Bickerstaff's Brainstem Encephalitis

Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. This condition has been named Bickerstaff's brainstem encephalitis (BBE). One patient had gross flaccid weakness in the four limbs. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. To clarify its clinical features, we reviewed detailed clinical profiles and laboratory findings for 62 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. Ninety-two per cent of the patients involved had had an antecedent illness. Besides ophthalmoplegia and ataxia, disturbance of consciousness was frequent (74%), and facial diplegia (45%), Babinski's sign (40%) and pupillary abnormality and bulbar palsy (34%) were present. Almost all the patients had a monophasic remitting course and generally a good outcome. Serum anti-GQ1b IgG antibody was positive in 66%, and MRI showed brain abnormality in 30% of the patients. Another striking feature was the association with flaccid symmetrical tetraparesis, seen in 60% of the patients. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: there was perivascular lymphocytic infiltration with oedema and glial nodules. Electrodiagnostic study results suggested peripheral motor axonal degeneration. Limb weakness in the BBE cases studied was considered the result of overlap with the axonal subtype of Guillain-Barre syndrome. These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory features of BBE. A considerable number of BBE patients have associated axonal Guillain-Barre syndrome, indicative that the two disorders are closely related and form a continuous spectrum.

Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barré syndrome.

Despite the advances of the recent decades in scientific methods of investigation, conditions are not infrequently seen, particularly in neurological practice, which fall outside the well-recognized syndromes and in which it is possible to hazard only a guess at the aetiological and pathological processes involved. If the patient recovers the exact explanation may never be certain, but one experience of such a condition may enable it to be recognized again, and, even in the face of ignorance about its nature, some idea of its likely course and prognosis may be formed. This was at one time the only method available to physicians, and it is not surprising that occasionally quite different morbid processes were classified under the same clinical heading. It is possible that in classing together the three cases recorded below, all of which were seen in this unit within eighteen months, one may be guilty of the same error, but their clinical features and course were so remarkably similar that it seemed highly probable that the same or a closely related pathological process was involved. The picture they presented was striking, and

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Mesencephalitis and Rhombencephalitis

Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum : clinical analysis of 581 cases

Background
Fisher syndrome is one of the regional variants of Guillain-Barre syndrome, characterised by impairment of eye movements (ophthalmoplegia), incoordination (ataxia) and loss of tendon reflexes (areflexia). It can occur in more limited forms, and may overlap with Guillain-Barre syndrome. A further variant is associated with upper motor neuron signs and disturbance of consciousness (Bickerstaff's brainstem encephalitis). All of these variants are associated with anti-GQ1b IgG antibodies. Intravenous immunoglobulin (IVIg) and plasma exchange are often used as treatments in this patient group. This review was undertaken to systematically assess any available randomised controlled data on acute immunomodulatory therapies in Fisher Syndrome or its variants.

Objectives
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.

Search methods
We searched the Cochrane Neuromuscular Disease Trials Specialized Register (February 2009), MEDLINE (January 1966 to February 2009), EMBASE (January 1980 to February 2009), CINAHL (January 1982 to February 2009) and LILACS (January 1982 to February 2009) for randomised controlled trials and quasi-randomised trials.

Selection criteria
All randomised and quasi-randomised controlled clinical trials (in which allocation was not random but was intended to be unbiased, e.g. alternate allocation, and non-randomised controlled studies were to have been selected. Since no such clinical trials were discovered, all retrospective case series containing five or more patients were assessed and summarised in the discussion section.

Data collection and analysis
All studies of Fisher Syndrome and its clinical variants were scrutinised for data on patients treated with any form of acute immunotherapy. Information on the outcome was then collated and summarised.

Main results
We found no randomised or non-randomised prospective controlled trials of immunotherapy in Fisher Syndrome or related disorders. We summarised the results of retrospective series containing five or more patients in the discussion section.

Authors' conclusions
There are no randomised controlled trials of immunomodulatory therapy in Fisher Syndrome or related disorders on which to base practice.

Treatment for Fisher syndrome, Bickerstaff's brainstem encephalitis and related disorders

Bickerstaff brainstem encephalitis is a clinical syndrome of ophthalmoplegia, cerebellar ataxia, and central nervous system signs and is associated with the presence of anti-GQ1b antibodies. There is a clinical continuum between Bickerstaff brainstem encephalitis and Miller Fisher syndrome. We describe the case of an 11-year-old boy with encephalopathy, external ophthalmoplegia, brainstem signs, and ataxia with raised titers of anti-GQ1b antibodies. He presented following a respiratory illness and had laboratory evidence of recent infection with Mycoplasma pneumoniae. M pneumoniae infection has been associated with both Bickerstaff brainstem encephalitis and Miller Fisher syndrome. This is only the second case in the literature of Bickerstaff brainstem encephalitis with raised titers of anti-GQ1b antibodies described in association with M pneumoniae infection. The patient responded to intravenous immunoglobulin administration.

Bickerstaff brainstem encephalitis associated with Mycoplasma pneumoniae infection.

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Bickerstaff's Brainstem Encephalitis

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References

Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barré syndrome.

Mesencephalitis and Rhombencephalitis

Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum : clinical analysis of 581 cases

Treatment for Fisher syndrome, Bickerstaff's brainstem encephalitis and related disorders

Bickerstaff brainstem encephalitis associated with Mycoplasma pneumoniae infection.