Chronic progressive sensory ataxic neuropathy associated with limited systemic sclerosis.
J Neurol Sci
; 241(1-2): 103-6, 2006 Feb 15.
Article
em En
| MEDLINE
| ID: mdl-16336975
ABSTRACT
We report the case of a 33-year-old woman with limited systemic sclerosis and chronic progressive sensory ataxic neuropathy. Sural nerve biopsy showed loss of myelinated fibers mostly those of large diameter, axonal degeneration and infiltration of macrophages, but no signs of vasculitis. Physical examination, laboratory testing, neurophysiological and neuroradiological examinations suggested that the dorsal root was primarily affected in this patient. Cytokine analysis by multiplex bead array assay revealed that IL-1beta and GM-CSF were increased both in serum and CSF. Although her symptoms did not respond to corticosteroid therapy, intravenous immunoglobulin (IVIg) therapy resulted in marked improvement. IVIg could be effective in case of immune-mediated reversible neuronal dysfunction associated with collagen disease without vasculitis.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Ataxia
/
Transtornos de Sensação
/
Doenças do Sistema Nervoso Periférico
Tipo de estudo:
Risk_factors_studies
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
J Neurol Sci
Ano de publicação:
2006
Tipo de documento:
Article
País de afiliação:
Japão