

Question 25

Pure word deafness

Answer 25

impaired auditory language comprehension, normal comprehension of reading (compared to wernickes), b/l sup temporal gyri (between Wernickes and Heschl's auditory cortex)

Question 26

Gerstman

Answer 26

finger agnosia + r/l + acalculia + agraphia, dominant inf parietal (angular gyrus)

Question 27

Transcortical sensory

Answer 27

Wernickes with intact repetition, thalamic aphasia, watershed MCA-PCA dominant

Question 28

Transcortical motor

Answer 28

broca with intact repetition, watershed ACA-MCA, disconnect from broca to supl motor

Question 29

Aphemia (pure word mutism)

Answer 29

no fluency, impaired repetition, intact auditory comprehension, writing and reading, dominant frontal operculum above broca