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Nigrostriatal dopaminergic function in familial amyotrophic lateral sclerosis patients with and without copper/zinc superoxide dismutase mutations

From the Department of Neurology (Dr. Przedborski and D.M. Donaldson and P.L. Murphy), Columbia University, New York, NY; the Departments of Neurology (Drs. Dhawan and Eidelberg) and Research (Dr. Mandel), North Shore University Hospital and Cornell University Medical College, Manhasset, NY; and the Cecil B. Day Laboratory for Neuromuscular Research (D. McKenna-Yasek and Dr. Brown), Massachusetts General Hospital, Harvard Medical School, Boston, MA.
Supported by grants from the Muscular Dystrophy Association (Drs. Przedborski and Brown, Jr.); the National Institute of Neurological Disorders and Stroke grant no. 1-K08-NS01724-02 (Dr. Przedborski); the Lowenstein Foundation (Dr. Przedborski); the Parkinson's Disease Foundation (Drs. Przedborski and Eidelberg); the Amyotrophic Lateral Sclerosis Association (Dr. Brown, Jr.); the Pierre L. de Bourgknecht ALS Research Foundation (Dr. Brown, Jr.); the Myrtle May MacLellan ALS Research Foundation (Dr. Brown, Jr.); the C.B. Day Investment Company (Dr. Brown, Jr.); the National Institutes of Health grant nos. 1PO1NS31248-01 and AG12922-01 (Dr. Brown, Jr.); the National Parkinson Foundation (Dr. Eidelberg); and Public Health Service grant R01 NS32368 (Dr. Eidelberg).
Received January 15, 1996. Accepted in final form April 12, 1996.
Address correspondence and reprint requests to Dr. S. Przedborski, BB-307, Department of Neurology, College of Physicians and Surgeons, Columbia University, 650 West 168th Street, New York, NY 10032.

Some cases of familial amyotrophic lateral sclerosis (FALS) are associated with copper/zinc superoxide dismutase (Cu/Zn-SOD) mutations, which are implicated in the death of motor neurons. Because Cu/Zn-SOD is present in high amounts in nigrostriatal dopaminergic neurons, we considered the possibility that FALS may be associated with subclinical nigrostriatal dopaminergic dysfunction. We used [(¹⁸) F]fluorodopa (FDOPA) and PET to study 14 FALS patients (50 +/- 11 years [mean +/- SD]): seven with (FALS-1) and seven without (FALS-0) Cu/Zn-SOD mutations. Fourteen age-matched normal volunteers (48 +/- 18 years) served as controls. Striato-occipital ratios (SORs) for the caudate and the putamen were calculated. Five of the 14 FALS patients had reduced striatal FDOPA uptake in the caudate nucleus, putamen, or both. Mean caudate SOR did not differ among FALS-1, FALS-0, and control subjects. Mean putamen SOR was significantly abnormal in FALS-0 but not in FALS-1 patients. These findings indicate that subclinical nigrostriatal dopaminergic dysfunction is present in some FALS patients and that FDOPA/PET abnormalities are more likely to be associated with FALS-0 status. This suggests that SOD mutations are less cytotoxic to dopaminergic than to motor neurons.

NEUROLOGY 1996;47: 1546-1551

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