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- CLINICA NEUROL, 41: 769－774, 2001
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Original Article
Neurological and neuropathological studies of amyotrophic lateral sclerosis/parkinsonism-dementia complex in the Kii peninsula of Japan
Yasumasa Kokubo, M. D. and Shigeki Kuzuhara, M. D.
Department of Neurology, Mie University School of Medicine
Hohara and Kozagawa in the Kii peninsula of Japan are reported to be high-incidence foci of amyotrophic lateral sclerosis (Kii ALS) and parkinsonism-dementia complex (Kii PDC). During the period between 1996 and 1999, three Kii ALS patients and 19 Kii PDC patients were confirmed neurologically in Hohara among which, one Kii ALS patient and two Kii PDC patients were examined neuropathologically. The ratio of positive family history where ALS or PDC occurred within the fourth degree of the relatives was 33.3% in the patients with Kii ALS, 78.9% in those with Kii PDC, and 72.7% in total. The ages of onset were between 57 years and 63 years (mean age: 60.0 years) in the patients with Kii ALS and between 53 years and 74 years (mean age: 66.5 years) in those with Kii PDC. All of the Kii ALS patients were female, and the male to female ratio of the Kii PDC patients was 1: 1.7. The clinical features of Kii ALS were basically similar to those of classical ALS. The core clinical features of Kii PDC consisted of dementia and parkinsonism, frequently accompanied by motor neuron symptoms. The cardinal neuropathological features of Kii ALS/PDC included many neurofibrillary tangles (NFTs) associated with loss of nerve cells in the cerebral cortex and the brain stem, as well as morphological alterations diagnostic of ALS. Ultrastructurally, NFTs consisted of paired helical filaments. When we compared the clinical features of these Kii ALS patients with those that had been surveyed in 1969, the male to female ratio changed from male dominance to female dominance and the mean age of the onset of the disease was delayed by approximately 10 years. The most frequent initial symptom had been weakness of the lower limbs in the survey in 1969 and was bulbar palsy in this study. As to Kii PDC, this is the first report of the clinical features of many cases.
(CLINICA NEUROL, 41: 769－774, 2001)
key words: amyotrophic lateral sclerosis, parkinsonism-dementia complex, Kii peninsula, Guam island, neurofibrillary tangle
(Received: 19-Mar-01)
