Peripheral neuropathy associated with erythrophagocytic lymphohistiocytosis
A 12 year old patient who developed clinical, biochemical and histological features of erythrophagocytic lymphohistiocytosis is described. In contrast to previously reported cases, the prominent neurological feature was a subacute sensorimotor polyneuropathy. Sural nerve biopsy showed a marked reduc...
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Published in | Journal of neurology, neurosurgery and psychiatry Vol. 51; no. 2; pp. 291 - 294 |
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Main Authors | , , , , , , , |
Format | Journal Article |
Language | English |
Published |
London
BMJ Publishing Group Ltd
01.02.1988
BMJ BMJ Publishing Group LTD |
Subjects | |
Online Access | Get full text |
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Summary: | A 12 year old patient who developed clinical, biochemical and histological features of erythrophagocytic lymphohistiocytosis is described. In contrast to previously reported cases, the prominent neurological feature was a subacute sensorimotor polyneuropathy. Sural nerve biopsy showed a marked reduction of myelinated fibres and severe axonal lesions, absence of histiocyte infiltration and deposits of IgM along the epineurium. In addition to the hypertriglyceridaemia previously described in this condition, an elevation of plasma very long-chain fatty acids and phytanic acid was found which suggests a transient impairment of peroxisomal functions. |
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Bibliography: | istex:D3435FB1591FC4DE270982F58020E4956E2895DD ark:/67375/NVC-5F2G4FHB-J PMID:3346698 href:jnnp-51-291.pdf local:jnnp;51/2/291 ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3 |
ISSN: | 0022-3050 1468-330X |
DOI: | 10.1136/jnnp.51.2.291 |