Long-term disability and prognosis in dentatorubral-pallidoluysian atrophy: a correlation with CAG repeat length.

@article{Hasegawa2010LongtermDA,
  title={Long-term disability and prognosis in dentatorubral-pallidoluysian atrophy: a correlation with CAG repeat length.},
  author={Arika Hasegawa and Takeshi Ikeuchi and Ryoko Koike and Nae Matsubara and Miyuki Tsuchiya and H Nozaki and Atsushi Homma and Jiro Idezuka and Masatoyo Nishizawa and Osamu Onodera},
  journal={Movement disorders : official journal of the Movement Disorder Society},
  year={2010},
  volume={25 11},
  pages={1694-700}
}
Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal dominant neurodegenerative disorder caused by CAG repeat expansion. Previous studies demonstrated that the onset of DRPLA is closely associated with CAG repeat length. However, the natural history of DRPLA has not yet been evaluated. We here retrospectively investigated the factors that determine the disease milestones and prognosis in 183 Japanese patients genetically diagnosed with DRPLA. We determined the age at onset, age at… CONTINUE READING