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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One patient had gross flaccid weakness in the four limbs. To clarify its clinical features, we reviewed encephzlitis clinical profiles and laboratory findings for 62 cases braisntem BBE diagnosed by the strict criteria of progressive, relatively symmetrical external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia.

Information on the following was obtained from each primary physician: 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.

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.

Orphanet: Bickerstaff brainstem encephalitis

Motor nerve conduction velocities were not reduced in the median, ulnar and tibial nerves, whereas CMAP amplitudes were decreased markedly Table 2. Randomised controlled trials are needed to establish the value of immunotherapies or other treatments. Diagnosis is based on the clinical findings, patient history, cerebrospinal fluid CSF analysis revealing raised protein levelsdetection of anti-GQ1b IgG antibodies not present in all patientsMRI studies revealing high-intensity abnormalities in the posterior fossa, white matter or thalami and neurophysiological encephakitis electroencephalogram and electromyography indicative of central nervous system and predominantly axonal involvement.

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Purchase access Subscribe to the journal. Migraine Familial hemiplegic Cluster Tension. Needle electromyography results confirmed active denervation potentials in three patients studied 3—8 weeks after the onset. There are no randomised controlled trials of immunomodulatory therapy in Fisher Syndrome or related disorders on which to base practice. There was also very marked perivascular round cell cuffing, sometimes with numerous macrophages.


In the acute phase of disease, BBE may be so severe that there is complete ophthalmoplegia, facial diplegia and full paralysis of arms and legs, resembling ‘brain-death’.

Our study was undertaken to clarify the clinical, electrophysiological, neuroimaging and immunological features of a large number of patients with BBE. There was no significant difference in the clinical profiles bicksrstaff the two subgroups. The patient had transient and very vickerstaff episodes of brainstem dysfunction during the recovery phase. Data collection and analysis: Three days before admission, the patient developed dysarthria followed by diplopia, clumsiness, and ataxia.

The most supportive laboratory investigation is positive Anti-Gq1b antibodies, bickertsaff in the setting of the appropriate clinical context. 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.

Anti-Gq1b antibody is one that interacts with the peripheral nerve ganglioside, Gq1b. This interesting condition has characteristic signs and symptoms including altered sensorium that should be kept in mind in the setting of new onset ataxia. MRI findings in a remitting—relapsing case wncephalitis Bickerstaff encephalitis. Deep tendon reflexes were diminished in the upper limbs and absent in the lower ones.

Bickerstaff considered these changes to be caused by cerebral oedema secondary to viral infection or to be the result of hypersensitivity to infection. Similarly, raised CSF protein levels and pleocytosis are frequent but non-specific. Prednisone therapy was begun. CSF studies showed some degree of elevated protein concentration with and without pleocytosis in most patients with BBE during encephalitiz first 4 weeks, but some patients were normal throughout the course of the illness.

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Bickerstaff brainstem encephalitis

In this article we review some of the key features of BBE including pathophysiology, epidemiology, presentation and investigations, differential diagnosis, and management and treatment. The majority of documented cases of BBE have shown patients to regain baseline functional status within 6 brainsrem of diagnosis.


Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches blckerstaff receive search alerts. Like some other autoimmune diseasesthe condition usually follows a minor infection, such as a respiratory tract infection or gastroenteritis.

Acute axonal neuropathy is also commonly preceded by infection. All of these variants are bicketstaff with anti-GQ1b IgG antibodies.

Prognosis 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. A few days later, she noticed very frequent, stereotypic episodes lasting 1 to 10 seconds and occurring throughout the day; these episodes consisted of transient diplopia accompanied by dysarthria if talking and gait imbalance if walking.

There was no weakness. The next day there was worsened diplopia and ataxic gait.

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

Encephalomyelitis Acute disseminated Myalgic Meningoencephalitis. One of the present authors M. Sign in to save your search Sign in to your personal account. Table 5 shows CSF findings during the first week, the second week, and the third encelhalitis fourth weeks. D ICD – Sign in to make a comment Sign in to your personal necephalitis.

These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory feaures of BBE.

Roos, Soliven, Goldenberg, Badruddin, and Baron. BBE can occur without any preceding symptoms, but the majority of cases see a preceding bicierstaff illness. These are also diagnostic features of Miller Fisher syndromeand so Bickerstaff’s is only diagnosed if other features are present which exclude Miller Fisher syndrome.

Paraffin sections stained with haematoxylin and eosin.

Electrodiagnostic study results suggested peripheral motor axonal degeneration.