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Stages of sleep pathology in spinocerebellar ataxia type 2 (SCA2)

From the Departments of Neurology (I.T., U.V., J.-S.K., K.K., H.S., G.A.), Psychiatry (I.T., B.P.) and Institute of Clinical Neuroanatomy (U.R.), J.W. Goethe-University, Frankfurt/Main, Germany; Institute for Human Genetics (D.N.), University of Giessen, Giessen, Germany; Department of Neurology (H.L.), Friedrich-Baur-Institute, LMU Munich, Munich, Germany; Institute for Human Genetics (S.T.), University of Dresden, Dresden, Germany; Department of Neurology (D.C.), University Hospital Darmstadt, Darmstadt, Germany.

Address correspondence and reprint requests to Prof. Georg Auburger, Section Molecular Neurogenetics, Building 26, Universitätsklinik, Theodor Stern Kai 7, 60590 Frankfurt/Main, Germany; e-mail: auburger{at}em.uni-frankfurt.de

Background: Autosomal dominant spinocerebellar ataxia type 2 (SCA2) bears clinical and neuropathologic similarities to sporadic multisystem atrophy (MSA) or Parkinson disease, in which sleep pathology is well documented. However, those clinical entities have a marked variability of the reported sleep disturbances, and their etiology is heterogeneous. In contrast, the study of SCA2 provides an opportunity to examine a molecularly homogeneous patient group, in which disease stages can be defined not only based on disease duration and ataxia scores, but also with regard to modulatory effects of mutation size.

Objective: To examine the presence and progression of sleep pathology in SCA2.

Methods: We analyzed eight patients with disease durations of 3 to 31 years, all with medium size SCA2 expansions (CAG 38 to 49), using clinical scores, sleep interviews, and video-polysomnography (VPSG) recordings.

Results: Almost all patients reported good subjective sleep quality and negated incidents of REM behavior disorder (RBD). At early disease stages, however, REM without atonia in four patients' VPSG suggested subclinical RBD. This was accompanied by a consistent reduction of REM density. In three patients at later SCA2 stages, REM sleep was undetectable, whereas slow wave sleep (SWS) was markedly increased at the cost of light sleep. Periodic leg movements, apnea, or hypopnea were not prominent.

Conclusions: Progressive loss of dream recall in spinocerebellar ataxia type 2 was found and correlated with stages of REM more than non-REM pathology in video-polysomnography. These stages correspond to the progressive atrophy from the pons, nigrostriatal projection, and locus ceruleus to the thalamus.

*These authors contributed equally and are listed in alphabetical order.

Supported by the European Community (EUROSCA, LSHM-CT-2004-503304).

Disclosure: The authors report no conflicts of interest.

Received March 28, 2006. Accepted in final form August 23, 2006.