Hemiparkinsonism-somatic hemiatrophy syndrome.
OBJECTIVE
This paper highlights the hemiparkinsonism-hemiatrophy (HPHA) syndrome as a unique presentation of the parkinsonian state. Clinically relevant diagnostic and treatment aspects are reviewed.


METHODS
We report a case of HPHA, in a 21-year-old, otherwise healthy, woman. Clinical and radiographic features of our case are presented. We also review the current literature on the clinical, radiological and pathophysiological mechanisms of HPHA.


RESULTS
In our case, despite the lack of benefit from anticholinergics and dopamine agonists (the patient declined treatment with levodopa), the patient showed a dramatic improvement with subthalamic nucleus stimulator (STN) implantation. There are no reported cases of use of STN stimulator in HPHA.


CONCLUSIONS
Hemiparkinsonism-hemiatrophy is a distinct entity that may be clinically and pathogenetically different from idiopathic Parkinson's disease; hence, HPHA needs to be considered as a possible syndrome in patients that have persistent unilateral parkinsonism. As medications are reported to be unhelpful in HPHA cases, early surgical intervention may be an option, such as in our case.
Jenkins
Mary
M
Department of Clinical Neurological Sciences, London Health Sciences Centre, ON, Canada.
Mendonca
Dan
D
Parrent
Andrew
A
Jog
Mandar S
MS
D000328:Adult; D004599:Electric Stimulation Therapy; D005150:Facial Hemiatrophy / Q000473:pathology; D005260:Female; D006130:Growth Disorders; D006801:Humans; D009139:Musculoskeletal Abnormalities / Q000473:pathology / Q000503:physiopathology; D010300:Parkinson Disease / Q000503:physiopathology* / Q000628:therapy*; D020531:Subthalamic Nucleus / Q000473:pathology / Q000503:physiopathology; D013577:Syndrome; D014202:Tremor / Q000209:etiology / Q000503:physiopathology / Q000628:therapy