Bickerstaff brainstem encephalitis
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Summary
Bickerstaff brainstem encephalitis (MONDO:0019208) is a disease. A subtype of postinfectious encephalitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: Unknown (Worldwide)
- Phenotypes (HPO): 47
Clinical features
Signs & symptoms
Clinical features (HPO)
47 HPO clinical features (Orphanet curated; top 47 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0000602 | Ophthalmoplegia | Very frequent (80-99%) |
| HP:0001251 | Ataxia | Very frequent (80-99%) |
| HP:0001289 | Confusion | Very frequent (80-99%) |
| HP:0002329 | Drowsiness | Very frequent (80-99%) |
| HP:0003403 | EMG: decremental response of compound muscle action potential to repetitive nerve stimulation | Very frequent (80-99%) |
| HP:0003431 | Decreased motor nerve conduction velocity | Very frequent (80-99%) |
| HP:0007305 | CNS demyelination | Very frequent (80-99%) |
| HP:0032169 | Severe infection | Very frequent (80-99%) |
| HP:0000651 | Diplopia | Frequent (30-79%) |
| HP:0001284 | Areflexia | Frequent (30-79%) |
| HP:0002090 | Pneumonia | Frequent (30-79%) |
| HP:0002094 | Dyspnea | Frequent (30-79%) |
| HP:0002270 | Abnormality of the autonomic nervous system | Frequent (30-79%) |
| HP:0002353 | EEG abnormality | Frequent (30-79%) |
| HP:0002922 | Increased CSF protein concentration | Frequent (30-79%) |
| HP:0003690 | Limb muscle weakness | Frequent (30-79%) |
| HP:0007131 | Acute demyelinating polyneuropathy | Frequent (30-79%) |
| HP:0007209 | Facial paralysis | Frequent (30-79%) |
| HP:0007256 | Abnormal pyramidal sign | Frequent (30-79%) |
| HP:0011947 | Respiratory tract infection | Frequent (30-79%) |
| HP:0012534 | Dysesthesia | Frequent (30-79%) |
| HP:0012696 | Abnormal thalamic MRI signal intensity | Frequent (30-79%) |
| HP:0030057 | Autoimmune antibody positivity | Frequent (30-79%) |
| HP:0000496 | Abnormality of eye movement | Occasional (5-29%) |
| HP:0000508 | Ptosis | Occasional (5-29%) |
| HP:0000639 | Nystagmus | Occasional (5-29%) |
| HP:0001259 | Coma | Occasional (5-29%) |
| HP:0001283 | Bulbar palsy | Occasional (5-29%) |
| HP:0001291 | Abnormal cranial nerve morphology | Occasional (5-29%) |
| HP:0001315 | Reduced tendon reflexes | Occasional (5-29%) |
| HP:0001348 | Brisk reflexes | Occasional (5-29%) |
| HP:0002273 | Tetraparesis | Occasional (5-29%) |
| HP:0002445 | Tetraplegia | Occasional (5-29%) |
| HP:0002878 | Respiratory failure | Occasional (5-29%) |
| HP:0003487 | Babinski sign | Occasional (5-29%) |
| HP:0004887 | Respiratory failure requiring assisted ventilation | Occasional (5-29%) |
| HP:0009916 | Anisocoria | Occasional (5-29%) |
| HP:0010628 | Facial palsy | Occasional (5-29%) |
| HP:0010831 | Impaired proprioception | Occasional (5-29%) |
| HP:0010871 | Sensory ataxia | Occasional (5-29%) |
| HP:0011499 | Mydriasis | Occasional (5-29%) |
| HP:0012229 | CSF pleocytosis | Occasional (5-29%) |
| HP:0012416 | Hypercapnia | Occasional (5-29%) |
| HP:0012531 | Pain | Occasional (5-29%) |
| HP:0030319 | Weakness of facial musculature | Occasional (5-29%) |
| HP:0031123 | Recurrent gastroenteritis | Occasional (5-29%) |
| HP:0100786 | Hypersomnia | Occasional (5-29%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | Bickerstaff brainstem encephalitis |
| Mondo ID | MONDO:0019208 |
| Orphanet | 79138 |
| ICD-11 | 163316971 |
| SNOMED CT | 427086003 |
| UMLS | C1960543 |
| MedGen | 743311 |
| GARD | 0018944 |
| Is cancer (heuristic) | no |
Disease family
This is a subtype of postinfectious encephalitis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › nervous system disorder › central nervous system disorder › encephalomyelitis › encephalitis › infectious encephalitis › postinfectious encephalitis › Bickerstaff brainstem encephalitis
Related subtypes (6): limbic encephalitis with LGI1 antibodies, Rasmussen subacute encephalitis, acute disseminated encephalomyelitis, encephalitis lethargica, steroid-responsive encephalopathy associated with autoimmune thyroiditis, rubella encephalitis
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 0.
Related Atlas pages
No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.