Juvenile levodopa-responsive Parkinsonism with early orobuccolingual dyskinesias and cognitive impairment.
A 13-year old girl presented with slowly progressive rest tremor of the hands, bradykinesia, and rigidity. The symptoms improved with dopaminergic medications, but severe drug-induced dyskinesias developed early. She subsequently developed cognitive slowing and difficulty initiating saccadic eye movements. She went on to have deep brain stimulation surgery. Experts discuss the syndromal diagnosis and predict the underlying pathology. The pathological diagnosis is given and clinical learning points are considered.
Espay
Alberto J
AJ
Department of Neurology, UC Neuroscience Institute, University of Cincinnati, Cincinnati, Ohio, USA. espayaj@ucmail.uc.edu
Paviour
Dominic C
DC
O'Sullivan
John D
JD
Schmidt
Robert E
RE
Revilla
Fredy J
FJ
Metman
Leo Verhagen
LV
D000293:Adolescent; D001921:Brain / Q000601:surgery; D003072:Cognition Disorders / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D046690:Deep Brain Stimulation; D004409:Dyskinesia, Drug-Induced / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D006801:Humans; D045586:Intranuclear Inclusion Bodies; D007980:Levodopa / Q000009:adverse effects* / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D019636:Neurodegenerative Diseases / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D020734:Parkinsonian Disorders / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D055815:Young Adult
D007980:Levodopa