Spinocerebellar ataxia type 6 (SCA6): neurodegeneration goes beyond the known brain predilection sites.

@article{Gierga2009SpinocerebellarAT,
  title={Spinocerebellar ataxia type 6 (SCA6): neurodegeneration goes beyond the known brain predilection sites.},
  author={Kristin Gierga and Helenius Jurgen Schelhaas and Ewout R. P. Brunt and Kay Seidel and W. Scherzed and Rupert Egensperger and Rob de Vos and Wilfred F. A. den Dunnen and Pieternella F Ippel and Elisabeth Petrasch-Parwez and Thomas Deller and Ludger Sch{\"o}ls and Udo R{\"u}b},
  journal={Neuropathology and applied neurobiology},
  year={2009},
  volume={35 5},
  pages={515-27}
}
AIMS Spinocerebellar ataxia type 6 (SCA6) is a late onset autosomal dominantly inherited ataxic disorder, which belongs to the group of CAG repeat, or polyglutamine, diseases. Although, it has long been regarded as a 'pure' cerebellar disease, recent clinical studies have demonstrated disease signs challenging the view that neurodegeneration in SCA6 is confined to the well-known lesions in the cerebellum and inferior olive. METHODS We performed a systematic pathoanatomical study throughout… CONTINUE READING
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