What is Bickerstaffs brain stem encephalitis?
Bickerstaffs brain stem encephalitis is an inflammatory disorder of the brain stem, probably autoimmune mediated, but without specific etiology.
It is characterized by acute progressive cranial nerve dysfunction (such as diplopia, ophthalmoparesis, facial palsy, and bulbar palsy), associated cerebellar ataxia, and altered mental status.
Very often it follows an illness, and an association with certain infections, including cytomegalovirus, Campylobacter jejuni, typhoid fever, and Mycoplasma pneumoniae has been reported.
Serum anti-GQ1b IgG antibodies may be present in patients with this condition. Diagnosis is by cerebrospinal fluid analysis showing central nervous system inflammation.
Brain magnetic resonance imaging (MRI) scan may show contrast enhanced abnormal signals in the brain stem, but a normal MRI scan does not exclude the diagnosis.
Treatments include intravenous steroid, therapeutic plasma exchange, and other immunosuppressive agents.
Although the initial presentation may be severe, there is usually a good outcome with full recovery. Occasional recurrent cases may need repeat treatments.