Dr. Farbman: Movement Disorders Flashcards
Movement disorders impair (blank) without affecting strength or cerebellar function.
voluntary movement
What are movement disorders a result of?
dysfunction of the basal ganglia
Are movements increased or decreased in movement disorders?
increased (hyperkinesia) or decreased (hypokinesia)
What are types of hypokinesia?
parkinsonism
What are types of hyperkinesia?
tremor chorea athetosis dystonia tics ballismus
What are the motor features of parkinsonism?
tremor
rigidity
bradykinesia
postural instability
What does a dysfunction of the substantia nigra affect?
production of dopamine
Causese of parkinsonism?
antipsychotic drugs *influence dopamine
postencephalitis
toxic agents *manganese, MPTP
Parkinson disease **most common
Idiopathic degeneration of neurons in substantia nigra causing loss of dopamine
Parkinson’s disease
When is the mean onset of Parkinson’s? Are males more affected or females?
63; 3 to 2 male to female
What is the prevalence of Parkinson’s?
160/100,000
*50,000 new cases annually
Cardinal symptoms of Parkinson’s disease?
resting tremor *asymmetric
rigidity *increased resistance to passive movement
hypokinesia *loss of automatic movements (armswing)
postural instability *difficulty rising from a chair
How do you diagnose Parkinson’s disease?
*clinical diagnosis
nuclear imagining
response to medication
What is the gold standard medication for Parkinson’s disease?
Levodopa
- *treats the symptoms, not the underlying disease
- *goes to brain, gets converted into dopamine (dopamine can’t cross BBB)
When is surgery an option in Parkinson’s?
only after maximal medical treatment
in dopa-responsive patients
What is the operation of choice for Parkinson’s disease?
deep brain stimulation at substantia nigra or GPi
What symptoms often precede Parkinson’s disease?
anosmia
constipation
REM sleep behavior disorder
What are other problems associated w Parkinson’s disease?
depression
dementia
orthostatic hypotension
Most common movement disorder
Usually in arms or neck
Different tremor than Parkinson’s
essential tremor
How does essential tremor differ from Parkinsons?
faster
BILATERAL
action tremor
may respond to alcohol
movements that are slow, sinuous and writhing in nature
athetosis
movements are sustained so they appear to be abnormal postures
dystonia
What is the dystonia treatment of choice?
botox for focal dystonia
DBS surgery for general dystonia
Very common Affect up to 20% of children Can “outgrow” them Rapid, purposeless movement Repetitive Often suppressible, at least for a time
tics
What defines tourette’s syndrome?
motor + vocal tics
Violent movements
Proximal muscles are involved
May look like chorea
Often resolves spontaneously
Hemiballismus
When is hemiballismus most commonly seen?
secondary to stroke in contralateral subthalamic nucleus
Irregular, unpredictable jerky movement
Can spread from one part of the body to another in random sequence
chorea
Causes of chorea
Syndenham’s *occurs in children after strep
Drug induced
Huntington’s disease *most common
What are three “marked peculiarities” of Huntington disease?
- hereditary
- tendency to insanity/suicide
- grave disease only in adult life
When is the mean onset of Huntington?
age 40
What are the symptoms of Huntingtons?
chorea speech disturbances falls cognitive psychiatric caudate atrophy *young onset variant w rigidity and akinesia
Huntington Disease is a fully penetrant (blank) disorder; the gene is on chromosome 4; the trinucleotide repeat is (blank); it demonstrates (blank)
autosomal dominant; CAG; anticipation
How many repeats of CAG will classify a person as normal? How many repeats will classify them as fully penetrant?
40
What percentage of Huntington pts are affected by depression?
50%
- 30% meet criteria for major depressive episode
- suicide more common than in other depressed patients
What percentage of Huntington pts experience mania?
10%
Why is Huntington difficult to treat?
progressive, neurogenerative
Cognitive problems with Huntington?
apathy
impulsivity
executive dysfunction
Triad of hepatic, neurological, and psychiatric
Autosomal recessive gene, which, when present, causes abnormal metabolism of copper
Build-up of free copper is toxic
Wilson disease
What is the gold standard test for Wilson disease?
24 degree urinary screen of copper - will be elevated if WD
*liver biopsy could be diagnostic
Symptoms of Wilson disease?
tremors parkinsonism chorea dystonia dysathria *hepatic symptoms always present jaundice varices spider veins psychiatric symptoms
What type of genetic disease is Wilson’s disease? What is the mutated gene?
autosomal recessive; ATP7B
How do you diagnose Wilson’s disease?
MRI - big bilateral thalamus, putamen
Midbrain shows face of the giant panda - high signal in tegmentum with normal red nucleus
Treatment of Wilson Disease
removal/lowering of copper
What do you need to diagnose a movement disorder?
good history
family history
good exam
**treat based on phenomenology of movements
