Microangiopathy of vasa nervorum in dysglobulinemic neuropathy

H. C. Powell, M. Rodriguez, R. A C Hughes

Research output: Contribution to journalArticle

43 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)386-394
Number of pages9
JournalAnnals of Neurology
Volume15
Issue number4
StatePublished - 1984

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Vasa Nervorum
Globulins
Capillary Permeability
Demyelinating Diseases
Myelin Sheath
Axons
Ischemia
Pericytes
Paraproteinemias
gamma-Globulins
Peripheral Nervous System Diseases
Peripheral Nerves
Nerve Fibers
Amyloid
Blood Vessels
Blood Proteins
Electron Microscopy
Coloring Agents

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Powell, H. C., Rodriguez, M., & Hughes, R. A. C. (1984). Microangiopathy of vasa nervorum in dysglobulinemic neuropathy. Annals of Neurology, 15(4), 386-394.

Microangiopathy of vasa nervorum in dysglobulinemic neuropathy. / Powell, H. C.; Rodriguez, M.; Hughes, R. A C.

In: Annals of Neurology, Vol. 15, No. 4, 1984, p. 386-394.

Research output: Contribution to journalArticle

Powell, HC, Rodriguez, M & Hughes, RAC 1984, 'Microangiopathy of vasa nervorum in dysglobulinemic neuropathy', Annals of Neurology, vol. 15, no. 4, pp. 386-394.
Powell HC, Rodriguez M, Hughes RAC. Microangiopathy of vasa nervorum in dysglobulinemic neuropathy. Annals of Neurology. 1984;15(4):386-394.
Powell, H. C. ; Rodriguez, M. ; Hughes, R. A C. / Microangiopathy of vasa nervorum in dysglobulinemic neuropathy. In: Annals of Neurology. 1984 ; Vol. 15, No. 4. pp. 386-394.
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