Clinicopathological features of Churg-Strauss syndrome-associated neuropathy

Citation
N. Hattori et al., Clinicopathological features of Churg-Strauss syndrome-associated neuropathy, BRAIN, 122, 1999, pp. 427-439
Citations number
62
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
BRAIN
ISSN journal
0006-8950 → ACNP
Volume
122
Year of publication
1999
Part
3
Pages
427 - 439
Database
ISI
SICI code
0006-8950(199903)122:<427:CFOCSN>2.0.ZU;2-F
Abstract
We assessed the clinicopathological features of 28 patients with peripheral neuropathy associated with Churg-Strauss syndrome. Initial symptoms attrib utable to neuropathy were acute painful dysaesthesiae and oedema in the dys aesthetic portion of the distal limbs. Sensory and motor involvement mostly showed a pattern of mononeuritis multiplex in the initial phase, progressi ng into asymmetrical polyneuropathy, restricted to the limbs. Parallel loss of myelinated and unmyelinated fibres due to axonal degeneration was evide nt as decreased or absent amplitudes of sensory nerve action potentials and compound muscle action potentials, indicating acute massive axonal loss. E pineurial necrotizing vasculitis was seen in 54% of cases; infiltrates cons isted mainly of CD8-positive suppressor/cytotoxic and CD4-positive helper T lymphocytes. Eosinophils were present in infiltrates, but in smaller numbe rs than lymphocytes. CD20-positive B lymphocytes were seen only occasionall y. Deposits of IgG, C3d, IgE and major basic protein were scarce. The mean follow-up period was 4.2 years, with a range of 8 months to 10 years. Fatal outcome was seen only in a single patient, indicating a good survival rate . The patients who responded well to the initial corticosteroid therapy wit hin 4 weeks regained self-controlled functional status in longterm follow-u p (modified Rankin score was less than or equal to 2), while those not resp onding well to the initial corticosteroid therapy led a dependent existence (P < 0.01), In addition the patients with poor functional outcomes had sig nificantly more systemic organ damage caused by vasculitis (P < 0.05), Necr otizing vasculitis mediated by cytotoxic T cells, leading to ischaemic chan ges, appears to be a major cause of Churg-Strauss syndrome-associated neuro pathy, The initial clinical course and the extent of systemic vasculitic le sions may influence the long-term functional prognosis.