Bickerstaff’s Brainstem Encephalitis (BBE) is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute. This condition has been named Bickerstaff’s brainstem encephalitis (BBE). One patient had gross flaccid weakness in the four limbs. Presumably because of the . Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by Edwin Bickerstaff in It may also affect the .

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In a review of cases of Anti-Gq1b Syndrome, antecedent illness was seen in 94 percent of patients, with upper respiratory tract infection being most common.

Almost all the patients had a monophasic remitting course and generally a good outcome. Data collection and analysis: Active denervation potentials positive sharp waves or fibrillation potentials were present in three, with limb weakness on days 21— To clarify its clinical features, we reviewed detailed clinical profiles and laboratory findings for 62 cases of BBE diagnosed by the strict criteria of progressive, relatively symmetrical external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia.

BBE, not FS, therefore was the diagnosis for those who showed drowsiness.

Sign in to access your subscriptions Sign in to your personal account. Copyright Brainwtem Medical Association. A Perivascular lymphocytic infiltration with surrounding oedema in the medulla oblongata. The brainstem had perivascular lymphocytic infiltration with perivascular oedema Fig. All of these variants are associated with anti-GQ1b IgG antibodies. A cervical spine MRI showed no abnormalities. Over the next few weeks, the episodes disappeared and the patient returned to her normal baseline condition.


D ICD – The trigeminal motor nucleus showed marked central chromatolysis of the neurons Fig. In this report, we describe a patient who had BBE with a number of remarkable features.

Treatment for Fisher syndrome, Bickerstaff’s brain stem encephalitis and related disorders

Slurred speech was present, but there was no facial or oropharyngeal palsy. On day 3 he remained alert. The episodes of brainstem dysfunction in our patient resembled similar events reported by Andermann et al 10 and Espir et al. Brain herniation Reye’s Hepatic encephalopathy Toxic encephalopathy Hashimoto’s encephalopathy.

Privacy Policy Terms of Use. Our findings based on electrodiagnostic criteria suggest that motor nerve dysfunction is predominantly axonal and that the form of GBS that overlaps with BBE may be the axonal subtype, i. In our case, the presence of Babinski signs, a disturbed state of consciousness, and the dramatic MRI signal abnormalities in the brainstem indicated a diagnosis of BBE. Table 4 shows abnormal MRI and neurophysiological findings.

Bickerstaff brainstem encephalitis – Wikipedia

Table 3 shows the clinical profiles of the 62 patients, 37 of whom were men and 25 women, aged 3—91 years median, 39 years. He considered that six of the 18 cases were typical FS, and the other 12 represented obscure brainstem lesions without peripheral polyneuropathy.

The brain weighed brainsyem.

Anti-GQ1b antibodies are found in two-thirds of patients with this condition. CSF studies showed some degree of elevated protein concentration with and without pleocytosis in most patients with BBE during the first 4 weeks, but some patients were normal throughout the course of the illness. The episodes increased in frequency so that they occurred up to hundreds of times a day.


Critical revision of the manuscript for important intellectual content: Etiology BBE has been reported to occur following infection with several agents including cytomegalovirus, Campylobacter jejuniand Mycoplasma pneumonie. The documents contained in this web site are presented for information purposes only.

Orphanet: Bickerstaff brainstem encephalitis

This page was last edited on 17 Marchat In the 37 patients who had BBE with limb weakness, muscle weakness was symmetrical and flaccid. Purchase access Subscribe to JN Learning for one year. On day 7, auditory evoked brainstem brainstdm showed the absence of waves II—V on both sides Fig. Launched inPractical Neurology is a publication uniquely dedicated to presenting encwphalitis approaches to patient management, synthesis of emerging research and data, and analysis of industry news with a goal to facilitate practical application and improved clinical practice for all neurologists.

Despite the fact that this elderly woman had significant neurological deficits and serious medical bainstem, she eventually returned to her baseline status, highlighting the importance of aggressive, supportive treatment in what can be a completely reversible process.