Hyperkinetic Movement disorders
Terms
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- What is dystonia?
- 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?
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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. - 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. - 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.
- Define chorea.
- 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.
- What disease is a major cause of chorea?
- Huntingtons Dz.
- Characterize Huntingtons Dz.
- Triad of dementia, psychiatric problems and movement disorders of expanded trinucleotide repeats.
- 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 - Chaaracterize tardive dyskinesia.
- Drug induced chorea occuring secondary to chronic therapy with dopamine antagonist medications. Dilantin/lithium
- Describe the mechanism of movement inhibition by botulinum toxin.
- Endocytosed at the presynaptic bouton , its light chain interferes with binding of ACH vesicles to the terminal membrane decreasing synaptic concentrations of ACH.
- What is a tic?
- 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
- What disorder is classically associated with tics?
- Gilles de la Tourette Syndrome
- What is the diagnostic criteria for Tourettes?
- Presentation between 2-15 years of age with motor and vocal tics.
- What is coprolalia?
- Uncontrolled urge to say shocking and dirty words frequently associated with Tourettes Syndrome.
- Define myoclonus.
- 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.
- 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 - How are tics treated?
- dopamine antagonists, dopamine depleters and benzodiazepines.