Neurofascin-155 IGG4 Neuropathy: Pathophysiological Insights, Spectrum of Clinical Severity and Response To treatment.
Muscle Nerve
; 57(5): 848-851, 2018 05.
Article
en En
| MEDLINE
| ID: mdl-29130507
ABSTRACT
INTRODUCTION:
Sensorimotor neuropathy associated with IgG4 antibodies to neurofascin-155 (NF155) was recently described. The clinical phenotype is typically associated with young onset, distal weakness, and in some cases, tremor.METHODS:
From a consecutive cohort of 55 patients diagnosed with chronic inflammatory demyelinating polyneuropathy, screening for anti-NF155 antibodies was undertaken. Patients underwent clinical assessment, diagnostic neurophysiology, including peripheral axonal excitability studies and nerve ultrasound.RESULTS:
Three of 55 chronic inflammatory demyelinating polyneuropathy patients (5%) tested positive for anti-NF155 IgG4. Patients presenting with more severe disease had higher antibody titers. Ultrasound demonstrated diffuse nerve enlargement. Axonal excitability studies were markedly abnormal, with subsequent mathematical modeling of the results supporting disruption of the paranodal seal.DISCUSSION:
A broad spectrum of disease severity and treatment response may be observed in anti-NF155 neuropathy. Excitability studies support the pathogenic role of anti-NF155 IgG4 antibodies targeting the paranodal region. Muscle Nerve 57 848-851, 2018.Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Inmunoglobulina G
/
Moléculas de Adhesión Celular
/
Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante
/
Factores de Crecimiento Nervioso
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Muscle Nerve
Año:
2018
Tipo del documento:
Article
País de afiliación:
Australia