Hemiparkinsonism-hemiatrophy syndrome.

Objective: To characterize the clinical and radiologic features of hemiparkinsonism-hemiatrophy syndrome (HPHA).

Methods: Medical records of patients with evidence of unilateral parkinsonism and ipsilateral body atrophy, evaluated at the Baylor College of Medicine Movement Disorders Clinic, were reviewed.

Results: The mean age at onset of the 30 patients who satisfied the criteria was 44.2 (15 to 63) years with a mean duration of symptoms for 9.7 (2 to 20) years. Half of all patients had dystonia at onset and dystonia was present in 21 (70%) patients during the course of the syndrome. Eleven (37%) also had scoliosis. Brain asymmetry on imaging studies was noted in 9 (30%) patients. Response to levodopa was rated as good in 18, moderate in 6, and poor in 6. Nine of 19 (47%) patients in whom birth history was available had difficult birth or had severe febrile illness in the first few months of life. Overall 10 (33%) patients had difficulty in walking during early childhood.

Conclusion: Although the clinical features of hemiparkinsonism-hemiatrophy syndrome are variable, suggesting a heterogeneous pathogenesis, perinatal and early childhood cerebral injury appears to play an important role in about half of the cases.