Abstract | BACKGROUND:
Systemic vasculitis has been classically categorized as a primary disorder, such as polyarteritis nodosa, Churg-Strauss syndrome, and Wegener granulomatous, or as a secondary process, representing a complication from a connective tissue disorder (eg, rheumatoid vasculitis), infection, medication, or malignancy. Peripheral neuropathy is a well-recognized consequence of systemic vasculitis due to peripheral nerve infarction with Wallerian degeneration. Rarely, neuropathy is the sole manifestation of vasculitis, referred to as nonsystemic vasculitic neuropathy (NSVN). These conditions are defined pathologically by tissue biopsy demonstrating disruption or destruction of the vessel wall with inflammatory cell infiltrates. REVIEW SUMMARY: The diagnosis of vasculitic neuropathy is straightforward in patients with an established diagnosis of systemic vasculitis and classic features of mononeuritis multiplex. Most patients have clinical features of a subacute, progressive, generalized but asymmetric, painful, sensorimotor polyneuropathy. Laboratory tests often indicate features of systemic inflammation, such as an elevated sedimentation rate or positive anti-neutrophil cytoplasmic antibody, and electrodiagnostic evaluation shows multiple mononeuropathies or a confluent, asymmetric axonal neuropathy. Nerve biopsy is necessary to establish the diagnosis in most cases, particularly in patients with NSVN. This review summarizes the current treatment of vasculitic neuropathy. CONCLUSION:
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Authors | Kenneth C Gorson |
Journal | The neurologist
(Neurologist)
Vol. 13
Issue 1
Pg. 12-9
(Jan 2007)
ISSN: 1074-7931 [Print] United States |
PMID | 17215723
(Publication Type: Journal Article, Review)
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Topics |
- Humans
- Nervous System Diseases
(pathology, physiopathology, therapy)
- Vasculitis
(pathology, physiopathology, therapy)
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