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J Korean Neurol Assoc. 1987;5(1):75-80.
- A Case Report of the POEMS syndrome
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M.S. Lee, M.D., I.N. Sunwoo, M.D., K.W. Kim, M.D., J.G. Chi, M.D.*
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Department of Neurology, College of Medicine, Yonsei University / Department of Pathology, College of Medicine, Seoul National University*
- POEMS증후군 1예 보고
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이명식, 선우일남, 김기환, 지제근
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연세대학교 신경과. 서울대학교 병리학.
- Abstract
- This is a case report of unusual multisystem disorder first described as a unique syndrome in 1968, the POEMS syndrome. The acronym POEMS stands for Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein, and Skin changes. The POEMS syndrome is also known as Crow-Fukase, or Takatsuki syndrome.
Since 1968, more than one hundred cases have been reported in Japan and also
several similar cases in other countries. As a as auther's knowledge, this may be the
first case of the POEMS syndrome in Korea.
The patient was a 32 years old male who had been admitted to the Severance
hospital because of subacutely progressive quadriparesis and sensory changes. The
patient was markedly emaciated and the skin was dark. On examination, the muslce
bulk was markedly decreased with moderate to severe general weakness and the
sensory functions were impaired in all modalities. The trunk and face were also
involved, but worse in distal part of the extremities. Hepatomegaly and testicular
hypotrophy was noticed on ;alpation. Laboratory studies disclosed hepatomegaly,
hypothyroidism, hypogonadism, IgG monoclonal gammopathy, and sclerotic bone lesion at
the thoracic spine.
There was only transient symptomatic improvement on steroid, but followed by
intractable progression. Complete atrio-ventricular conduction block with orthopnea
occurred as a terminal event and the patient was expired at 10 months of the course."
Keywords :
- 초록
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