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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Focal lymphocytic infiltration was seen in the dorsal root ganglia. Bickerstaff considered these changes to be caused by cerebral oedema secondary to viral infection or to be the result of hypersensitivity to infection. Our understanding of BBE has greatly evolved with the recognition of the role of Anti-Gq1b antibodies in its pathogenesis.

BBE, not FS, therefore was the diagnosis for those who showed drowsiness.

Ataxic gait disappeared, and on day 35 he could stand on one foot. Evidence of recent Campylobacter jejuni infection was tested serologically, as brinstem elsewhere Koga et al.

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

These are also diagnostic features of Miller Fisher syndromeand bickerstzff Bickerstaff’s brainetem only diagnosed if other features are present which exclude Miller Fisher syndrome.

With these strict criteria, BBE, not FS, was diagnosed for patients with drowsiness, although in Case 2 in the original report by Fisher there was mild drowsiness.

The age of the patient was not at all typical for patients with BBE and contrasts with previously presented cases. According to our BBE diagnostic criteria, the condition was diagnosed for 62 of the 98 patients.


To provide the best available evidence from randomised controlled trials on the role of acute immunomodulatory therapy in the treatment brainsstem Fisher Syndrome and related disorders. All tendon jerks were absent. The episodes increased in frequency so that they occurred up to hundreds of times a day. Information on the outcome was then collated and summarised.

Magnetic resonance imaging showed a decrease in the abnormal signal in the brainstem with no evidence of recent stroke FigureD and E. Electrodiagnostic study results suggested peripheral motor axonal degeneration. Additional examples bicierstaff Anti-Gq1b syndrome include acute axonal neuropathy and the pharyngeal-cervical-brachial GBS variant.

On April 10, the patient was more responsive and a tetraparesis became apparent. Purchase access Subscribe to JN Learning for one year.

Bickerstaff brainstem encephalitis – Wikipedia

About Practical Neurology Launched inPractical Neurology is a publication uniquely dedicated brainetem presenting current approaches to patient management, synthesis of emerging research and data, and analysis of industry news with a goal to facilitate practical application and improved clinical practice for all neurologists.

Results of the sensory examination were normal. In the 37 patients who had BBE with limb weakness, muscle weakness was symmetrical and flaccid. Plantar responses were indifferent. These patients have been shown to have a delayed onset of recovery for poorly understood reasons. He underwent four sessions of immunoadsorption, on days 2, 5, 7 and Sign in to access your subscriptions Sign in to your personal account.

btainstem We found no randomised or non-randomised prospective controlled trials of immunotherapy in Fncephalitis Syndrome or related disorders. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. For a diagnosis of BBE, the following diseases must be excludable: This review was undertaken to systematically assess any available randomised controlled data on acute immunomodulatory therapies in Fisher Syndrome or its variants.


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. The clinical features and course of the condition, the associated auto-antibodies against brainetem antigens, and the response to treatment, all suggest that Bickerstaff brainstem encephalitis is an autoimmune disease.

The median CSF protein concentration after the second week was higher than that during the first week.

Bickerstaff brainstem encephalitis

On day 18 the distal and proximal CMAP amplitudes had further decreased, but the motor conduction velocity continued to be normal. Brain Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic.

Light reflexes encephalitid prompt. Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic.

Dissecting out migraine complexity through comprehensive analysis of allodynia. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: