| H01842 | |
| H number | H01842 |
| Name | Bickerstaff brainstem encephalitis |
| Description | Bickerstaff's brainstem encephalitis (BBE) is a rare immune disorder in children, characterized by progressive ophthalmoplegia, ataxia, and disturbance of consciousness. Although the etiology of BBE is unknown, it has been linked to various antecedent infections. BBE is associated with the anti-GQ1b IgG antibody, which is present in 66% of a population of patients who had BBE. An autoimmune mechanism produced by microbial infection may trigger its pathogenesis. Intravenous immunoglobulin (IVIg) is effective in immune-mediated disorders such as Fisher syndrome (FS) and Guillain-Barre syndrome (GBS). IVIg is also effective in treatment of BBE, which is similar to the clinical and immunologic features of FS or GBS. Combined therapy of IVIg and high-dose methylprednisolone is a more efficacious therapy than IVIg alone. Most patients make a clinical recovery over six months. |
| Category | Immune system disease; Nervous system disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | See also H01436 Guillain-Barre syndrome and H01689 Fisher syndrome. |
| Other DBs | ICD-11: 8C01.0 ICD-10: G61.0 |
| Reference | PMID:25379047 AUTHORS Rho YI TITLE Overlapping Guillain-Barre syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus. JOURNAL Korean J Pediatr 57:457-60 (2014) DOI:10.3345/kjp.2014.57.10.457 PMID:22984203 AUTHORS Shahrizaila N, Yuki N TITLE Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome. JOURNAL J Neurol Neurosurg Psychiatry 84:576-83 (2013) DOI:10.1136/jnnp-2012-302824 PMID:14706228 AUTHORS Matsuo M, Odaka M, Koga M, Tsuchiya K, Hamasaki Y, Yuki N TITLE Bickerstaff's brainstem encephalitis associated with IgM antibodies to GM1b and GalNAc-GD1a. JOURNAL J Neurol Sci 217:225-8 (2004) DOI:10.1016/j.jns.2003.09.008 |