#8 Hyperkinetic Movement Disorders

Question 1

What is chorea?

Answer 1

Irregular, involuntary, non-repetitive, unpredictable movements
Can be rapid or flowing, distal or proximal
Can affect any body part

Question 2

What are the three choreaform disorders?

Answer 2

Ballism (proximal and flingy), athetosis (proximal sinuous), and chorea (distal and rapid)

Question 3

Having a stroke in which pathway causes chorea?

Answer 3

A stroke in the indirect pathway causes loss of movement inhibition, therefore hyperkinesia

Question 4

What are some acute causes of chorea?

Answer 4

Medications, vascular, immune-related, hormonal, infectious, metabolic disorders, toxins/substance abuse

Question 5

What are some chronic causes of chorea?

Answer 5

Genetic causes (Huntington disease for ex)
Dopaminergic blockers, infections, nutrition, endocrine

Question 6

What type of inheritance is Huntington disease gained by?

Answer 6

Autosomal dominant inheritance, and the risk for men and women is the same

Question 7

What is Huntington’s disease?

Answer 7

Autosomal dominant CAG repeat, and the age of onset and progression is dependent on the size of triplet repeat. Has a slow progression, and 50% of AOO dependent on other genes

Question 8

What determines the onset of the HD disease?

Answer 8

The number of repeats determines onset, the more repeats you have, the earlier the disease manifests

Question 9

What are some early stage motor symptoms of HD?

Answer 9

Restlessness, motor impersistence, slowing of eye movement

Question 10

What are some mild-moderate stage symptoms HD?

Answer 10

Chorea gradually becomes more apparent and disabling

Question 11

What are some symptoms of late stage HD?

Answer 11

Chorea lessens, significant rigidity and bradykinesia
Gait and balance problems

Question 12

What are the 5 clinical features of HD?

Answer 12

Motor, psychiatric, cognitive, weight loss, apathy

Question 13

What happens to the basal ganglia in HD?

Answer 13

If degenerates due to neuron loss, and you get large opening and spaces in the brain as a result

Question 14

What are some treatments of HD?

Answer 14

Interdisciplinary management, ongoing psychosocial support, genetic counseling, and research

Question 15

What is a new treatment that is currently being researched?

Answer 15

Antisense oligonucleotides that are introduces into the cell, and they bind to mRNA to stop translation of it and therefore no toxic protein is made

Question 16

What is dystonia? What is it classified by? What are some causes?

Answer 16

Abnormal, sustained muscle contraction and postures, may be associated with tremor, may be alleviated by sensory tricks
It is classified by age of onset and distribution
Causes: brain injury, toxic, genetic (kids), and some forms may be related to decreased dopamine

Question 17

What is primary generalized dystonia?

Answer 17

Childhood onset, progressive, begins in legs then trunk then arms, and most commonly caused by the DYT 1 mutation (autosomal dominant with variable penetrance and expressivity)

Question 18

What is focal dystonia? What are some treatments?

Answer 18

Adult onset, non-progressive
4 types:
Blepharospasm (eyes), Oromandibular (mouth and jaw), Occupational (writing and playing instruments), Cervical (neck)
Treatment: botulinum toxin injections (BOTOX), medications, surgery (DBS of GPi)

Question 19

What are tics? What are the two differential diagnoses of tics?

Answer 19

Brief, non-rhythmic semi-suppressible movements, there are simple motor and vocal, as well as complex vocal and motor
Primary: transient tics of childhood, chronic tics, tourette syndrome
Secondary: infections, other disorders, stroke, etc

Question 20

Are tics common? What do we think causes tics?

Answer 20

Tics are common, about 3% of young children have them
There are chronic motor tics
Chronic motor tics and vocal tics are tourettes syndrome
The pathophysiology is unknown, but thought to be due to disinhibition of the indirect circuit going into the motor cortex and limbic system

Question 21

What is Tourette’s syndrome?

Answer 21

Multiple motor and 1 or more vocal tics for longer than one year, is it often associated with obsessive compulsive symptoms, ADHD and behaviour problems

Question 22

How do you treat Tourettes?

Answer 22

Education and counselling, modification of home/school/work environment, treat the hyperactivity and ADHD symptoms with clonidine and CNS stimulants, and treat the OCD behaviour with SSRI, and other drugs