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CHRONIC DEMYELINATING POLYRADICULONEUROPATHY ASSOCIATED WITH MALIGNANT HISTIOCYTOSIS Yasuhito Watahiki 1 , Yutaka Hirata 1 , Ken Nagata 1 , Yuichi Satoh 1 , Masayuki Baba 2 , Kazuharu Kudoh 3 , Akira Miura 3 1Department of Neurology, Research Institute for Brain and Blood Vessls-Akita 2Department of Neurology, CVD Research Institute, Hirosaki University School of Medicine 3Third Department of Internal Medicine, Akita University School of Medicine pp.405-410
Published Date 1989/4/1
DOI https://doi.org/10.11477/mf.1406206300
  • Abstract
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A case of malignant histiocytosis (MH) pre-senting with peripheral nerve involvement is des-cribed.

A 67-year-old man initially noted left facial weakness on July 20, 1986. The symptom was improved within a week, however, two weeks later he noticed numbness in his fingers and a burning pain in his legs, which was followed by double vision and progressive weakness in all four limbs. On examination, he was found to have total ophthalmoparesis of the right eye and a trace of the left facial palsy. There was severe weakness of the leg muscles, as well as mild weakness of the arms. This was accompanied by wasting of his limbs. All tendon reflexes were absent. Planter responses were flexor. Al-though cutaneous sensation was intact, vibratory sense was markedly impaired in the legs below the knees. The sphincter function was mildly disturbed. The sedimentation rate was 32 mm/hr. The hemoglobin was 11. 9 g/dl and the leukocyte count was 5, 700/mm3. The platelet count fell to 60, 000/mm3. Results of routine biochemical and radiological studies were unremarkable. The cere-brospinal fluid protein level was 129 mg/dl with a normal cell count. Motor nerve conduction study revealed marked reduction in amplitude of the compound muscle action potential, slow motor nerve conduction velocities and multifocal con-duction blocks along the nerve trunks (the left ulnar nerve in the forearm, the bilateral tibial nerve in the lower leg). F-wave was absent or elicitable with prolonged latency and with in-creased chrono-dispersion. Sensory nerve had nor-mal or nearly normal conduction. EMG sampling showed an impaired interference pattern during voluntary contraction and a few denervation po-tentials at rest.

He was treated with prednisolone and his ophthalmoparesis was partially improved without obvious improvement in weakness in four limbs. Shortly thereafter, his weakness worsened again and pancytopenia developed. Bone marrow aspira-tion was hypoplastic and disclosed erythrophago-cytic histiocytes and immature reticulum cells. The latter were large and irregular, and had abundant basophilic cytoplasm and primitive nuc-lei. These findings were believed to be consistent with a MH. The patient died of pulmonary bleed-ing about five months after the onset of his initial neurological symptoms. Permission for post-mortem examination was not given.

MH, also called histiocytic medullary reticulosis, is a relatively rare disease. The clinical features of MH include fever, wasting, generalized lym-phadenopathy, and hepatosolenomegaly, freauentivaccompanied by jaundice and pancytopenia. Al-though skin and central nervous system involve-ment has been described, relatively little attention has been directed toward the peripheral nerve involvement. The present case is the first Japanesecase of peripheral neuropathy associated with MH, in which electrophysiological evidence of conduc-tion block was confirmed.


Copyright © 1989, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 2185-405X 印刷版ISSN 0006-8969 医学書院

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