Chronic inflammatory axonal polyneuropathy.
J Neurol Neurosurg Psychiatry
; 91(11): 1175-1180, 2020 11.
Article
em En
| MEDLINE
| ID: mdl-32917820
ABSTRACT
OBJECTIVES:
Chronic inflammatory axonal polyneuropathy (CIAP) is defined on the basis of the clinical, electrophysiological and nerve biopsy findings and therapeutic responses of 'immunotherapy responding chronic axonal polyneuropathy (IR-CAP)'.METHODS:
The diagnosis of IR-CAP was made when all of three of the following mandatory criterion were met (1) acquired, chronic progressive or relapsing symmetrical or asymmetrical polyneuropathy with duration of progression >2 months; (2) electrophysiological evidence of axonal neuropathy in at least two nerves without any evidence of 'strict criteria of demyelination'; and (3) definite responsiveness to immunotherapy.RESULTS:
Thirty-three patients with IR-CAP showed similar clinical features of chronic inflammatory demyelinating polyneuropathy (CIDP) except 'motor neuropathy subtype'. High spinal fluid protein was found in 27/32 (78%) cases. 'Inflammatory axonal neuropathy' was proven in 14 (45%) of 31 sural nerve biopsies. DISCUSSIONS IR-CAP could well be 'axonal CIDP' in view of clinical similarity, but not proven as yet. Thus, IR-CAP is best described as CIAP, a distinct entity that deserves its recognition in view of responsiveness to immunotherapy.CONCLUSION:
Diagnosis of CIAP can be made by additional documentation of 'inflammation' by high spinal fluid protein or nerve biopsy in addition to the first two diagnostic criteria of IR-CAP.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Polineuropatias
/
Doenças Autoimunes do Sistema Nervoso
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Polirradiculoneuropatia Desmielinizante Inflamatória Crônica
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Inflamação
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article