Shigehiro Nagai, Yoshiaki Saito, Yukari Endo, Takashi Saito, Kenji Sugai, Akihiko Ishiyama, Hirofumi Komaki, Eiji Nakagawa, Masayuki Sasaki, Kimiteru Ito, Yuko Saito, Sayuri Sukigara, Masayuki Ito, Yu-Ichi Goto, Shuichi Ito, Kentaro Matsuoka
We delineate a complication of hypoalbuminemia in dentatorubral-pallidoluysian atrophy (DRPLA), which we have found to be common in this disorder. In addition, we explored the pathogenesis of this phenomenon through clinical and histological examinations. Clinical course and laboratory findings of nine patients with childhood-onset DRPLA (aged 6-49 years; CAG repeat length 62-93) were retrospectively reviewed. Autopsied specimens from three patients were examined by histopathological and immunohistochemical analyses...
May 2013: Journal of Neurology