A 63-year-old man presented with an 11-month history of progressive myoclonus in the right abdominal wall. Administration of clonazepam reduced the frequency and amplitude. When the therapy was discontinued, the frequency and amplitude of the myoclonus increased, and synchronous and weak myoclonus also was observed in the left abdomen. The trunk was twisted just after the appearance of the abdominal myoclonus associated with myoclonic jerks spreading from the rostral to caudal paraspinal muscles. Later in the clinical course, the myoclonus became stimulus sensitive and was induced by tendon tap given anywhere on the body, with the latency ranging from 50 to 150 ms irrespective of the sites of tapping. Myoclonus seen in the abdominal wall was segmental and considered to be of spinal origin. The reflex myoclonus had a 150-ms refractory period. It can be postulated that increased excitability of anterior horn cells at a certain segment might make a spino-bulbo-spinal reflex manifest at the corresponding segment. This myoclonus is considered to be a new form of spinal reflex myoclonus, because the abdominal myoclonic jerk seems to trigger another myoclonic jerk involving the paraspinal muscles.
Background: Although white matter changes visible with MRI are generally considered to result from ischemia, it has become clear that these changes also appear in patients with Alzheimer’s disease (AD). However, their significance in AD is unknown. Objective: We evaluated the clinical significance of white matter changes in AD. Methods: Ninety-six AD patients (79.4 ± 5.92 years old) and 48 age-matched control subjects (80.0 ± 7.03 years old) participated in the study. Three neuroradiologists assessed the degree of periventricular hyperintensities (PVH) and deep white matter hyperintensities (DWMH) using a modified Fazekas’ rating scale. We examined whether there was a difference in the severity and the histogram pattern of the white matter changes, or in vascular factors (hypertension, diabetes mellitus, and ischemic heart disease) between the two groups. We also analyzed the association between the severity of the white matter changes and the degree of dementia (MMSE score and disease duration). Results: There were no differences in the vascular factors between AD and control subjects. The degree of PVH in AD was severe compared with that in the control subjects. In histograms of the number of subjects with each degree of PVH severity, the distribution of AD patients had peaks at both the low and intermediate degrees of PVH, while most of the controls had a low degree of PVH. There was no difference in the degree or the histogram pattern of DWMH between the two groups. The severity of white matter changes was not associated with severity of dementia in AD. Conclusions: Although PVH might have several causative factors, and may have some clinical significance, the change itself does not contribute to the progression of AD.
The prevalence of dementia in Japan in 1985 for people aged 65 years or older was 4.8%. Vascular dementia (VD) has been reported to be the commonest subtype. A report from Tokyo in 1970 showed that it was 2.8 times more frequent than Alzheimer type dementia (SDAT). We assessed the prevalence of dementia in a rural area (population: 12,931. 25.3% were 65 years or older in 1994) in Kyoko. First, we questioned subjects about their demographic circumstances, memory disturbance, apraxia (agnosia) and daily activities. Our questionnaire was answered by 3,132 (95.8%) subjects, and 2,280 of them agreed to be examined by neurologists. Those who met the appropriate criteria of the DSM-III-R and NINCDS-ADRDA were diagnosed with dementia. By use of the Hachinski ischemic score (HIS), we distinguished VD from non-vascular dementia. In this study patients with non-vascular dementia were diagnosed with SDAT by the neurologists. Analysis of the data revealed that 4.8% of the study population was demented. According to the HIS results, only 3 of 15 had vascular dementia. The prevalence of dementia was the same as the average prevalence in Japan, even though the elderly population of this town was twice as high as the average. It is hard to determine the prevalence of dementia in any community. Many factors must be taken into consideration: the coverage rate, the criteria for dementia, and whether to include institutionalized residents. The prevalence of dementia will increase with the aging of the population, and we must collect accurate data in order to plan efficiently.
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