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Hyperkinetic Movement disorders


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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?
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.
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.
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?
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.

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