Other Movement Disorders

Question 1

Possible etiologies of dystonia

Answer 1

• Idiopathic
• Genetics
• Drug-induced

Question 2

HD gene

Answer 2

Located on chromosome 4, which contains CAG

Question 3

HD hereditary?

Answer 3

Inherited as an autosomal dominant trait

**50/50 chance of passing the gene to child if dx with HD

Question 4

Common causes of death from HD

Answer 4

• PNA
• Heart failure
• Infection

Question 5

Early stage

Answer 5

• Symptoms warrant diagnosis; still independent
• Cognitive - Difficulty thinking through complex tasks
• Behavior - Depression, irritability, hyper sexuality, disinhibition
• Motor - Involuntary twitching in extremities, subtle loss of coordination

Question 6

PT causes no change in ** in HD.

Answer 6

Physical activity, number of falls

Question 7

Most commonly affected areas for essential tremor

Answer 7

#1 hands
#2 head

Question 8

Sequence of DA pathway disruption

Answer 8

Indirect (encephalon) – Direct (substance P)

Question 9

Meds for essential tremors

Answer 9

• Beta blockers
• Anti-seizure meds
• Tranquilizers
• Botox injections
• Anti- psychotics/depressants

Question 10

What does a PET and functional MRI show?

Answer 10

Show changes in the brain prior to sx onset

Question 11

CAG repeates

Answer 11

<28 = Normal
29-34 = Person will not develop HD but next generation is at risk
35-39 = Possible development of HD
>40 = Person will develop HD

Question 12

Meds for dystonia

Answer 12

• Dopaminergics
• Anticholinergics
• GABA agonists
• Anti-convulsants
• Botox

Question 13

Hallmark sign

Answer 13

Atrophy of the striatum that later involves the cerebral cortex and subcortical structures

Question 14

What is dystonia?

Answer 14

Involuntary muscle contractions that cause slow repetitive movements or abnormal postures

Question 15

Essential tremor

Answer 15

Characterized by involuntary and rhythmic shaking (action); relieved by rest and sleep
*Asymmetrical (similar to PD)

Question 16

Medications manage?

Answer 16

• Abnormal movements associated with HD
• Psyc disorders associated with HD
• *Other meds for PD, epilepsy, psychosis may be of benefit as well

Question 17

Tetrabenazine

Answer 17

Used to suppress chorea

Question 18

PT improves ** in HD.

Answer 18

UHDRS motor score, fitness, and gait speed

Question 19

Onset of age with essential tremor

Answer 19

40 years old

Question 20

Late stage

Answer 20

• Requires Max A, bedridden, may have feeding tube
• Cognitive - Significantly impaired, unable to recognize loved ones
• Behavior - Highly apathy, decreased depression but typically psychotic
• Motor - Decreased chorea; rigidity, dystonia, and bradykinesia more common

Question 21

PT causes mixed results in ** in HD.

Answer 21

Walking endurance, balance, an muscle strength

Question 22

Preclinical stage

Answer 22

• Prior to diagnosis
• Cognitive - Mild impairment in executive function, attention, memory, and emotional processing
• Behavior - Exhibit depression and irritability

Question 23

Frequency of suicide ideation

Answer 23

Common during at risk soft neuro signs, possible HD, and stage 2

Question 24

HD age of onset and death

Answer 24

Onset 40 years

Death 15-20 years after onset

Question 25

Dystonia prognosis

Answer 25

Stabilization of condition within 3-5 years after onset with subtle changes with environment, stress, etc.

Question 26

Middle stage

Answer 26

• May require Min-Mod A, difficulty swallowing, staggering gait
• Cognitive - Difficulty organizing and thinking clearly
• Behavior - Similar to early stages, more apathetic
• Motor - Notable chorea

Question 27

What does MRI and CT scans show?

Answer 27

Show decrease in striatal volume and increase in size of frontal horns of lateral ventricles