Dr. Farbman: Movement Disorders

Question 1

Movement disorders impair (blank) without affecting strength or cerebellar function.

Answer 1

voluntary movement

Question 2

What are movement disorders a result of?

Answer 2

dysfunction of the basal ganglia

Question 3

Are movements increased or decreased in movement disorders?

Answer 3

increased (hyperkinesia) or decreased (hypokinesia)

Question 4

What are types of hypokinesia?

Answer 4

parkinsonism

Question 5

What are types of hyperkinesia?

Answer 5

tremor
chorea
athetosis
dystonia
tics 
ballismus

Question 6

What are the motor features of parkinsonism?

Answer 6

tremor
rigidity
bradykinesia
postural instability

Question 7

What does a dysfunction of the substantia nigra affect?

Answer 7

production of dopamine

Question 8

Causese of parkinsonism?

Answer 8

antipsychotic drugs *influence dopamine
postencephalitis
toxic agents *manganese, MPTP
Parkinson disease **most common

Question 9

Idiopathic degeneration of neurons in substantia nigra causing loss of dopamine

Answer 9

Parkinson’s disease

Question 10

When is the mean onset of Parkinson’s? Are males more affected or females?

Answer 10

63; 3 to 2 male to female

Question 11

What is the prevalence of Parkinson’s?

Answer 11

160/100,000

*50,000 new cases annually

Question 12

Cardinal symptoms of Parkinson’s disease?

Answer 12

resting tremor *asymmetric
rigidity *increased resistance to passive movement
hypokinesia *loss of automatic movements (armswing)
postural instability *difficulty rising from a chair

Question 13

How do you diagnose Parkinson’s disease?

Answer 13

*clinical diagnosis
nuclear imagining
response to medication

Question 14

What is the gold standard medication for Parkinson’s disease?

Answer 14

Levodopa

• *treats the symptoms, not the underlying disease
• *goes to brain, gets converted into dopamine (dopamine can’t cross BBB)

Question 15

When is surgery an option in Parkinson’s?

Answer 15

only after maximal medical treatment

in dopa-responsive patients

Question 16

What is the operation of choice for Parkinson’s disease?

Answer 16

deep brain stimulation at substantia nigra or GPi

Question 17

What symptoms often precede Parkinson’s disease?

Answer 17

anosmia
constipation
REM sleep behavior disorder

Question 18

What are other problems associated w Parkinson’s disease?

Answer 18

depression
dementia
orthostatic hypotension

Question 19

Most common movement disorder
Usually in arms or neck
Different tremor than Parkinson’s

Answer 19

essential tremor

Question 20

How does essential tremor differ from Parkinsons?

Answer 20

faster
BILATERAL
action tremor
may respond to alcohol

Question 21

movements that are slow, sinuous and writhing in nature

Answer 21

athetosis

Question 22

movements are sustained so they appear to be abnormal postures

Answer 22

dystonia

Question 23

What is the dystonia treatment of choice?

Answer 23

botox for focal dystonia

DBS surgery for general dystonia

Question 24

Very common
Affect up to 20% of children
Can “outgrow” them
Rapid, purposeless movement
Repetitive
Often suppressible, at least for a time

Answer 24

tics

Question 25

What defines tourette's syndrome?

Answer 25

motor + vocal tics

Question 26

Violent movements Proximal muscles are involved May look like chorea Often resolves spontaneously

Answer 26

Hemiballismus

Question 27

When is hemiballismus most commonly seen?

Answer 27

secondary to stroke in contralateral subthalamic nucleus

Question 28

Irregular, unpredictable jerky movement | Can spread from one part of the body to another in random sequence

Answer 28

chorea

Question 29

Causes of chorea

Answer 29

Syndenham's *occurs in children after strep Drug induced Huntington's disease *most common

Question 30

What are three "marked peculiarities" of Huntington disease?

Answer 30

1. hereditary 2. tendency to insanity/suicide 3. grave disease only in adult life

Question 31

When is the mean onset of Huntington?

Answer 31

age 40

Question 32

What are the symptoms of Huntingtons?

Answer 32

``` chorea speech disturbances falls cognitive psychiatric caudate atrophy *young onset variant w rigidity and akinesia ```

Question 33

Huntington Disease is a fully penetrant (blank) disorder; the gene is on chromosome 4; the trinucleotide repeat is (blank); it demonstrates (blank)

Answer 33

autosomal dominant; CAG; anticipation

Question 34

How many repeats of CAG will classify a person as normal? How many repeats will classify them as fully penetrant?

Answer 34

40

Question 35

What percentage of Huntington pts are affected by depression?

Answer 35

50% * 30% meet criteria for major depressive episode * suicide more common than in other depressed patients

Question 36

What percentage of Huntington pts experience mania?

Answer 36

10%

Question 37

Why is Huntington difficult to treat?

Answer 37

progressive, neurogenerative

Question 38

Cognitive problems with Huntington?

Answer 38

apathy impulsivity executive dysfunction

Question 39

Triad of hepatic, neurological, and psychiatric Autosomal recessive gene, which, when present, causes abnormal metabolism of copper Build-up of free copper is toxic

Answer 39

Wilson disease

Question 40

What is the gold standard test for Wilson disease?

Answer 40

24 degree urinary screen of copper - will be elevated if WD | *liver biopsy could be diagnostic

Question 41

Symptoms of Wilson disease?

Answer 41

``` tremors parkinsonism chorea dystonia dysathria *hepatic symptoms always present jaundice varices spider veins psychiatric symptoms ```

Question 42

What type of genetic disease is Wilson's disease? What is the mutated gene?

Answer 42

autosomal recessive; ATP7B

Question 43

How do you diagnose Wilson's disease?

Answer 43

MRI - big bilateral thalamus, putamen | Midbrain shows face of the giant panda - high signal in tegmentum with normal red nucleus

Question 44

Treatment of Wilson Disease

Answer 44

removal/lowering of copper

Question 45

What do you need to diagnose a movement disorder?

Answer 45

good history family history good exam **treat based on phenomenology of movements