Two Cases of Progressive Supranuclear Palsy

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  • SAWAGUCHI Yoshiyasu
    The First Department of Internal Medicine, Kyorin University School of Medicine
  • KOIKE Hidemi
    The First Department of Internal Medicine, Kyorin University School of Medicine
  • WAKABAYASHI Yukio
    The First Department of Internal Medicine, Kyorin University School of Medicine
  • YOSHINO Yoshikazu
    The First Department of Internal Medicine, Kyorin University School of Medicine

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Other Title
  • 進行性核上性麻痺の 2 症例
  • シンコウセイ カク ジョウセイ マヒ ノ 2 ショウレイ

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Description

Two cases of progressive supranuclear palsy, both 76-year-old men, were reported. They showed typical symptoms such as supranuclear ophthalmoplegia especially for vertical gaze in Case 1 and for all directions in Case 2, pseudobulbar palsy (dysarthria, dysphagia), axial dystonic rigidity with extension of the neck and mild dementia. In addition, difficulty in lid opening was observed in Case 2, which was thought to be, so called "apraxia of lid opening". The association of this unique symptom with progressive supranuclear palsy was discussed. In Case 1, trihexyphenidyl was effective in horizontal ocular movements, although restricted vertical gaze was not improved. There have been 49 cases of this disease reported so far in Japan, since 1964 when Steele, Richardson and Olszewski in Canada established it as a disease entity by presenting 9 cases including extensive pathological investigation in 7 cases. Forty two cases in Japan, who were comparatively well described, consisted of 33 men and 9 women. Symptoms began mostly in later than sixth decade (range 36-76 years, mean 62 years). "Apraxia of lid opening" was concomitantly observed in 7 cases, namely about 17%.

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