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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Orphanet: Bickerstaff brainstem encephalitis

Three of the 62 patients died during the course of illness, but the outcome for the BBE patients was generally good. Because of frequent spontaneous recovery and rare occurrence, today there is a lack of generalized consensus on the role of specific treatments for BBE. 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.

Episodes that involve more complex abnormalities are not common but have been described. All tendon jerks were absent. Despite the fact that this elderly woman had significant neurological deficits and serious medical problems, she eventually returned to her baseline status, highlighting the importance of aggressive, supportive treatment in what can be a completely reversible process.

Wendy Terry Publisher wterry bmctoday. In addition, we present pathological findings for a patient with BBE. In BBE MRI brain findings are variable, where imaging can be unremarkable or hypointense foci on Tweighted images and hyperintense foci on T2-weighted images can be seen.


During these episodes, the patient exhibited transient ataxia, diplopia, and dysarthria—deficits that had been seen on a continuous basis earlier in the disease course. Between episodes, results of an examination showed minimal dysarthria, full eye movements, no ptosis, a questionable right Babinski sign, slight clumsiness, and a slow gait with a negative Romberg sign.

Results of the sensory examination were normal.

Bickerstaff brainstem encephalitis – Wikipedia

Purchase access Subscribe now. Sign in to make a comment Sign in to your personal account. The cerebellum showed occasional grumose degeneration in the dentate nucleus. Management and treatment Management is based on immunotherapy with intravenous immunoglobulin IVIg or plasma exchange.

Detailed information Professionals Clinical practice guidelines Deutsch Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. To provide the best available evidence from randomised controlled trials on the role of acute immunomodulatory therapy in the treatment of Fisher Syndrome and related disorders.

His pupils were dilated and light reflexes were absent.

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

Intravenous bdainstem IVIg and plasma exchange are often used as treatments in this patient group. Healthy Cognitive Aging There are evidence-based tools for staying sharp throughout the lifespan. This systematic review found no randomised controlled trials of treatments for Fisher syndrome, Bickerstaff’s brain stem encephalitis or their variants. Check this box if you wish to receive a copy of your message.


Over the next several days, the brainstej improved in mental state, ptosis, eye movements, and renal function and was therefore extubated. A recent Cochrane review was not able to give any specific recommendations for the treatment of both BBE and MFS because of an apparent lack of randomized trials evaluating treatment in such clinical settings.

Our present day resources bear no clinical guidelines that describe specific diagnostic criteria for BBE. But in contradistinction to BBE, these patients had no change in sensorium and almost universally these patients were areflexic.

Limb strength in the arms and legs was 4 on the MRC scale. Health care resources for this disease Expert centres Diagnostic tests 2 Patient organisations 18 Orphan drug s Yet it should be noted enfephalitis negative antibody testing does not preclude the diagnosis: By day 73, nystagmus had disappeared. Controlled clinical trials are needed to vickerstaff this proposal. BBE patients definitely have CNS involvement that is responsible for disturbance of consciousness, extensor plantar responses and hemisensory loss, but not all the features of BBE, including ophthalmoplegia and ataxia, are necessarily explained by central lesions.

On day 18 the distal and proximal CMAP amplitudes had further decreased, but the motor conduction velocity continued to be normal.