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Encéphalite de Bickerstaff: à propos de deux cas

Service central de radiologie, CHU Ibn Rochd, 1, rue des Hôpitaux, Casablanca, Maroc
Journal de Radiologie (Impact Factor: 0.57). 12/2011; DOI: 10.1016/j.jradio.2010.08.004
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    ABSTRACT: 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-Barré 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-Barré syndrome, indicative that the two disorders are closely related and form a continuous spectrum.
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    ABSTRACT: Bickerstaff brainstem encephalitis (BBE) is a rare inflammatory, demyelinating disease that generally has a good prognosis. To describe the course of a patient with severe BBE and multiple medical complications. Case report. Academic medical center. An 81-year-old woman with BBE who fully recovered. The patient had transient and very frequent episodes of brainstem dysfunction during the recovery phase. Clinical and biochemical evaluation with magnetic resonance imaging. Bickerstaff brainstem encephalitis is a potentially reversible syndrome that needs early diagnosis (facilitated by magnetic resonance imaging) and prompt aggressive and supportive treatment. Frequent episodes of transient brainstem dysfunction occurred in our patient during recovery, possibly due to ephaptic transmission.
    Archives of neurology 07/2008; 65(6):821-4. DOI:10.1001/archneur.65.6.821 · 7.58 Impact Factor
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