Lec 10: Disorders of the Basal Ganglia

Question 1

Function is to control and regulate the activities of the motor and the premotor cortical areas

Answer 1

Basal ganglia

Question 2

Dopamine is (inhibitory,stimulatory) to D1 and (inhibitory,stimulatory) to D2

Answer 2

excitatory to the D1 receptors and inhibitory to D2 receptors

Question 3

D1 receptor stimulation

Answer 3

stimulates the cortex

Question 4

D2 receptor stimulation

Answer 4

inhibits the cortex

Question 5

Slow, writhing, continuous, involuntary movements of distal muscles

Answer 5

athetosis

Question 6

The huntington gene has 23 CAG repeats upon investigation, this means what?

Answer 6

Patient is unaffected by huntington’s (9-33 unaffected, >40 leads to Huntington’s)

Question 7

Triad of Huntington’s Disease

Answer 7

Chorea, dementia, behavioral abnormalities

Question 8

What are the gross pathological findings in Huntington’s disease?

Answer 8

atrophy and gliosis of the caudate nucleus and putamen

Question 9

Which are NOT medications for chorea in Huntington's?
A. haloperidol
B. bromocriptine
C. tetrabenzene
D. Clonazepam

Answer 9

B. bromocriptine is for muscle rigidity and bradykinesia

Question 10

Most common cause of hemiballism

Answer 10

on the opposite subthalamic nucleus

Question 11

Most common cause of Bilateral ballismus

Answer 11

Nonketotic hyperosmolar coma

Question 12

Twisting, patterned movements that tend to be sustained at the peak

Answer 12

DYSTONIA

Question 13

Early (<26 years) onset of dystonia tends to (generalize/remain localized)

Answer 13

generalize

Question 14

What enzyme is deficient in Dopa-responsive dystonia?

Answer 14

tyrosine hydrolase

Question 15

Treatment for cervical dystonia

Answer 15

Botulinum toxin

Question 16

Type of tremor that results from lesions affecting the cerebellothalamic and nigrostriatal pathways

Answer 16

Holmes’ Tremor

Question 17

Diagnostic Criteria for XDP

Answer 17

1. DYSTONIA alone

2. PARKINSONIAN traits

Question 18

What phase of XDP? HYPERKINETIC/dystonic disorders

Answer 18

dystonia phase

Question 19

What phase of XDP? HYPOKINETIC movements

Answer 19

Parkinsonism Phase

Question 20

Best available treatment for XDP

Answer 20

Zolfidem

Question 21

6 cardinal features of parkinsonism

Answer 21

o Resting tremor
o Bradykinesia/hypokinesia/akinesia
o Rigidity
o Flexed posture of neck, trunk and limbs (brought about by rigidity)
o Loss of postural reflexes
o Freezing

Question 22

Hallmark of MSA

Answer 22

dysautonomia (ANS malfunction) where patients have orthostatic hypotension and impotence.

Question 23

Predominant symptom is cerebellar dysfunction
A. MSA-P
B. MSA-C

Answer 23

B. MSA-C

Question 24

Hot cross bun sign
A. MSA-P
B. MSA-C

Answer 24

B. MSA-C

Question 25

“Alien limb” phenomenon A. Corticobasal ganglionic degeneration B. Multiple system atrophy C. Idiopathic Parkinsons

Answer 25

A. Disturbance of movement and the cortical dysfunctions are clearly asymmetrically distributed in CBGD (isang kamay lang yung may ideomotor apraxia etc)

Question 26

Inborn error of copper metabolism with hepatic cirrhosis and basal ganglia damage

Answer 26

Wilson's disease

Question 27

What is the type of inheritance for Wilson's disease?

Answer 27

AR

Question 28

Best treatment for Wilson's disease

Answer 28

Liver transplant

Question 29

Diagnostic of Parkinson's disease except: a. Bradykinesia b. Rigidity c. Tremor d. Postural (proprioceptive) instability

Answer 29

D