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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Plantar responses were indifferent.

All tendon jerks were absent. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.

Because of frequent spontaneous recovery and rare occurrence, today there is a lack of generalized consensus on the role of specific treatments for BBE. Kufs or not Kufs: Bbickerstaff Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss.

Bronchial mucosal lymphocytic infiltration was detected in the lungs, indicative of systemic infective processes. Report of a case.

Orphanet: Bickerstaff brainstem encephalitis

It should be brainstej not all cases present classically with this triad of features, and absence of one does not rule out the diagnosis. But in contradistinction to BBE, these patients had no change in sensorium and almost universally these patients were areflexic.

A remarkable feature of our patient was the presence of brief paroxysmal episodes of brainstem dysfunction that appeared after recovery of much of her neurological function. A magnetic resonance angiogram showed no abnormalities.


Additional examples of Anti-Gq1b syndrome include acute axonal neuropathy and the pharyngeal-cervical-brachial GBS variant. For advertising rates and opportunities: It furthers the University’s objective of excellence in research, scholarship, and education by publishing worldwide. B A glial nodule composed of microglia in the medulla oblongata.

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

C Trigeminal motor nucleus showing beainstem chromatolysis. Citing articles via Web of Science Until this is available, your continued use of this site will be deemed as consent to use of cookies. He considered that six of the 18 cases were typical FS, and the other 12 represented obscure brainstem lesions without peripheral polyneuropathy.

Four of the patients had been admitted to our hospital and the others were referred to our neuroimmunological laboratory for serum antiganglioside antibody testing from hospitals throughout Japan between December and February These changes were bickkerstaff as evidence of encephalitis.

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. Ninety-two per cent of the patients involved had encephalutis an antecedent illness. There was also very marked perivascular round cell cuffing, sometimes with numerous macrophages.

Bickerstaff brainstem encephalitis

On day 7, he was drowsy but easily aroused by stimulation. Yet it should be noted that negative antibody testing does not preclude the diagnosis: An electroencephalogram showed marked generalized slowing in the theta range, suggestive of an encephalopathy. Since no such clinical trials were discovered, all retrospective case series containing five or more patients were assessed and summarised in the discussion section.


Focal lymphocytic infiltration was seen in the dorsal root ganglia. Roos, Goldenberg, and Badruddin. We found no randomised or non-randomised prospective controlled trials of immunotherapy in Fisher Syndrome or related disorders.

Bickerstaff brainstem encephalitis – Wikipedia

Brain Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic. Various treatments of BBE have been used in the past, including corticosteroids, intravenous immunoglobulin, bickerztaff plasmapheresis. A second MRI showed extension of the brainstem involvement without new lesions. The episodes increased in frequency so that they occurred up to hundreds of times a bicierstaff.

The diagnosis was made quickly, partly through the use brqinstem magnetic resonance imaging MRI studies. These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory feaures of BBE. In order to diagnose Bickerstaff brainstem encephalitis, ataxia and ophthalmoplegia must be present.

There is also a need for randomized controlled trials to evaluate if certain therapeutic agents or procedures may hasten recovery for patients with BBE.