Rinshō shinkeigaku = Clinical neurology
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Case Reports
[A case of Wernicke-Korsakoff syndrome caused by gastrojejunostomy--specific findings of MRI and SPECT].
A 63-year-old woman with Wernicke-Korsakoff syndrome, despite the absence of alcoholism and malnutrition, is reported. She had undergone gastrojejunostomy for ileus 30 years ago, and this operation was thought to be an important risk factor for her vitamin B1 deficiency. ⋯ Presumably this phenomenon explains the Korsakoff psychosis. A history of gastrojejunostomy, even if normal intake is possible, is a risk factor for vitamin B1 deficiency, and should be considered in the differential diagnosis of Wernicke-Korsakoff syndrome.
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Review Case Reports
[A case of ornithine transcarbamylase deficiency presenting severe symptoms in adulthood].
An adult female case of ornithine transcarbamylase (OTC) deficiency is presented in the following. The patient had had past episodes of drowsiness with a duration less than a few minutes several times a year during childhood. She suddenly became comatose at 25 years of age, and died after 13 months of persistent vegetative state. ⋯ OTC activity of a liver specimen was 65 percent of the normal level. This is a rare case demonstrating hyperglycinemia and an elevated level of serum OTC. The importance of ruling out defective ureagenesis in adults with disturbed consciousness should be emphasized.
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A 48-year-old female with multiple sclerosis (MS) accompanied by intractable hiccups of over one month' duration and the sleep apnea syndrome was reported. This MS patient had been well controlled until September 16, 1991 when she experienced nausea, vomiting and hiccups. The patient was admitted to Kawasaki Medical School Hospital on October 9, 1991. ⋯ These symptoms disappeared following the administration of amitriptyline. There have been few reports of the combination of intractable hiccups and the sleep apnea syndrome in MS. The MRI findings suggest that the causative lesion of these symptoms is in the tegmentum of the medulla oblongata.
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In contrast to juvenile rigid form of Huntington's disease (HD) in which myoclonus is often seen, only 5 patients with myoclonus complicating adult HD have been reported. We herein described an adult HD patient who suffered from severe action myoclonus leading to physical disability. To our knowledge, this is the first case report in Japan. ⋯ Clonazepam (4 mg a day) was instituted with a pronounced reduction in myoclonus and a return to her previous level of daily life activity. Although myoclonic jerks are often recognized in juvenile patients with rigid form of HD, they have been considered to exert a minor influence on physical disability. By contrast, our present observation and review of literature suggest that myoclonus may lead to severe motor impairment in adult HD.