TY - JOUR
T1 - Update on classification, epidemiology, clinical phenotype and imaging of the nonsystemic vasculitic neuropathies
AU - Collins, Michael P.
AU - Dyck, P. James B.
AU - Hadden, Robert D.M.
N1 - Publisher Copyright:
© 2019 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2019/10/1
Y1 - 2019/10/1
N2 - Purpose of reviewSingle-organ vasculitis of the peripheral nervous system (PNS) is often designated nonsystemic vasculitic neuropathy (NSVN). Several variants or subtypes have been distinguished, including migratory sensory neuropathy, postsurgical inflammatory neuropathy, diabetic radiculoplexus neuropathies, skin-nerve vasculitides, and, arguably, neuralgic amyotrophy. NSVN often presents as nondiabetic lumbosacral radiculoplexus neuropathy (LRPN). This review updates classification, clinical features, epidemiology, and imaging of these disorders.Recent findingsA recent study showed the annual incidence of LRPN in Olmstead County, Minnesota to be 4.16/100000:2.79/100000 diabetic and 1.27/100000 nondiabetic. This study was the first to determine the incidence or prevalence of any vasculitic neuropathy. In NSVN, ultrasonography shows multifocal enlargement of proximal and distal nerves. In neuralgic amyotrophy, MRI and ultrasound reveal multifocal enlargements and focal constrictions in nerves derived from the brachial plexus. Histopathology of these chronic lesions shows inflammation and rare vasculitis. Diffusion tensor imaging of tibial nerves in NSVN revealed decreased fractional anisotropy in one study.SummarySingle-organ PNS vasculitides are the most common inflammatory neuropathies. Neuralgic amyotrophy might result from PNS vasculitis, but further study is necessary. The usefulness of focal nerve enlargements or constrictions in understanding pathological mechanisms, directing biopsies, and monitoring disease activity in NSVN should be further investigated.
AB - Purpose of reviewSingle-organ vasculitis of the peripheral nervous system (PNS) is often designated nonsystemic vasculitic neuropathy (NSVN). Several variants or subtypes have been distinguished, including migratory sensory neuropathy, postsurgical inflammatory neuropathy, diabetic radiculoplexus neuropathies, skin-nerve vasculitides, and, arguably, neuralgic amyotrophy. NSVN often presents as nondiabetic lumbosacral radiculoplexus neuropathy (LRPN). This review updates classification, clinical features, epidemiology, and imaging of these disorders.Recent findingsA recent study showed the annual incidence of LRPN in Olmstead County, Minnesota to be 4.16/100000:2.79/100000 diabetic and 1.27/100000 nondiabetic. This study was the first to determine the incidence or prevalence of any vasculitic neuropathy. In NSVN, ultrasonography shows multifocal enlargement of proximal and distal nerves. In neuralgic amyotrophy, MRI and ultrasound reveal multifocal enlargements and focal constrictions in nerves derived from the brachial plexus. Histopathology of these chronic lesions shows inflammation and rare vasculitis. Diffusion tensor imaging of tibial nerves in NSVN revealed decreased fractional anisotropy in one study.SummarySingle-organ PNS vasculitides are the most common inflammatory neuropathies. Neuralgic amyotrophy might result from PNS vasculitis, but further study is necessary. The usefulness of focal nerve enlargements or constrictions in understanding pathological mechanisms, directing biopsies, and monitoring disease activity in NSVN should be further investigated.
KW - epidemiology
KW - lumbosacral radiculoplexus neuropathy
KW - neuralgic amyotrophy
KW - peripheral neuropathy
KW - ultrasonography
KW - vasculitis
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U2 - 10.1097/WCO.0000000000000727
DO - 10.1097/WCO.0000000000000727
M3 - Review article
C2 - 31313704
AN - SCOPUS:85071441419
SN - 1350-7540
VL - 32
SP - 684
EP - 695
JO - Current opinion in neurology
JF - Current opinion in neurology
IS - 5
ER -