Bickerstaff encephalitis
| Classification according to ICD-10 | |
|---|---|
| G04.8 | Bickerstaff encephalitis |
| ICD-10 online (WHO version 2019) | |
The Bickerstaff's encephalitis is a rare autoimmune inflammatory disease of the brain stem , which with disturbance of cranial nerves associated and often severe disorders of consciousness. It was first described by Edwin Bickerstaff in 1951 . Close relationships with Miller-Fisher syndrome have been discussed in recent years .
course
A flu-like prodromal stage with headache, fatigue and a slight increase in temperature is typical . Then it comes to severe disorders of the brain stem functions, one of the cranial nerves (with double vision , facial paralysis , dysphagia ), on the other hand, heavy disturbances of consciousness and ataxia . Since the long tracts running through the brain stem are also affected, the muscles' own reflexes are often changed and pyramidal tract signs appear (→ Babinski reflex ). The symptoms usually increase over one to four weeks. Due to the severity of the disease, longer intensive care treatment is often required. Recovery usually takes months to over a year, and the majority of those affected recover completely.
Diagnosis
Magnetic resonance imaging reveals inflammatory changes in the area of the bridge , midbrain and partly in the thalamus . In the cerebrospinal fluid is inflammatory cells found slightly elevated and (thus also signs of inflammation) proliferated protein and ganglioside antibodies (GQ1b antibodies - as with Miller Fisher syndrome). Electrophysiologically, the evoked potentials ( SEP and AEP ) are particularly noticeable.
therapy
Due to the rarity of the disease, there are no evidence-based guidelines for treatment. Due to the similarity to the other diseases associated with ganglioside antibodies, treatment is usually carried out with plasmapheresis or immunoglobulins . Individual reports on positive effects are documented in the specialist literature.
Individual evidence
- ↑ Bickerstaff ER: Mesencephalitis and rhombencephalitis . In: Br Med J . 2, No. 4723, 1951, pp. 77-81. doi : 10.1136 / bmj.2.4723.77 . PMID 14848512 . PMC 2069534 (free full text).
- ↑ Bickerstaff ER: Brain-stem encephalitis: further observations on a grave syndrome with benign prognosis . In: Br Med J . 1, No. 5032, 1957, pp. 1384-7. doi : 10.1136 / bmj.1.5032.1384 . PMID 13436795 . PMC 1973653 (free full text).
- ↑ Overell include: Treatment for Fisher syndrome, Bickerstaff's brainstem encephalitis and related disorders. In: Cochrane Database Syst Rev. 2007; (1), S. CD004761. PMID 17253522
