MCID: BCK018
MIFTS: 21

Bickerstaff Brainstem Encephalitis

Categories: Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Bickerstaff Brainstem Encephalitis

MalaCards integrated aliases for Bickerstaff Brainstem Encephalitis:

Name: Bickerstaff Brainstem Encephalitis 58 36
Bickerstaff's Brainstem Encephalitis 71

Characteristics:

Orphanet epidemiological data:

58
bickerstaff brainstem encephalitis
Inheritance: Not applicable; Age of onset: All ages; Age of death: any age;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

KEGG 36 H01842
ICD10 via Orphanet 33 G61.0
UMLS via Orphanet 72 C1960543
Orphanet 58 ORPHA79138
UMLS 71 C1960543

Summaries for Bickerstaff Brainstem Encephalitis

KEGG : 36 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. 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.

MalaCards based summary : Bickerstaff Brainstem Encephalitis, also known as bickerstaff's brainstem encephalitis, is related to encephalitis and guillain-barre syndrome. Affiliated tissues include brain, cerebellum and spinal cord.

Wikipedia : 74 Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first... more...

Related Diseases for Bickerstaff Brainstem Encephalitis

Diseases related to Bickerstaff Brainstem Encephalitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 46)
# Related Disease Score Top Affiliating Genes
1 encephalitis 11.0
2 guillain-barre syndrome 10.9
3 ataxia and polyneuropathy, adult-onset 10.9
4 kearns-sayre syndrome 10.9
5 miller fisher syndrome 10.7
6 ocular motor apraxia 10.5
7 demyelinating disease 10.5
8 aceruloplasminemia 10.4
9 quadriplegia 10.4
10 neuropathy 10.4
11 pathologic nystagmus 10.4
12 autoimmune disease 10.3
13 polyneuropathy 10.3
14 facial paralysis 10.3
15 central nervous system disease 10.3
16 axonal neuropathy 10.3
17 nervous system disease 10.3
18 dysphagia 10.3
19 encephalopathy 10.3
20 spondyloarthropathy 1 10.2
21 systemic lupus erythematosus 10.2
22 helicobacter pylori infection 10.2
23 stroke, ischemic 10.2
24 hydrops, lactic acidosis, and sideroblastic anemia 10.2
25 sleep apnea 10.2
26 ptosis 10.2
27 respiratory failure 10.2
28 inflammatory spondylopathy 10.2
29 locked-in syndrome 10.2
30 critical illness polyneuropathy 10.2
31 chronic inflammatory demyelinating polyradiculoneuropathy 10.2
32 demyelinating polyneuropathy 10.2
33 blepharospasm 10.2
34 peripheral nervous system disease 10.2
35 spondylitis 10.2
36 cerebrovascular disease 10.2
37 subacute delirium 10.2
38 48,xyyy 10.2
39 antisynthetase syndrome 10.2
40 autoimmune encephalitis 10.2
41 multifocal motor neuropathy 10.2
42 pure autonomic failure 10.2
43 myoclonus 10.2
44 tremor 10.2
45 acute motor axonal neuropathy 10.2
46 acute motor and sensory axonal neuropathy 10.2

Graphical network of the top 20 diseases related to Bickerstaff Brainstem Encephalitis:



Diseases related to Bickerstaff Brainstem Encephalitis

Symptoms & Phenotypes for Bickerstaff Brainstem Encephalitis

Drugs & Therapeutics for Bickerstaff Brainstem Encephalitis

Search Clinical Trials , NIH Clinical Center for Bickerstaff Brainstem Encephalitis

Genetic Tests for Bickerstaff Brainstem Encephalitis

Anatomical Context for Bickerstaff Brainstem Encephalitis

MalaCards organs/tissues related to Bickerstaff Brainstem Encephalitis:

40
Brain, Cerebellum, Spinal Cord, Testes

Publications for Bickerstaff Brainstem Encephalitis

Articles related to Bickerstaff Brainstem Encephalitis:

(show top 50) (show all 189)
# Title Authors PMID Year
1
A Probable Case of Recurrent Bickerstaff Brainstem Encephalitis With Fulminant Course in a Pediatric Patient. 61
31876654 2020
2
Acute psychosis secondary to atypical Bickerstaff brainstem encephalitis. 61
31593905 2019
3
Paroxysmal Dysarthria Ataxia-Tremor-Blepharospasm Syndrome in Bickerstaff Brainstem Encephalitis: A Variant of Paroxysmal Dysarthria Ataxia Syndrome. 61
31744988 2019
4
Acute vestibular syndrome associated with anti-GQ1b antibody. 61
31399495 2019
5
Antecedent infections in Fisher syndrome: sources of variation in clinical characteristics. 61
30955122 2019
6
Guillain-Barré syndrome and related diseases after influenza virus infection. 61
31355311 2019
7
[Clinical features of 19 children with Bickerstaff brainstem encephalitis]. 61
31060129 2019
8
Clinical spectrum of the anti-GQ1b antibody syndrome: a case series of eight patients. 61
30747336 2019
9
Bickerstaff Brainstem Encephalitis and overlapping Guillain-Barré syndrome in children: Report of two cases and review of the literature. 61
30502045 2019
10
Long-term Sequelae of Pediatric Bickerstaff Brainstem Encephalitis Includes Autonomic and Sleep Dysregulation. 61
30604651 2019
11
An overlapping case of Bickerstaff brainstem encephalitis and acute motor axonal neuropathy variant of Guillain-Barré syndrome associated with systemic lupus erythematosus. 61
30444497 2019
12
Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis. 61
30564620 2018
13
Varied antibody reactivities and clinical relevance in anti-GQ1b antibody-related diseases. 61
30246056 2018
14
Optic neuropathy and decorticate-like posture as presenting symptoms of Bickerstaff's brainstem encephalitis: A case report and literature review. 61
30144776 2018
15
[Cerebellar and brainstem hypoperfusion in Bickerstaff's brainstem encephalitis: a case report]. 61
30270334 2018
16
Overlap of Bickerstaff brainstem encephalitis/Guillain-Barré syndrome simulating brain death. 61
30233023 2018
17
A Case Report of Overlapping Miller Fisher Syndrome, Guillain-Barré Syndrome, and the Bickerstaff Brainstem Encephalitis. 61
29953036 2018
18
Miller Fisher syndrome, Bickerstaff brainstem encephalitis and Guillain-Barré syndrome overlap with persistent non-demyelinating conduction blocks: a case report. 61
30031375 2018
19
Bickerstaff's brainstem encephalitis with overlapping Guillain-Barré syndrome: Usefulness of sequential nerve conduction studies. 61
29459060 2018
20
The incidental finding of elevated anti GQ1B antibodies in a patient with selective small fiber neuropathy. 61
29627021 2018
21
Headache and Ophthalmoparesis: Case Report of an "Atypical" Incomplete Miller-Fisher Syndrome. 61
29878345 2018
22
Anti-ganglioside complex antibody profiles in a recurrent complicated case of GQ1b-seronegative miller fisher syndrome and Bickerstaff brainstem encephalitis: a case report. 61
29792178 2018
23
Bickerstaff brainstem encephalitis in a patient with ankylosing spondylitis on tumour necrosis factor-alpha inhibitor. 61
29465358 2018
24
Miller-Fisher syndrome complicated by Bickerstaff brainstem encephalitis: A case report. 61
29489680 2018
25
Pediatric Bickerstaff brainstem encephalitis: a systematic review of literature and case series. 61
29177548 2018
26
Prediction of disease progression in Miller Fisher and overlap syndromes. 61
29065229 2017
27
Early electrophysiological findings in Fisher-Bickerstaff syndrome. 61
28888467 2017
28
A case of Bickerstaff brainstem encephalitis with transient reflex myoclonus. 61
28637936 2017
29
Guillain-Barré Syndrome, variants & forms fruste: Reclassification with new criteria. 61
28514704 2017
30
Acute disseminated encephalomyelitis following Campylobacter jejuni gastroenteritis: Case report and review of the literature. 61
27888275 2017
31
Cerebrospinal fluid findings in Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathies. 61
29110767 2017
32
Posterior reversible encephalopathy as the first manifestation of Bickerstaff's brainstem encephalitis. 61
27821089 2016
33
Altered gene expression of glycosyltransferases and sialyltransferases and total amount of glycosphingolipids following herpes simplex virus infection. 61
27588895 2016
34
An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord. 61
27540012 2016
35
Clinical classification of 103 Japanese patients with Guillain-Barré syndrome. 61
27653863 2016
36
Optic Neuritis in a Patient with Bickerstaff's Brainstem Encephalitis. 61
27486938 2016
37
Proposal for a New Exercise Method for Dysphagia with Velopharyngeal Inadequacy: A Case of Bickerstaff's Brainstem Encephalitis. 61
27628607 2016
38
Poster 345 Rehabilitation of Bickerstaff Brainstem Encephalitis: A Case Report. 61
27673099 2016
39
Paediatric brainstem encephalitis associated with glial and neuronal autoantibodies. 61
26918533 2016
40
[Bickerstaff brainstem encephalitis after upper respiratory infection]. 61
27406055 2016
41
Long-term effect of rituximab in a case with late-onset Rasmussen´s encephalitis with anti-ganglioside IgGQ1b and anti-GAD antibodies positivity. Case Report. 61
27618602 2016
42
How often and when Fisher syndrome is overlapped by Guillain-Barré syndrome or Bickerstaff brainstem encephalitis? 61
26969889 2016
43
Generalized pan-autonomic failure as a presenting sign of Bickerstaff's brainstem encephalitis. 61
26780560 2016
44
Recurrent Guillain-Barré syndrome, Miller Fisher syndrome and Bickerstaff brainstem encephalitis. 61
27084218 2016
45
An Overlapping Case of Miller Fisher Syndrome, Bickerstaff's Encephalitis, and the ASMAN Variant of Guillain-Barre Syndrome. 61
27974981 2016
46
Rehabilitation of a patient with overlap of acute transverse myelitis and Bickerstaff's brainstem encephalitis: a case report. 61
28053734 2016
47
Teaching NeuroImages: Extensive vasogenic edema in Bickerstaff brainstem encephalitis. 61
26810429 2016
48
Relapsing-Remitting Severe Bickerstaff's Brainstem Encephalitis - Case Report and Literature Review. 61
28096906 2016
49
Recurrent Diplopia in a Pediatric Patient with Bickerstaff Brainstem Encephalitis. 61
27293928 2016
50
Facial diplegia: etiology, clinical manifestations, and diagnostic evaluation. 61
26677119 2015

Variations for Bickerstaff Brainstem Encephalitis

Expression for Bickerstaff Brainstem Encephalitis

Search GEO for disease gene expression data for Bickerstaff Brainstem Encephalitis.

Pathways for Bickerstaff Brainstem Encephalitis

GO Terms for Bickerstaff Brainstem Encephalitis

Sources for Bickerstaff Brainstem Encephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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