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A case-control and nerve biopsy study of CREST multiple mononeuropathy
P J Dyck1, G G Hunder, P J Dyck
1Peripheral Neuropathy Research Laboratory, Mayo Clinic and Mayo Foundation, Rochester, MN 14304, USA.
Neurology
|December 31, 1997
Summary
Peripheral neuropathy is more common in CREST syndrome patients. Necrotizing vasculitis is identified as the cause of this multiple mononeuropathy in individuals with CREST syndrome.
Area of Science:
- Neurology
- Rheumatology
- Pathology
Background:
- CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasis) is a variant of systemic sclerosis.
- Peripheral neuropathy is a potential complication, but its cause in CREST syndrome is not well-established.
Purpose of the Study:
- To investigate the prevalence and cause of peripheral neuropathy in patients with CREST syndrome.
Main Methods:
- Clinical evaluation of 7 CREST syndrome patients with unexplained neuropathy.
- Nerve conduction studies and sural nerve biopsies.
- Histopathological analysis of nerve biopsies for inflammation and vasculitis.
- Comparison with age, sex, and referral-matched control subjects.
Main Results:
- Seven out of 536 CREST syndrome patients had unexplained peripheral neuropathy.
- Multiple mononeuropathy was significantly more frequent in CREST patients (6/7) than controls (0/2).
- Sural nerve biopsies showed multifocal fiber loss, perivascular inflammation, and evidence suggestive of necrotizing vasculitis in most cases.
Conclusions:
- Peripheral neuropathy, specifically multiple mononeuropathy, occurs more frequently than by chance in CREST syndrome.
- Necrotizing vasculitis is the underlying cause of peripheral neuropathy in CREST syndrome patients.