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NEUROLOGY 1994;44:1622
© 1994 American Academy of Neurology

Metabolic topography of the hemiparkinsonism-hemiatrophy syndrome

S. Przedborski, MD, PhD, N. Giladi, MD, S. Takikawa, MD, T. Ishikawa, MD, V. Dhawan, PhD, P. Spetsieris, PhD, T. Chaly, PhD, S. Fahn, MD and D. Eidelberg, MD

Movement Disorder Group, Department of Neurology (Drs. Przedborski, Giladi, and Fahn), Columbia University, New York, NY; and the Departments of Neurology (Drs. Takikawa, Ishikawa, Dhawan, Spetsieris, and Eidelberg) and Research (Dr. Chaly), North Shore University Hospital/Cornell University Medical College, Manhasset, NY.

We estimated regional and global metabolic rates for glucose using 18F-fluorodeoxyglucose (FDG) and PET in six patients with hemiparkinsonism-hemiatrophy syndrome (HPHA; mean age, 41.0 ± 12.4 years). We used 18F-fluorodopa (FDOPA) and PET in two patients to quantify presynaptic nigrostriatal dopaminergic function. We compared measures of brain glucose metabolism and striatal FDOPA uptake with those calculated for 10 age-matched normal volunteers (mean age, 35.1 ± 8.0 years) and 10 patients with typical unilateral Parkinson's disease (unilat-PD; mean age, 58.2 ± 13.8 years). All six HPHA patients demonstrated significant metabolic reductions (>3 SD) in the contralateral basal ganglia or frontal cortex as compared with normal control values. Mean normalized glucose metabolism was reduced in the contralateral caudate and lentiform nuclei (p < 0.005) as compared with that in unilat-PD and normal controls. In both patients studied with FDOPA, contralateral striatal uptake was significantly reduced (>3 SD) as compared with normal control values. These results suggest that the clinical manifestations of HPHA arise through a combination of pre- and postsynaptic nigrostriatal dopaminergic dysfunction. FDG and PET may be useful in differentiating this disorder from typical unilat-PD.

Address correspondence and reprint requests to Dr. David Eidelberg, Department of Neurology, North Shore University Hospital/Cornell University Medical College, 300 Community Drive, Manhasset, NY 11030.

Supported by grants from the Parkinson Disease Foundation and the Dystonia Medical Research Foundation. D.E. is a faculty fellow of the Parkinson Disease Foundation and the United Parkinson Foundation. S.T. and T.I. are Veola T. Kerr fellows of the Parkinson Disease Foundation.

Received January 5, 1994. Accepted in final form March 1, 1994.




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