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The Japanese Journal of Rehabilitation Medicine Volume 45, Issue 8 （August 2008）

The Japanese Journal of Rehabilitation Medicine 45巻8号（2008年8月発行）

Japanese English

《症例報告》

洞機能不全症候群および起立性頻脈症候群をきたした多発性硬化症の1例 A Case of Multiple Sclerosis with Sick Sinus Syndrome and Postural Orthostatic Tachycardia Syndrome 谷掛万里 ¹ , 降矢芳子 ¹ , 形岡博史 ¹ , 川原誠 ¹ , 平野牧人 ¹ , 上野聡 ¹ Mari TANIKAKE ¹ , Yoshiko FURUYA ¹ , Hiroshi KATAOKA ¹ , Makoto KAWAHARA ¹ , Makito HIRANO ¹ , Satoshi UENO ¹ ¹奈良県立医科大学神経内科 ¹Department of Neurology, Nara Medical University, Medial School キーワード：多発性硬化症（multiple sclerosis） , 洞機能不全症候群（sick sinus syndrome） , 起立性頻脈症候群（postural orthostatic tachycardia syndrome） , 延髄背側（dorsal medulla） , 右迷走神経核（right posterior nucleus of vagus） Keyword: 多発性硬化症（multiple sclerosis） , 洞機能不全症候群（sick sinus syndrome） , 起立性頻脈症候群（postural orthostatic tachycardia syndrome） , 延髄背側（dorsal medulla） , 右迷走神経核（right posterior nucleus of vagus） pp.535-540

発行日 2008年8月18日 Published Date 2008/8/18

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はじめに

　多発性硬化症（Multiple sclerosis，以下MS）は大脳，小脳，脳幹部等の中枢神経系をおかす脱髄性疾患である．今回我々は迷走神経核を含む延髄背側の脱髄巣により洞機能不全症候群（Sick sinus syndrome，以下SSS）および起立性頻脈症候群（Postural orthostatic tachycardia syndrome，以下POTS）を呈した症例を経験したのでここに報告する．

Abstract : A 19-year-old girl was admitted to our hospital with nausea, vomiting, hiccups, constipation and syncope. After hiccups or vomiting sinus arrest developed and lasted more than 5-8 seconds. She lost consciousness every one hour. Based on an electrocardiographic diagnosis of sick sinus syndrome (SSS), a temporary pacemaker was implanted. The next day, although her syncope and bradycardia disappeared, she had orthostatic tachycardia of over 120 beats/minute and swelling of the legs, which led to a diagnosis of postural orthostatic tachycardia syndrome (POTS). Neurologically, she showed the right-sided tongue deviation and parasympathetic system disorders revealed by coefficient of variation of R.R interval (CV_R-R), the Achner eye-ball pressure test, the valsalva ratio, and the head-up-tilt test. Brain MRI disclosed a small hyperintense lesion on a T2-weighted image with gadolinium enhancement in the right dorsal medulla including the hypoglottis nucleus and the posterior nucleus of vagus. After steroid pulse therapy (methyl prednisolone 1 g/day×3 days, 5 times) was administered, this lesion became smaller and finally disappeared. Before the lesion disappeared, she was able to begin rehabilitation by wearing elastic stockings and treatment with midodrine hydrochloride. The following year, she developed other MRI-proven brain lesions, suggestive of demyelination. Such a spinal and temporal distribution of lesions led to a diagnosis of multiple sclerosis (MS). A case of POTS caused by MS has not been reported previously, however, MS often affects the medullary paraventricular regions associated with autonomic failures. Autonomic failures often prevent patients from experiencing early rehabilitations. We should promptly give symptomatic treatment against autonomic failures, which leads to good patient recovery not only in patient vitality but also functionality.