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NEUROLOGY 2004;63:2149-2152
© 2004 American Academy of Neurology
Brief Communications

Hereditary spastic paraplegia with frontal lobe dysfunction

A clinicopathologic study

D. Yanase, MD, K. Komai, MD, PhD, T. Hamaguchi, MD, S. Okino, MD, PhD, H. Yokoji, MD, PhD, T. Makifuchi, MD, PhD, H. Takano, MD, PhD and M. Yamada, MD, PhD

From the Department of Neurology and Neurobiology of Aging (Drs. Yanase, Komai, Hamaguchi, Okino, and Yamada), Kanazawa University Graduate School of Medical Science, Department of Neurology (Dr. Yokoji), Noto General Hospital, Ishikawa, Department of Clinical Research (Dr. Makifuchi), National Saigata Hospital, Niigata, and Department of Neurology (Dr. Takano), Brain Research Institute, Niigata University, Japan.

Address correspondence and reprint requests to Dr. D. Yanase, Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1, Takara-machi, Kanazawa 920-8640, Japan; e-mail: d.yanase{at}med.kanazawa-u.ac.jp

The authors report an unusual family with hereditary spastic paraplegia (HSP) with frontal lobe dysfunction having the onset in the sixth decade. All the patients showed hypoperfusion in the frontal lobes and thalami on SPECT. Neuropathologic findings revealed thin corpus callosum and degeneration in the thalamic dorsomedial nuclei as well as degeneration of the corticospinal tracts. This family was likely affected by a novel form of HSP characterized by frontal lobe dysfunction caused by thalamic degeneration.

Received November 25, 2003. Accepted in final form July 13, 2004.




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