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Mizue Tobo, MD; Yoshio Mitsuyama, MD; Koichi Ikari, MD; Kokichi Itoi, MD

Arch Neurol. 1984;41(10):1091-1094.

Abstract
• The adult type of neuronal ceroid lipofuscinosis (NCL) occurred in a 49-year-old man and his 51-year-old sister. They showed episodic stuporous and psychotic states, mental retardation, generalized convulsions, and ichthyosis vulgaris. At autopsy the woman had excessive accumulation of lipofuscin throughout the CNS. The degree of neuronal lipopigment accumulation was very severe in the neurons of the thalamus, substantia nigra, inferior olivary nuclei, motor nuclei of the brain stem, and cerebral cortex. Mental symptoms, such as stupor, excitement, hallucinations, and delusions, were the predominant clinical manifestations and so were misdiagnosed as schizophrenia. Though the clinical diagnosis of the adult type of NCL (Kufs' disease) is difficult because of its wide variety of manifestations, symptoms such as episodic psychotic and stuporous states accompanied by convulsive disorders with mild neurologic signs may be an indication of this disease.

Author Affiliations
From the Department of Neuropsychiatry, Medical College of Oita (Japan) (Dr Tobo); Department of Psychiatry, Miyazaki (Japan) Medical College (Dr Mitsuyama); Department of Neuropsychiatry (Dr Ikari), Faculty of Medicine, Kyushu University, and Jyono Prison (Dr Itoi), Kitakyushu, Fukuoka, Japan.

Footnotes
Accepted for publication Sept 14, 1983.

Reprint requests to Department of Neuropsychiatry, Medical College of Oita, Hazama-machi, Oita 879-56, Japan (Dr Tobo).

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