Microangiopathy of vasa nervorum in dysglobulinemic neuropathy
- 1 April 1984
- journal article
- research article
- Published by Wiley in Annals of Neurology
- Vol. 15 (4), 386-394
- https://doi.org/10.1002/ana.410150414
Abstract
Thickening of vessel walls resulting from endothelial proliferation was observed in the vasa nervorum of eleven patients with peripheral neuropathy associated with dysglobulinemia. Electron microscopy showed endothelial proliferation accompanied by abnormal accumulation of masses of intracytoplasmic filaments in each case. Of the eleven patients with dysglobulinemia, nine had monoclonal gammopathy and two were found to have polyclonal elevation of gamma globulin levels. Symptoms of neuropathy characteristically preceded detection of serum protein abnormalities by months to years. Nerve fiber lesions involved primarily the axon in four cases; segmental demyelination was the principal abnormality in the other seven. Both abnormalities were present to some degree in all eleven patients. Biphasic myelinopathy with uniform separation of myelin lamellae attributable to globulin deposition was observed in four cases. The microvascular changes included endothelial cytoplasmic enlargement, virtually obliterating the vessel lumen in many instances, with thickening of pericytes, in which intracytoplasmic filaments were prominent and pinocytotic vesicles numerous. No extracellular filaments were noted, and amyloid stains were negative. Possible effects of these microvascular changes include ischemia resulting from severe vascular luminal narrowing, and altered vascular permeability. Severe loss of axons in this group of neuropathies may be the result of ischemia, whereas altered vascular permeability may admit globulin into the endoneurium, where it may directly affect the myelin sheath and precipitate demyelination.This publication has 22 references indexed in Scilit:
- IgM paraproteins with immunological specificity for a Schwann cell component and peripheral nerve myelin in patients with polyneuropathyJournal of the Neurological Sciences, 1983
- Monoclonal IgM K antibody precipitating with chondroitin sulfate C from patients with axonal polyneuropathy and epidermolysisNeurology, 1983
- Demyeliating neuropathy and monoclonal IgM antibody to myelin‐associated glycoproteinNeurology, 1983
- Prevalence of monoclonal protein in peripheral neuropathyNeurology, 1981
- The spectrum of peripheral neuropathy in myelomaNeurology, 1981
- Plasma-Cell Dyscrasia and Peripheral Neuropathy with a Monoclonal Antibody to Peripheral-Nerve MyelinNew England Journal of Medicine, 1980
- Cryoglobulinemic neuropathy: A pathological studyAnnals of Neurology, 1980
- Cytoplasmic filaments in intracerebral cortical vesselsAnnals of Neurology, 1978
- Waldenstrom's macroglobulinemia and neuropathyNeurology, 1975
- Polyneuropathy in Waldenström's macroglobulinaemiaJournal of the Neurological Sciences, 1974