Bickerstaff brainstem encephalitis
MONDO:0019208Mondo
Findings
No curated finding names Bickerstaff brainstem encephalitis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Bickerstaff's brainstem encephalitis (BBE) is a rare post-infectious neurological disease characterized by the association of external ophthalmoplegia, ataxia, lower limb arreflexia, extensor plantar response and disturbance of consciousness (drowsiness, stupor or coma).
Definition from the Mondo Disease Ontology (MONDO:0019208), read 2026-09-29. CC BY 4.0.
Features
47 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- AtaxiaHPOHP:0001251
- Very frequent (80% to 99% of cases)
- CNS demyelinationHPOHP:0007305
- Very frequent (80% to 99% of cases)
- ConfusionHPOHP:0001289
- Very frequent (80% to 99% of cases)
- Decreased motor nerve conduction velocityHPOHP:0003431
- Very frequent (80% to 99% of cases)
- DrowsinessHPOHP:0002329
- Very frequent (80% to 99% of cases)
- EMG: decremental response of compound muscle action potential to repetitive nerve stimulationHPOHP:0003403
- Very frequent (80% to 99% of cases)
- OphthalmoplegiaHPOHP:0000602
- Very frequent (80% to 99% of cases)
- Severe infectionHPOHP:0032169
- Very frequent (80% to 99% of cases)
- Abnormal pyramidal signHPOHP:0007256
- Frequent (30% to 79% of cases)
- Abnormal thalamic MRI signal intensityHPOHP:0012696
- Frequent (30% to 79% of cases)
- Abnormality of the autonomic nervous systemHPOHP:0002270
- Frequent (30% to 79% of cases)
- Acute demyelinating polyneuropathyHPOHP:0007131
- Frequent (30% to 79% of cases)
Show the remaining 35
- AreflexiaHPOHP:0001284
- Frequent (30% to 79% of cases)
- Autoimmune antibody positivityHPOHP:0030057
- Frequent (30% to 79% of cases)
- DiplopiaHPOHP:0000651
- Frequent (30% to 79% of cases)
- DysesthesiaHPOHP:0012534
- Frequent (30% to 79% of cases)
- DyspneaHPOHP:0002094
- Frequent (30% to 79% of cases)
- EEG abnormalityHPOHP:0002353
- Frequent (30% to 79% of cases)
Where it sits
- A kind of