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Hemiparkinsonism-hemiatrophy syndrome

From the Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX.

Address correspondence and reprint requests to Dr. Joseph Jankovic, Professor of Neurology, Director, Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Suite 1801, 6550 Fannin, Houston, TX 77030 josephj{at}bcm.tmc.edu

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.

Abbreviations: DBS = deep brain stimulation; FDG = [¹⁸F]-fluorodeoxyglucose; FDP = [¹⁸F]-fluorodopa; FESP = [¹⁸F]-fluoroethylspiperone; HA = hemiatrophy; HP = hemiparkinsonism; HPHA = hemiparkinsonism-hemiatrophy syndrome; PD = Parkinson disease; STN = subthalamic nucleus; UPDRS = Unified PD Rating Scale; VIM = ventral intermediate.

Disclosure: The authors report no conflicts of interest.

Received January 15, 2007. Accepted in final form May 8, 2007.