Chapter 6 Movement

Question 1

Nuclei that are unique to the indirect pathway and result in disinhibition of GP interna resulting in increased thalamic inhibition.

Answer 1

GP externa and STN

Question 2

Direct pathway function

Answer 2

Does the work - promotes movement via D1 receptors

Question 3

Indirect pathway function

Answer 3

inhibits movement via D2

Question 4

MOA of ropinirole and pramipexole

Answer 4

D2 and D3 Agonists

Question 5

entacapone MOA

Answer 5

COMT inhibitor

Question 6

PD Rx that can cause dyskinesia

Answer 6

dopamine agnosists

Question 7

Gene implicated in hereditary PD

Answer 7

LRRK2

Question 8

Tongue protrusion dystonia, chorea, dx by blood smear

Answer 8

Neuroacanthocytosis

Question 9

Huntington’s disease

Answer 9

chrom 4, AD, CAG

Question 10

Primary generalized dystonia

Answer 10

chrom 9, DYT1, torsin A, AD

Question 11

Filipino with dystonia and PD

Answer 11

DYT3, Lubag, X-linked

Question 12

Dystonia in young girl with diurnal variation and PD

Answer 12

dopa responsive, AD, GCH1 on chrom 14

Question 13

Episodic Ataxia type 1

Answer 13

HCN1A facial twitching

Question 14

Episodic Ataxia type 2

Answer 14

CACN1A4 (FH migraine as well), nystagmus and dysarthria

Question 15

Neurotransmitter implicated in familial hyperkplexia

Answer 15

glycine

Question 16

high arched feet, scoliosis, neuropathy, ataxia, cardiomyopathy

Answer 16

Friedrichs ataxia, GAA, Frataxin on chrom 9, AR

Question 17

SCA type 3

Answer 17

CAG, AD, ataxia spasticity neuropathy

Question 18

ataxia with high AFP

Answer 18

Ataxia-telangiectasia and ataxia with oculomotor aprax type 2

Question 19

Fragile X tremor ataxia syndrome in older individual

Answer 19

grandchild with MRDD, CGG in FMR1 gene, T2 hyperintensities in cerebellum/inferior peduncle

Question 20

cerebrotendinous xanthomatosis

Answer 20

test for serum cholestanol, ataxia, cataracts and tendon xantomas

Question 21

PKAN

Answer 21

eye of the tiger sign (hypointensity surrounded but hyperintensity in the BG

Question 22

cerebrotendinous xanthomatosis

Answer 22

test for serum cholestanol, ataxia, cataracts and tendon xantomas, chrom 2 27 sterol hydroxylase

Question 23

Halo sign

Answer 23

hyperintensity in the cerebral peduncles seen in BPAN (beta-propellar protein- associated neurogeneration)

Question 24

Halo sign

Answer 24

hyperintensity in the cerebral peduncles seen in BPAN (beta-propellar protein- associated neurogeneration)

Question 25

potential etiology of hemifacial spasm

Answer 25

vascular loop around CNVII

Question 26

DRPLA (dentatorubro-pallidoluysian atrophy

Answer 26

CAG on chrom 12, asian descent

Question 27

Lesch-nyhan

Answer 27

HGPRT, x-linked

Question 28

low serum cholesteral and low vit E

Answer 28

abetalipoproteinemia

Question 29

spiculated red blood cells

Answer 29

acanthocytes

Question 30

Wilson’s

Answer 30

ATP7B on chrom 13

Question 31

PNKD with prolonged exercise (5-30 minutes potential gene

Answer 31

GLUT-1

Question 32

MOlecular layer of cerebellum

Answer 32

stellate and basket cells (inhibitory)

Question 33

Granular layer of cerebellum

Answer 33

granule cells and Golgi interneurons

Question 34

Purkinje cells

Answer 34

GABA inhibitory

Question 35

Treatment for Episodic ataxia II and III

Answer 35

acetazolamide

Question 36

Treatment for Episodic ataxia II and III

Answer 36

acetazolamide

Question 37

isolated cerebellar dysfunction, GI disease to test for

Answer 37

celiac dz

Question 38

inability to move eyes without head thrusts

Answer 38

Ataxia-telangiectasias - AR, ATM gene chrom 11, hypogammaglobuinemia

Question 39

SCA7

Answer 39

retinopathy

Question 40

striopallidodentat calcinosis

Answer 40

Fahr’s isease, PKism

Question 41

REM sleep behavior disorders seen in

Answer 41

SYNnucleinopathies (PK, LBD, MSA

Question 42

Tauopathies

Answer 42

CBD, PSP, FTD

Question 43

PK, dystonia, choreoathetosis

Answer 43

NBIAs (neurodegeneration with brain iron accumulation)