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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Limb strength in the arms and legs was 4 on the MRC scale. This systematic review found no randomised controlled trials bickerstaf treatments for Fisher syndrome, Encwphalitis brain stem encephalitis or their variants.

At times, there is an overlap between BBE and GBS, which prompted Odaka et al 2 to classify some patients with BBE who had symmetrical flaccid limb weakness as having BBE associated with GBS; electrophysiological study results of these patients can suggest a motor axonal neuropathy, presumably representing an acute motor axonal neuropathy variant of GBS. To clarify its clinical features, we reviewed detailed clinical profiles and laboratory findings for bickerstatf cases of BBE diagnosed by the strict criteria of progressive, relatively symmetrical external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia.

Plantar responses were indifferent. Effective management and treatment of BBE and other variants of Anti-Gq1b syndrome requires prompt recognition and diagnosis. A few days later, she noticed very frequent, stereotypic episodes lasting 1 to 10 seconds and occurring throughout brainsrem day; these episodes consisted of transient diplopia accompanied by dysarthria if talking and gait imbalance if walking.

For a diagnosis of BBE, the following diseases must be excludable: There was proximal upper limb weakness, of 4 on the MRC scale. The EEG is often abnormal, but shows only slow wave activity, which also occurs in many other conditions, and so is of limited value in diagnosis.

From Wikipedia, the free encyclopedia. Our present day resources bear no clinical guidelines that describe specific diagnostic criteria for BBE. Deep tendon reflexes were absent, but extensor plantar responses were present.


Our patient’s episodes exhibited several brainstem abnormalities suggesting mass synchronous firing as a result of ephaptic transmission see Smith and McDonald 12 for a review.

The patient had dysmetria and slowed rapid alternating movements in the upper extremities. The association of BBE with Anti-Gq1b antibody syndrome is supported by infection being the mutual precipitant for a subsequent immune-mediated reaction. The clinical features and course of the condition, the associated auto-antibodies against relevant antigens, and the response to treatment, all suggest that Bickerstaff brainstem encephalitis is an autoimmune disease.

In a recent review, Odaka et al 2 defined BBE as a disorder with progressive ophthalmoplegia and ataxia and with features suggesting a central nervous system process, such as disturbed consciousness or hyperreflexia. Furthermore, a considerable number of patients with BBE developed flaccid tetraparesis, which is presumed to be a sign of overlapping GBS. CSF studies showed some degree of elevated protein concentration with and without pleocytosis in most patients with BBE during the first 4 weeks, but some patients were normal throughout the course of the illness.

Roos, Goldenberg, and Badruddin. The next year, Ropper criticized their report. Receive exclusive offers and updates from Oxford Academic. View large Download slide. On April 5, acyclovir administration was discontinued and mechanical ventilation for airway protection and hemodynamic stabilization was started.

Hassan T, Mumford C.

Bickerstaff brainstem encephalitis – Wikipedia

On day 7, auditory evoked brainstem responses showed the absence of waves II—V on both sides Fig. Ataxia most commonly presents with truncal and limb involvement, however solitary involvement can be seen as well.

The brainstem had perivascular lymphocytic infiltration with perivascular oedema Fig. Four of the patients had been admitted to our hospital encrphalitis the others were referred to our neuroimmunological laboratory for serum antiganglioside antibody testing from hospitals throughout Japan between December and February Purchase access Subscribe to the journal.

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.


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

Oxford University Press is a department of the University of Oxford. The brain weighed g. Etiology BBE has been reported to occur following infection with several agents including cytomegalovirus, Campylobacter jejuniand Mycoplasma pneumonie. There are no randomised controlled trials of immunomodulatory therapy in Fisher Syndrome or related disorders on which to base practice.

Vibration sense was mildly decreased in the lower limbs. Sign in to customize your interests Sign in to your personal account. It should be noted not all cases present classically with this triad of features, and absence of one does not rule out the diagnosis. Create a free personal account to download free article PDFs, sign up for alerts, and more. Inflammatory conditions, which affected the brainstem, may have exerted a certain influence on dentate neurons.

In order to diagnose Bickerstaff brainstem encephalitis, ataxia and ophthalmoplegia must be present. Management and treatment Management is based on immunotherapy with intravenous immunoglobulin IVIg or plasma exchange.

Results of serological studies on a variety of respiratory pathogens showed no elevations that would suggest a recent infection. Citing articles via Web of Science Sign in to download free article PDFs Sign in to access encephaligis subscriptions Sign in to your personal account. This review was undertaken to systematically assess any available randomised controlled data on acute immunomodulatory therapies in Fisher Syndrome or its variants.

A further variant brajnstem associated with upper motor neuron signs and disturbance of consciousness Bickerstaff’s brainstem encephalitis. All 18 patients had ophthalmoplegia and ataxia. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. In this report, we describe a patient who had BBE with a number of remarkable features.