Nō to shinkei = Brain and nerve
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We report a 63-year-old man who died of respiratory failure. He was well until 1992 (57 years of his age), when he had an onset of progressive weakness of the bilateral upper limbs. He showed no improvement with TRH administration in other hospital. ⋯ Neither motor cortex nor cortico-spinal tracts were affected. Demyelination, remyelination or cellular infiltrations were not apparent in the right median nerve and sciatic nerves. The neuropathologic features were compatible with SPMA.
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The Short-Memory Questionnaire (SMQ) developed by Koss et al. is a standardized, validated reliable informant-based scale to assess everyday memory problems. In the previous study, we prepared its Japanese version and validated the reliability in assessing patients with Alzheimer Disease. In the present study, we examined the relations between the performance evaluated by the Japanese version of the SMQ combined with the Mini-Mental State Examination (MMSE) and demographic variables (age, sex, and education). ⋯ Low MMSE scores were related to low education level and older females. Low SMQ scores were not related to aging, but linked to low education level and males. Both the MMSE and SMQ, which can be easily administered, are affected by education years and sex. In addition, MMSE score depends on age. Therefore, careful consideration should be paid to age, education level and sex for future execution of the MMSE and SMQ.
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Case Reports
[A rare case of trapped fourth ventricle: an unique symptom appeared after fourth ventriculo-peritoneal shunting].
We experienced a unique case of trapped fourth ventricle after shunting for post-meningitic hydrocephalus. A five-year-old infant was diagnosed as meningitis shortly after his birth, and secondarily suffered from hydrocephalus. He underwent lateral-ventriculo-peritoneal shunting, fourth-ventriculo-cisterna-magna shunting and so on, but bilateral abducens palsy appeared. ⋯ We tried to improve his symptom in one way or another by keeping the fourth ventricle in appropriate volume. His abducens palsy was controlled by switching the on-off valve between forth ventricle and peritoneum. We expect that a higher-pressure programmable shunt valve or a lower-flow-regulating shunt system be invented in order to cope with the cases like ours.
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Case Reports
[Two cases in which the presence of ciliospinal response led to indecisiveness in the evaluation of brain death].
The ciliospinal reflex was first described by Budge in 1852. This reflex is used as an indicator of brain stem and autonomic nervous system functioning. In the Japanese guideline for determining brain death, the absence of this reflex is considered essential. ⋯ A noxious stimulation to the face will be registered through the brain stem, but if stimulation is in the neck or upper trunk, it may go directly to the spinal center. Because of the latter pathway to the spinal center, this reflex might remain in patients in whom the brain stem is completely nonfunctioning. Therefore, the presence of this reflex dose not always preclude a state of brain death.
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The purpose of this study was to investigate the clinical features, diagnosis, and treatment modalities of three cases with neurosarcoidosis, which involved the central nervous system (CNS). ⋯ The main pathological changes of neurosarcoidosis are granulomatous angitis of the venular walls and occasionally, of the capillaries near the meninx and Virchow-Robin space. The patients also had symptoms of secondary meningoencephalitis. These changes were mainly located in the hypothalamus and pituitary gland. The patients had complex symptoms resulting from endocrine system granuloma, as well as from cerebral ischemia. The severity of the disease and effectiveness of treatment, can be evaluated by measuring ACE levels in the cerebrospinal fluid (over 1. 0 IU/l), and by Gd-enhanced MRI. Early pulse steroid therapy with subsequent oral steroid administration is thought to be important for neurosarcoidosis treatment, in order to prevent irreversible damage in the CNS.