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Hemiparkinsonism-hemiatrophy syndrome
Subhashie Wijemanne1, Joseph Jankovic
1Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Suite 1801, 6550 Fannin, Houston, TX 77030, USA.
Neurology
|October 17, 2007
Summary
Hemiparkinsonism-hemiatrophy syndrome (HPHA) presents with variable symptoms, often including dystonia and scoliosis. Perinatal or early childhood brain injury may contribute to about half of these cases.
Area of Science:
- Neurology
- Movement Disorders
- Clinical Neuroscience
Background:
- Hemiparkinsonism-hemiatrophy syndrome (HPHA) is a rare neurological condition characterized by unilateral parkinsonian symptoms and ipsilateral body atrophy.
- Understanding its clinical and radiological spectrum is crucial for diagnosis and management.
Purpose of the Study:
- To comprehensively characterize the clinical and radiologic features of hemiparkinsonism-hemiatrophy syndrome.
- To explore potential contributing factors to the syndrome's development.
Main Methods:
- Retrospective review of medical records from patients diagnosed with HPHA at a specialized movement disorders clinic.
- Inclusion criteria required evidence of unilateral parkinsonism and ipsilateral body atrophy.
Main Results:
- The study analyzed 30 patients with a mean age of onset of 44.2 years.
- Dystonia was a common feature, present in 70% of patients, and 37% had scoliosis.
- Brain asymmetry was observed in 30% of patients, and 47% had a history of difficult birth or early febrile illness, suggesting potential early cerebral injury.
Conclusions:
- Hemiparkinsonism-hemiatrophy syndrome exhibits heterogeneous clinical features, indicating diverse underlying causes.
- Perinatal and early childhood cerebral injury appears to be a significant contributing factor in approximately half of the cases.
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