Rinshō shinkeigaku = Clinical neurology
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The Multiple Sclerosis Quality of Life-54 instrument (MSQOL-54) is a specific quality of life (QOL) scale in English for multiple sclerosis (MS). It is composed of 54 items, and is a combination of the 36-item short form health survey (SF-36) and 18 disease-specific questions, such as fatigue, mental sexual and cognitive dysfunction. We developed the Japanese-translated version of MSQOL-54. ⋯ In comparison with an original article in English, the validation of the Japanese-translated version MSQOL-54 may be acceptable. There were no correlations between the results of the Japanese-translated version MSQOL-54 and EDSS except for physical function and physical health composite score. The Japanese-translated version of MSQOL-54 may provide unique information not readily evaluated by EDSS, and may be useful as clinical outcome measures in patients with MS.
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We report a 73-year-old man who presented symptoms of low back pain and SIADH followed by weakness of all four limbs and sensory disturbance of the lower legs 2 month after the first symptoms. He was referred to our department because of the evolution of weakness. Neurological examination on admission revealed weakness of the arms and legs, areflexia, and hypoesthesia of the lower legs. ⋯ He was diagnosed as having CIDP complicating with SIADH. An association between SIADH and AIDP has been much reported previously. To our knowledge, however, there has been no report of SIADH complicating with CIDP.
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Non-invasive ventilatory therapy has prolonged survival of myopathy patients with hypoventilation. Efficacy of non-invasive ventilation depends on both elastance and resistance of the respiratory system. Although these parameters are important in the prescription of respiratory management, conventional respiratory function test does not show the appropriate answer in patients with severe respiratory muscle weakness. In muscular dystrophy, muscle tends to be shortened due to its fibrosis, when muscle becomes atrophic and weak; fibrosis of respiratory muscle tissues presumably causes high thoracic elastance. We evaluated the total respiratory system elastance and resistance during proportional assist ventilation (PAV) in myopathy patients. ⋯ The respiratory system elastance and resistance measured during PAV are useful parameters in evaluation of mechanical features of the lung, thorax and airway. It is recommended to keep both parameters normal in patients who may require ventilatory assist due to progression of respiratory muscle weakness.
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The incidence and mechanism of diplopia were investigated in 31 patients with Wallenberg's syndrome resulting from acute dorsolateral medullary infarction. Diplopia was found in 10 of 31 patients (32%), with 5 patients reporting vertical diplopia alone and 5 reporting vertical and horizontal diplopia. Diplopia in Wallenberg's syndrome is considered to be caused by a lesion involving the otolith-ocular system. ⋯ Concomitant horizontal diplopia may require involvement of the medial longitudinal fasciculus (MLF), which produces skew deviation in mirror image; the unaffected eye becomes deviated inferiorly. When downward deviation of the eye affected by dysfunction of the vestibular nucleus and that due to MLF dysfunction affecting the other eye are comparable, only horizontal diplopia becomes apparent. MLF syndrome may be accompanied by paralytic pontine exotropia (PPE) or non-paralytic pontine exotropia (NPPE), both of which may also participate in the appearance of horizontal diplopia.
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In Japan, we have first alerted the necessity of critical care neurology (CCN) and neuro-ICU in 2000, however, CCN is still unrecognized. In the United States and Germany, CCN has become recognized as one of the important subspecialty in neurology, and also, there is progressive increase of neuro-intensive care unit (neuro-ICU) after 1990s. In this symposium, the present status, problems, and future direction of clinical study and practice in CCN were reviewed from the standpoints as follows; 1 Quantitative and sequential analysis of literatures in CCN: MEDLINE search disclosed marked increase in CCN-related literatures since 1990s. 2 Categorical analysis of important literatures selected by German Society of CCN. 3 Analysis of clinical practice in CCN through neuro-ICU simulation study: in 442 inpatients who admitted to our department during 1998. 4 Analysis of requisites in critical care in neurology practice both in diagnostic and therapeutic aspects. 5 Historical development of CCN. 6 Evaluation of the effect of neuro-ICU. 7 Short-term and long-term problems for the activation of CCN. In conclusion, 1) CCN is still immature in Japan, and needs early establishment, including neuro-ICU and educational system for developing neurointensivist, 2) Extraction of problems, decision-making, and rapid differential diagnosis are critical to prevent and manage life-threatening neurological conditions.