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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On day 3 he remained alert. Similarly, raised CSF protein levels and pleocytosis are frequent but non-specific. Create a personal account to register for email alerts with links to free full-text articles. 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.

The majority of documented cases of BBE have shown patients to regain baseline functional status within 6 months of diagnosis. Laboratory studies of the identifiable causes of disorientation analysis of blood gas, blood sugar, sodium, creatinine, thiamin, renin activity and ammonia were all normal. Ataxic gait disappeared, and on day 35 he could stand on one foot. Roos, Goldenberg, and Badruddin.

A Perivascular lymphocytic infiltration with surrounding oedema in the medulla oblongata. Encephalomyelitis Acute disseminated Myalgic Meningoencephalitis. Analysis and interpretation of data: A tentative diagnosis of BBE was made, and the patient was transferred to the neurological intensive care unit. Degenerative SA Friedreich’s ataxia Ataxia-telangiectasia.

The patient had transient and very frequent episodes of brainstem dysfunction during the recovery phase. Anti-Gq1b antibody is one that interacts with the peripheral nerve ganglioside, Gq1b. On April 6, the patient was noted to have bilateral Babinski signs with unsustained ankle clonus; she became more difficult to arouse, with extensor posturing on the right side.


A ecephalitis MRI showed extension of the brainstem involvement without new lesions. In the 37 patients who had BBE with limb weakness, muscle weakness was symmetrical and flaccid. It is therefore possible that a proportion of cases are due to other causes, such as infection or lymphomabut remain undiagnosed.

Bickerstaff brainstem encephalitis – Wikipedia

Formal tests of limb coordination showed bickersatff and dysmetria. BrainVolumeIssue 10, 1 OctoberPages —, https: Miller Fisher described cases of ophthalmoplegia and ataxia in a edition of. There are no randomised controlled trials of immunomodulatory therapy in Fisher Syndrome or related disorders on which to base practice. Paraffin sections stained with haematoxylin and eosin.

The episodes increased bickdrstaff frequency so that they occurred up to hundreds of times a day. At about the same time, C. Hassan T, Mumford C. Evidence of recent Campylobacter jejuni infection was tested serologically, as reported elsewhere Koga et al.

One month before admission, the patient had developed a severe nonproductive cough that was diagnosed as interstitial pneumonitis and treated with moxifloxacin hydrochloride. The immunological profile common to BBE and FS lends supports to a common pathogenesis, but it does not provide the answer to the question of whether these syndromes are separate entities.

Disease definition Bickerstaff’s brainstem encephalitis BBE is a rare post-infectious neurological disease characterized by the association of external ophthalmoplegia, ataxia, lower limb arreflexia, extensor plantar response and disturbance of consciousness drowsiness, stupor or coma.

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

Three days before admission, the patient encephwlitis dysarthria followed by diplopia, clumsiness, and ataxia. The discovery that a large number of affected patients tested positive for the Anti-Gq1b antibody led to a greater understanding of BBE. An autopsy was performed.

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The majority of patients achieved spontaneous recovery without the need for treatment despite the alarming initial presentation of BBE. Bicoerstaff alerts New issue alert. All studies of Fisher Syndrome and its clinical variants were scrutinised for data on patients treated with any form of acute immunotherapy.

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.

Neurological examination revealed mild bilateral blepharoptosis, limitation of horizontal gaze and incomplete convergence. By the evening he could not swallow. Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic.

Bickersraff reflexes were absent in 11, normal in three and brisk in four. Electrophysiological examination on day 9 showed reduced amplitudes of CMAPs with normal motor conduction velocities in the median, ulnar and tibial nerves, indicative of primary axonal degeneration Table 2. On April 10, the patient was more responsive and a tetraparesis became apparent.

Deep tendon reflexes were diminished in the upper limbs and absent in the lower ones. Create a free personal account to download free article PDFs, sign up for alerts, and more. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: