Only a few Japanese migraineurs receive benefits of medical services and recent advances of headache medicine. Public education concerning headaches is one of the most urgent issues in Japan.
Two patients with "pure akinesia" who showed the characteristic changes of progressive supranuclear palsy (PSP) at necropsy are described. They had akinesia but no rigidity or tremor, and ophthalmoplegia was not observed during the course of illness. The symptoms of "pure akinesia" was not improved by levodopa therapy but was considerably improved by L-threo-3,4-dihydroxyphenylserine. At necropsy, pathological findings were not different from those reported for PSP. It is suggested that "pure akinesia" is an atypical manifestation of PSP, and that norepinephrinergic neurons may be involved in some types of PSP.
Background: The increasing number of patients with dementia in Japan, together with the rapid aging of society, is currently considered to have a substantial impact on Japan's medical, economic and sociological systems. Therefore, the longitudinal estimation of changes in the prevalence of dementia based on accurate diagnostic evaluation has important implications. Methods: We undertook three separate epidemiological studies on longterm changes, 10 years apart (1980, 1990 and 2000), in the prevalence of dementia in an elderly population using identical methods (DSM-III and Hachinski's ischemic score) for the same rural area in Japan (Daisen-cho). Results: The percentage of the population that was elderly (over 65 years of age) increased steadily from 16.0% in 1980 to 21.7% in 1990 and 27.1% in 2000. The prevalence of dementia (cases/100 people aged 65 years or older, adjusted to the population structure of 1980) in 1980, 1990 and 2000 was 4.4, 4.5 and 5.9, respectively, for all types of dementia, 1.9, 2.5 and 3.6, respectively, for Alzheimer-type dementia (DAT) and 2.0, 1.7 and 2.2, respectively, for vascular dementia (VaD). Conclusions: These findings of an increase in the number of cases and prevalence of DAT and VaD in a Japanese rural community have important implications for interventional medicine.
A 30-year-old woman with Marfan's syndrome had chronic intractable headaches and spontaneous intracranial hypotension. The pain was concentrated over the occipitonuchal region, had elements of both migraine and tension headache, and was often aggravated by postural change. Myelography showed multiple, large, lumbosacral arachnoid diverticula. Radioisotope cisternography revealed a halolike accumulation in the lumbosacral region and rapid uptake of isotope in the urinary bladder, indicating cerebrospinal fluid leakage. Epidural blood patching brought immediate relief from the positional headaches. We concluded that patients with Marfan's syndrome and undifferentiated chronic headaches should be radiologically evaluated for spinal meningeal defects.
We investigated the genotype frequencies of patients with spinocerebellar ataxias (SCA), using a community-based prevalence study among 613,349 inhabitants in Tottori prefecture, Japan. Prevalence date was April 1, 1998. On this date, 109 SCA patients were identified in this community. The prevalence of SCA was 17.8 per 100,000 individuals. The most common cause of inherited SCA was a mutation at the SCA6 locus (25%), followed by mutation at the SCA1 locus (15%), SCA3 locus (5%) and dentatorubral-pallidoluysian atrophy locus (5%). None of the expanded alleles was found in SCA2, SCA7 or Friedreich’s ataxia. Mutation at SCA6 was also the most common form of sporadic SCA at 11%. Prevalences per 100,000 individuals were as follows: SCA6, 2.40; SCA1, 0.48; DRPLA, 0.32, and SCA3, 0.16.
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