Movement Disorders

Posted by e-Medical PPT
Wilson’s disease
AR trait caused by mutation in copper-transporting P-type ATPase, causing abnormal copper accumulation in the liver, BG and other organs

Liver disease (40%):  
Subclinical liver disease
95% under age 20                      
Acute hepatitis  
Fulminant hepatitis                                      
Cirrhosis              

              
Neurological Manifestations
Invariably have liver dysfunction
Isolated tremor (50%)
Dysarthria
Drooling
Dysphagia
Gait abnormality, parkinsonian syndrome, generalized dystonic syndrome

Idiopathic Torsion Dystonia (Oppenheim’s Dystonia)
8.2 years (6-9 years)
Normal development, walk normally
Symptoms start in legs in early onset and in arms and cranial structures in later onset
Becomes generalized within one year of onset
Intact mental function & no psychiatric illness
Positive Family History suggesting autosomal dominant inheritance
Course is progressive in early stages followed by Spontaneous stabilization
AD with reduced penetrance
DYT1 gene is responsible for early-onset idiopathic torsion dystonia in Ashkenazi Jewish families, and in one large non-Jewish family
Gene is mapped to chromosome 9q32-34
Glycosaminoglycan (GAG) deletion
Alteration of norepinephrine concentration in several areas of the brainstem

Drug-Induced Movement Disorders
Parkinsonism
Acute dystonia
Tardive dyskinesia
Neuroleptic Malignant Syndrome
Other drug-induced movement disorders

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