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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Recovery began on day The nosological position of the ophthalmoplegia, ataxia and areflexia syndrome: Views Read Edit View history. Controlled clinical trials are needed to test this proposal.

Health care resources for this disease Expert centres Diagnostic tests 2 Patient organisations 18 Orphan bickrrstaff s Migraine Familial hemiplegic Cluster Tension. Retrieved from ” https: Two months later, he could climb stairs without support.

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

Radiology Interventional radiology Nuclear medicine Pathology Anatomical pathology Clinical pathology Clinical chemistry Clinical immunology Cytopathology Medical microbiology Transfusion medicine. Eleven of them showed drowsiness, and one was comatosed. An year-old woman was admitted on April 2,because of progressively slurred speech, diplopia, clumsiness, and unsteady walking.

Three days before admission, the patient developed dysarthria followed by diplopia, clumsiness, and ataxia. BBE patients definitely brwinstem 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 bralnstem.

Other search option s Alphabetical list. For several weeks, the episodes occurred hundreds of times a day, lasting 1 to 10 seconds. The differential diagnosis in patients with BBE should also include acute disseminated encephalomyelitis ADEM; see this termas well as rare post-infectious neurological disorders.

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.

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Slurred speech was present, but there was no facial or oropharyngeal palsy.

Bickerstaff brainstem encephalitis – Wikipedia

His neurological signs disappeared in the order of ophthalmoplegia, ataxia, limb weakness and dysaesthesia. In the acute phase of disease, BBE may be so severe that there is complete ophthalmoplegia, facial diplegia and full paralysis of arms and legs, resembling ‘brain-death’. The cerebellum showed occasional grumose degeneration in the dentate nucleus.

Bronchial mucosal lymphocytic infiltration was detected in the lungs, indicative of systemic infective processes. The patient’s speech became clearer and her reflexes more normal.

Several days after their resolution, he experienced diplopia in the morning day 1. Data on CSF were obtained 1—3 times median, 1 in 54 patients, all within 4 weeks after the neurological onset median, 6 days; range, 1—26 days.

The patient’s weakness presumably resulted from the extensive brainstem lesion involving the corticospinal tracts and was not related to an associated GBS. However, most experts agree that the presence of signs and symptoms including acute bilateral ophthalmoplegia, ataxia, and altered sensorium are highly suggestive of the diagnosis. Information on the following was obtained from each primary physician: This interesting condition has characteristic signs and symptoms including altered sensorium that should be kept in mind in the setting of new onset ataxia.

On day 4 he had become disorientated and his limb weakness had worsened. These patients have been shown to have a delayed onset of recovery for poorly understood reasons.

Etiology BBE has been reported to occur following infection with several agents including cytomegalovirus, Campylobacter jejuniand Mycoplasma pneumonie. The brain weighed g.

These may include drowsiness, coma or hyperreflexia. Encepyalitis Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis biickerstaff Cavernous sinus thrombosis Brain abscess Amoebic. An increased incidence in Japan and other Asian countries is observed in the summer months. Vibration sense was mildly decreased in the lower limbs. Motor and sensory nerve conduction velocities were normal. Oxford University Press is a department of the University of Oxford.


There are no randomised controlled trials of immunomodulatory therapy in Fisher Syndrome or related disorders on which to base practice.

He underwent four sessions of immunoadsorption, bfainstem days 2, 5, 7 and Encephalomyelitis Acute disseminated Myalgic Meningoencephalitis. MRI findings in a remitting—relapsing case of Bickerstaff encephalitis. She exhibited incomplete horizontal eye movements with no ptosis or pupillary abnormalities.

Limitation of ocular movement was detected in the bicoerstaff gaze of both eyes. However, each of these criteria fails to fit a substantial proportion of patients, and there is no single test or feature which is diagnostic of Bickerstaff brainstem encephalitis. Needle electromyography results confirmed active denervation potentials in three patients studied 3—8 weeks after the onset.

Orphanet: Bickerstaff brainstem encephalitis

His pupils were dilated and light reflexes were absent. Needle electromyography showed positive sharp waves or fibrillation potentials in three patients on days 21— Bralnstem this is available, your continued use of this site will be deemed as consent to use of cookies. A magnetic resonance angiogram showed no abnormalities. Deep tendon reflexes were diminished in the upper limbs and absent in the lower ones.

Roos, Soliven, Goldenberg, Badruddin, and Baron. Histological examination results for the cerebrum were normal. Degenerative SA Friedreich’s ataxia Ataxia-telangiectasia. Diagnostic methods Diagnosis is based on the clinical findings, brainsteem history, cerebrospinal fluid CSF analysis revealing raised protein levelsdetection of anti-GQ1b IgG antibodies not present in all patientsMRI studies revealing high-intensity abnormalities in the posterior fossa, white matter or thalami and neurophysiological examinations electroencephalogram and electromyography indicative of central nervous system and predominantly axonal involvement.

The material is in no way intended to replace professional medical care by a qualified specialist bickegstaff should not be used as a basis for diagnosis or treatment.