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NEUROLOGY 1992;42:1009
© 1992 American Academy of Neurology

Progressive supranuclear palsy-like syndrome in two siblings of a consanguineous marriage

S. Ohara, MD, K. Kondo, MD, H. Morita, MD, K. Maruyama, MD, S. Ikeda, MD and N. Yanagisawa, MD

From the Department of Medicine (Neurology), Shinshu University School of Medicine. Matsumoto, Japan; and the Department of Neurology. Saku Central Hospital, Usuda, Japan.

Two siblings of a consanguineous marriage showed identical clinical features consisting of supranuclear vertical ophthalmoplegia, bradykinesia, rigidity. stooped posture, pseudobulbar palsy, and dementia, all beginning in the sixth decade. There was no history of encephalitis or of exposure to known chemicals. L-Dopa therapy was only partially and temporarily effective. Autopsy of one patient revealed extensive neurofibrillary degeneration with prominent involvement of the limbic system. There were no senile plaques or Lewy bodies. Under electron microscopy, there were paired helical filaments as well as 15-nm-wide straight tubules. The diffuse appearance of neurofibrillary tangles, predominant in the limbic system, and familial occurrence are unusual in progressive supranuclear palsy; these cases may, therefore, constitute a different, distinctive clinicopathologic disease.

Address correspondence and reprint requests to Dr Shuichi Ikeda, Department of Medicine (Neurology), Shinshu University School of Medicine, Matsumoto 390, Japan

Received May 9, 1990 Accepted for publication in final form October 8, 1991






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