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

B A glial nodule composed of microglia in the medulla oblongata. CSF samples were tested at the various hospitals. This suggests that elements of the autoimmune mechanism are common to both, and that they are not distinct but are closely related conditions. Effective management and treatment of BBE and other variants of Anti-Gq1b syndrome requires prompt recognition and bicerstaff.

There is also a need for randomized controlled trials to evaluate if certain therapeutic agents or procedures may hasten recovery for patients with BBE. BrainVolumeIssue 10, 1 OctoberPages —, https: Needle electromyography was performed in six patients.

Ataxic gait disappeared, and on day 35 he could stand on one foot. 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. Purchase access Subscribe to JN Learning for one year. The biickerstaff of disturbance of consciousness in ehcephalitis with BBE with overlapping GBS was almost the same for drowsiness and for stupor, semicoma and coma. Wendy Terry Publisher wterry bmctoday.

Three patients had residual symptoms; one each had psychic change, dementia and dysphagia.

On days 4, 5, 8, 9, 10, 11 and 12, he underwent seven sessions of immunoadsorption. Create a free personal brainstsm to access your subscriptions, sign up for alerts, and more. Almost all the patients had a monophasic remitting course and generally a good outcome. Therefore, patients are generally classified as having BBE rather than MFS if there is evidence of corticospinal tract abnormalities, a disturbed state of consciousness, or a significant brainstem lesion on MRI or at autopsy.


Our patient was treated with corticosteroids, which seemed to have a positive therapeutic effect. This page was last edited on 17 Marchat Slurred speech was present, but there was no facial or oropharyngeal palsy. Treatment of the 62 patients was as follows: Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic. In this report, we describe a patient who had BBE with a number of remarkable features. The aetiology of this syndrome was speculated to be similar to that of GBS because of evidence of prodromal upper respiratory infection, areflexia and CSF albuminocytological dissociation.

Anti-GQ1b antibodies are found in two-thirds brainsfem patients with this condition.

In this condition, neuronal excitability can spread laterally to adjacent axons that may have an abnormally lowered threshold for firing as a result of demyelination. Six Nos 1, 2, 3, 5, 7 and 8 of the eight cases described by Bickerstaff fulfil our tentative criteria for BBE, whereas none satisfy the criteria for FS Table 1.

On day 29 there was no extraocular muscles paresis, except for limitation of abduction. Although the clinical picture is severe, the disease course is generally monophasic with complete remission of symptoms within 6 months in over half of the patients.

Limitation of ocular movement was detected in the lateral gaze of both eyes. Additional examples of Anti-Gq1b syndrome include acute axonal neuropathy and the pharyngeal-cervical-brachial GBS variant.

The immunological profile common to BBE and FS lends supports to a common pathogenesis, but it does not provide the answer to bickersstaff question of whether these syndromes are separate entities. It is also possible that there is more than one autoimmune disease that can cause an illness which would currently be diagnosed bickerstavf Bickerstaff’s. The clinical, electrophysiological and immunological findings reported in our study support the original hypothesis of Bickerstaff and Cloakethat BBE is closely related to GBS and that they form a continuous spectrum.

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Various treatments of BBE have been used in the past, including corticosteroids, intravenous immunoglobulin, and plasmapheresis.

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

Information brainsfem the following was obtained from each primary physician: Drafting of the manuscript: It should be noted not all cases present classically with this triad of features, and absence of one does not rule out the diagnosis. In order to diagnose Bickerstaff brainstem encephalitis, ataxia and ophthalmoplegia must be present. The patient had dysmetria and slowed rapid alternating movements in the upper extremities.

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. The age of the patient was not at all typical for patients with BBE and bickerwtaff with previously presented cases. Cases of prolonged mild disturbance of consciousness and acute encephalopathy.

Yet it should be noted that bickwrstaff antibody testing does not preclude the diagnosis: