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31 Cards in this Set
- Front
- Back
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What is the most common movement disorder in children?
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Tics
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What are the most common movement disorders in adults?
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Parkinson's disease
Parkinson-like disorders Tremors Gait abnormalities |
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Akinesia
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reduced spontaneous movements
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Bradykinesia
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slowed movements
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Chorea
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means "dance" in Greek, and looks that way
random, purposeless, jerky movements |
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Clonus
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regular large amplitude jerk or series of jerks, slower than myoclonus, associated with spasticity or seizures
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Describe the cause of Huntington's disease
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Autosomal dominant disease
100% penetrance caused by excess CAG repeat (polyglutamine excess) that exhibits toxic gain of function |
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Describe the clinical manifestation of Huntington's disease
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Generally begins in middle age (some exceptions)
Chorea Dementia Behavioral disturbances |
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What is the significance of the triple nucleotide repeats in Huntington's?
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The number of repeats determines how early the disease prevents
The more CAG rpts, the earlier manifestation |
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How passes on a greater number of repeats to their children: mothers or fathers?
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Fathers
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Can Huntington's arise de novo?
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Spontaneous mutations are very rare; most all cases are hereditary
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*HD has the highest rate of suicide of any disorder, psychiatric or neurologic*
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- that was just a fact to remember. couldn't figure out how to make a question out of it -
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What treatments are available for Huntington's?
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Symptomatic treatment (chorea, psychosis, depression)
No treatment for disease progression |
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What behavioral disturbances are present in pts with Huntington's disease?
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Impulsivity
Disinhibition Depression Psychosis (sometimes) Mania (rare) Sociopathy |
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Describe the Huntington's disease gait
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Wide base
Poor balance Choreic movements of upper body |
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How many repeats are necessary to cause Huntington's disease?
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40+ repeats
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Why may pts with Huntington's have the highest degree of suicide vs all other disorders?
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Behavioral changes of the disease - impulsive, depression
Knowledge of the future outcome of the disease :( |
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What are the clinical manifestations of Parkinson's Disease?
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Resting, pill-rolling tremor (80% pts)
Akinesia (masked face) Bradykinesia Rigidity (cogwheel) Postural change Greater imbalance Gait Micrographia (smaller handwriting) Dysarthria |
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What does "Parkinsonism" refer to?
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The symptoms of akinesia and rigidity
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Describe the Parkinson's gait.
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One or both arms remain at the hip, no arm swing
Shortened stride length Shuffle when turn (no pivot turn) |
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Describe the onset of Parkinson's disease
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Generally asymmetric; first affected side generally remains worse throughout disease course
Median age of onset = 60 |
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What causes Parkinson's disease?
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Loss of dopaminergic cells in the substantia nigra pars compacta
SNc projects mainly to putamen - loss of dopamine NT seen in putamen |
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What is required for the diagnosis of Parkinson's disease and is considered the pathological hallmark of the disease?
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Lewy bodies
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What drugs produce Parkinson's-like symptoms?
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alpha-methyltyrosine
Reserpine Haloperidol (anti-psychotic) |
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What is the nature of Parkinson's treatments?
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Symptomatic; don't improve nor treat the disease itself
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What drugs are used to treat Parkinson's symptoms?
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L-DOPA + carbidopa
Amantidine (glutamate antagonist) Apomorphine (D2 agonist) Bromocriptine (D2 agonist) Pergolide (D2 agonist) Deprenyl, Selegiline (MAO-B inhibitor) COMT Inhibitors Anticholinergics (tremor, drooling, bladder hyperactivity) |
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Is Huntington's disease a dopamine excess state?
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No, despite the ability to pharmacologically elicit Huntington's chorea by excess dopamine
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What is the risk in taking Parkinson's drugs long-term or in excess?
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May develop choreiform movements
e.g. Michael J Fox |
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Is there a clinical test to diagnose Parkinson's disease?
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No
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What increases the risk of developing Parkinson's disease?
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First degree family history
Not smoking cigaretts Not drinking enough coffee |
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With regard to dopaminergic cells in the substantia nigra, when do the clinical signs of Parkinson's begin to present?
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When 50-80% of these dopaminergic cells are lost :(
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