Corticosteroid-resistant bulbar neurosarcoidosis responsive to intravenous immunoglobulin.
Pract Neurol
; 15(4): 289-92, 2015 Aug.
Article
em En
| MEDLINE
| ID: mdl-25935926
ABSTRACT
We report an intriguing case of corticosteroid-resistant bulbar neurosarcoidosis responding to intravenous immunoglobulin. A 37-year-old man presented with dysphagia to solids and liquids, dysphonia, fatigue and 50â
lb weight loss over 2â
months. We suspected sarcoidosis, based on an elevated serum angiotensin-converting enzyme concentration and hilar lymphadenopathy on chest imaging; we subsequently confirmed this after transbronchial biopsy found non-caseating granulomas. MR scan of brain was normal; barium swallow showed severe oropharyngeal dysphagia and electromyography identified bulbar muscle denervation. He took corticosteroids for 3â
weeks without improvement, requiring a percutaneous endoscopic gastrostomy tube for nutrition, but then he promptly improved with a 2-day course of intravenous immunoglobulin. Although there have been a few reports of intravenous immunoglobulin helping peripheral neurosarcoidosis, this case suggests that it also helps bulbar neurosarcoidosis. This case shows that bulbar neurosarcoidosis can mimic the clinical and electrophysiological features of fatal neurological disorders such as progressive bulbar palsy. The case illustrates the diagnostic challenge particularly when imaging is inconclusive and there is no response to corticosteroids. It also suggests that intravenous immunoglobulin can be considered before cytotoxic therapy for corticosteroid-resistant neurosarcoidosis, particularly in decompensated patients, given its favourable side effect profile. We also review the literature on bulbar neurosarcoidosis.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Sarcoidose
/
Doenças do Sistema Nervoso Central
/
Imunoglobulinas Intravenosas
/
Fatores Imunológicos
Tipo de estudo:
Prognostic_studies
Limite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Pract Neurol
Ano de publicação:
2015
Tipo de documento:
Article
País de afiliação:
Estados Unidos