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33 Cards in this Set
- Front
- Back
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What is dystonia?
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Disorder dominanted by sustained muscle contraction causing twisting and repetitive movemement or abnormal postures. Idiopathic is the most common typer.
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An important disease to rule out in a patient presenting with chorea or dystonia is?
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Wilsons dz
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Characterize Wilsons Dz?
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Autosomal recessive disorder of copper metabolism in which toxic effects of copper overload liver causing hepato-lenticular degenration.
Resulting in brain dysfunction, including involuntary movt. |
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Discuss diagnosis and treatment of Wilsons Dz.
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1. Blood panel showing decreased serum ceruloplasmin, increased 24hr urinary copper excretion and Kayser-fleischer rings.
Treatment is life saving...Pencillamine copper chelating agent. |
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What is the treament for focal dystonia?
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Focal dystonia occurs more often in adults. Bleopharospasm (abnormal closure of the eyelids) and other focal abnormalities are treated with intramuscular injections of botulinum toxin.
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Define chorea.
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Irregular, unpredictbale jerky movemnts which flit from one part of the body to another in continuous random fashion appereaing to be semipurposeful. Fidgety quality when mild, dancing quality when severe.
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What disease is a major cause of chorea?
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Huntingtons Dz.
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Characterize Huntingtons Dz.
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Triad of dementia, psychiatric problems and movement disorders of expanded trinucleotide repeats.
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Diffentiate athetosis from ballism.
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Ballism- high amplitude fast chorea can present at times with flinging movt of limbs usually secondary to lesion in subthalamic nucleus
athetosis- periperal slow chorea...similar to piano playing of the extremities |
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Chaaracterize tardive dyskinesia.
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Drug induced chorea occuring secondary to chronic therapy with dopamine antagonist medications. Dilantin/lithium
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Describe the mechanism of movement inhibition by botulinum toxin.
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Endocytosed at the presynaptic bouton , its light chain interferes with binding of ACH vesicles to the terminal membrane decreasing synaptic concentrations of ACH.
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What is a tic?
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Abrupt, transient, sterotypic movt (clonic) jerky movements repeated at irregular interval. Patience experience an urge to execute this behavior temporarily relieved by its performance.
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What disorder is classically associated with tics?
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Gilles de la Tourette Syndrome
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What is the diagnostic criteria for Tourettes?
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Presentation between 2-15 years of age with motor and vocal tics.
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What is coprolalia?
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Uncontrolled urge to say shocking and dirty words frequently associated with Tourettes Syndrome.
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What is dystonia?
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Disorder dominanted by sustained muscle contraction causing twisting and repetitive movemement or abnormal postures. Idiopathic is the most common typer.
|
|
|
An important disease to rule out in a patient presenting with chorea or dystonia is?
|
Wilsons dz
|
|
|
Characterize Wilsons Dz?
|
Autosomal recessive disorder of copper metabolism in which toxic effects of copper overload liver causing hepato-lenticular degenration.
Resulting in brain dysfunction, including involuntary movt. |
|
|
Discuss diagnosis and treatment of Wilsons Dz.
|
1. Blood panel showing decreased serum ceruloplasmin, increased 24hr urinary copper excretion and Kayser-fleischer rings.
Treatment is life saving...Pencillamine copper chelating agent. |
|
|
What is the treament for focal dystonia?
|
Focal dystonia occurs more often in adults. Bleopharospasm (abnormal closure of the eyelids) and other focal abnormalities are treated with intramuscular injections of botulinum toxin.
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Define chorea.
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Irregular, unpredictbale jerky movemnts which flit from one part of the body to another in continuous random fashion appereaing to be semipurposeful. Fidgety quality when mild, dancing quality when severe.
|
|
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What disease is a major cause of chorea?
|
Huntingtons Dz.
|
|
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Characterize Huntingtons Dz.
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Triad of dementia, psychiatric problems and movement disorders of expanded trinucleotide repeats.
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Diffentiate athetosis from ballism.
|
Ballism- high amplitude fast chorea can present at times with flinging movt of limbs usually secondary to lesion in subthalamic nucleus
athetosis- periperal slow chorea...similar to piano playing of the extremities |
|
|
Chaaracterize tardive dyskinesia.
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Drug induced chorea occuring secondary to chronic therapy with dopamine antagonist medications. Dilantin/lithium
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Describe the mechanism of movement inhibition by botulinum toxin.
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Endocytosed at the presynaptic bouton , its light chain interferes with binding of ACH vesicles to the terminal membrane decreasing synaptic concentrations of ACH.
|
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What is a tic?
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Abrupt, transient, sterotypic movt (clonic) jerky movements repeated at irregular interval. Patience experience an urge to execute this behavior temporarily relieved by its performance. *** TIcs are unvoluntary....not involuntary due to the fact that they can be supressed
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What disorder is classically associated with tics?
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Gilles de la Tourette Syndrome
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What is the diagnostic criteria for Tourettes?
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Presentation between 2-15 years of age with motor and vocal tics.
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What is coprolalia?
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Uncontrolled urge to say shocking and dirty words frequently associated with Tourettes Syndrome.
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Define myoclonus.
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Rapid shock like movements arrythmic and randomly distributed usually occuring in MSECS!! often occuring secondary to sudden stimuli such as sound, light or visual threat.
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What pathology is commonlcy causal for generalized myoclonus?
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Metabolic encephalopathy, hepatic, renal anoxic
Degenerative dz CJD Creutzfeldt-Jakob, AD Alzeihmers associated with secondary (the more common) myoclonus |
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How are tics treated?
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dopamine antagonists, dopamine depleters and benzodiazepines.
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