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Ullrich Wüllner, MD; Michael Reimold, MD; Michael Abele, MD; Katrin Bürk, MD; Martina Minnerop, MD; Bernd-Michael Dohmen, MD; Hans-Juergen Machulla, PhD; Roland Bares, MD; Thomas Klockgether, MD

Arch Neurol. 2005;62:1280-1285.

Background  The spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of autosomal dominant ataxias: some mutations, including SCA1, SCA2, and SCA3, are multisystemic disorders characterized by a variety of noncerebellar symptoms while others, like SCA6, give rise to a pure cerebellar syndrome.

Objective  To identify impairments of the dopaminergic system and regional changes of glucose metabolism in SCA1, SCA2, SCA3, and SCA6.

Methods  We used [¹¹C]d-threo-methylphenidate and [¹⁸F]fluorodeoxyglucose positron emission tomography to identify cerebral dopamine terminal loss and specific regional metabolic patterns in SCA1, SCA2, SCA3, and SCA6.

Results  The binding potential of [¹¹C]d-threo-methylphenidate was reduced in the striatum in SCA2 and SCA3; in contrast to patients with Parkinson disease, no increased susceptibility of the putamen was evident. Decreased regional cerebral glucose metabolism was found in the cerebellum of all patients with SCA, the brainstem of SCA1, SCA2, SCA3, the thalamus and putamen of SCA3, and the parietal cortex of patients with SCA2. A trend toward increased regional cerebral glucose metabolism was found in the temporal cortex of all patients with SCA, pronounced in SCA6.

Conclusions  Specific biochemical patterns point to different mechanisms of neuronal dysfunction in SCA1, SCA2, SCA3, and SCA6; dopamine terminal loss is severe in SCA2 but distinct from Parkinson disease.

Author Affiliations: Department of Neurology, University of Bonn, Bonn, Germany (Drs Wüllner, Abele, and Klockgether); Department of Nuclear Medicine, Ottfried-Müller-Straße, Tübingen, Germany (Drs Reimold, Dohmen, and Bares); Department of Neurology, University of Tübingen (Dr Bürk); Brain Imaging Centre West, Research Centre Jülich, Jülich, Germany (Dr Minnerop); and Radiopharmacy, University of Tübingen (Dr Machulla).

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