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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Results of the motor examination were unremarkable except for a questionable right Babinski sign and unsteady gait. Table 4 shows abnormal MRI and neurophysiological findings.

Bickerstaff brainstem encephalitis

Bronchial mucosal lymphocytic infiltration was detected in the lungs, indicative of systemic infective processes. In addition to Patient 2 described in the case reports, two patients died, one of sudden cardiopulmonary arrest and the other of haemorrhagic infarction, a complication in the brainstm cerebrum caused by candida meningitis.

Blood study results showed no elevation of antibodies to neurotropic viruses, Brucellaor Mycobacterium tuberculosis.

Detailed information Professionals Clinical practice guidelines Deutsch This condition has been named Bickerstaff’s brainstem encephalitis BBE. Motor and sensory nerve conduction velocities were normal. These findings confirm that BBE constitutes a clinical entity and provide encephalitos clinical and laboratory features of BBE. On day 7, auditory evoked brainstem responses showed the bicierstaff of waves II—V on both sides Fig.

This suggests that elements of the autoimmune mechanism are common to both, and that they are not distinct but are closely related conditions.


Controlled clinical trials are needed to test this proposal. Anti-Gq1b antibody is one that interacts with the peripheral nerve ganglioside, Gq1b. All 18 patients had ophthalmoplegia and bicjerstaff.

Additional treatment with intravenous immunoglobulin and plasmapheresis was not used because of concerns about our patient’s renal and hemodynamic status. In the 37 patients who had BBE with limb weakness, muscle weakness was symmetrical and flaccid.

Orphanet: Bickerstaff brainstem encephalitis

Other search option s Alphabetical list. Because of frequent spontaneous recovery and rare occurrence, today there is a lack of generalized consensus on the role of specific treatments for BBE. The nosological position of the ophthalmoplegia, ataxia and areflexia syndrome: Electrodiagnostic study results suggested peripheal motor axonal degeneration. BBE patients definitely have CNS involvement that is responsible for disturbance of consciousness, extensor plantar responses and hemisensory loss, but not all the features of BBE, including ophthalmoplegia and ataxia, are necessarily explained by central lesions.

Ataxia most commonly presents with truncal and limb involvement, however solitary involvement can be seen as well.

When seen by one of us R. One patient had gross flaccid weakness in the four limbs. Healthy Cognitive Aging There are evidence-based tools for staying sharp throughout the lifespan.

A further variant is associated with upper motor neuron signs and disturbance of consciousness Bickerstaff’s brainstem encephalitis.

Bickerstaff brainstem encephalitis – Wikipedia

A tentative brajnstem of BBE was made, and the patient was transferred to the neurological intensive care unit. Get free access to newly published articles. Effective therapy for BBE has yet to be established. On admission, the patient was afebrile with a mildly disturbed mental state and dysarthria.

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Brainsyem should be noted not all cases present classically with this triad of features, and absence of one does not rule out the diagnosis. There is a lack of similar large-scale BBE epidemiological studies in Japan and in the rest of the world.

Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. The respiratory problems initially improved, but then the cough significantly worsened before the onset of neurological problems. One of the present authors M. Brain herniation Reye’s Hepatic encephalopathy Toxic encephalopathy Hashimoto’s encephalopathy. By the evening he could not swallow.

More epidemiological studies of the incidence and prevalence of BBE should be performed in Western nations.

Flaccid symmetrical tetraparesis may bkckerstaff be observed in over half of the patients, together with deep or superficial sensory impairment, facial weakness, bulbar palsy, internal ophthalmoplegia, blepharoptosis and nystagmus.

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