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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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In this article we review some of the key features of BBE including pathophysiology, epidemiology, presentation and investigations, differential diagnosis, and management and treatment.

For several weeks, the episodes occurred hundreds of times a day, lasting 1 to 10 seconds.

Bickerstaff brainstem encephalitis

Abstract Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. On day 7, auditory evoked brainstem responses showed the absence of waves II—V on both sides Fig.

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Episodes of sensory disturbance are not encephlaitis in demyelinating processes such as multiple sclerosis. The spectrum of IgG GQ1b syndrome: Diagnostic methods Diagnosis is based on the clinical findings, patient 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 bainstem and predominantly axonal involvement.

These patients have been shown to have a delayed onset of recovery for poorly understood reasons. The respiratory problems initially improved, but then the cough significantly worsened before the onset of neurological problems. These may include drowsiness, coma or hyperreflexia.

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Magnetic resonance imaging showed a decrease in the abnormal signal in the brainstem with no evidence of recent stroke FigureD and E. J Neurol Neurosurg Psychiatry.

An electroencephalogram showed marked generalized slowing in the theta range, suggestive of an encephalopathy. Close mobile search navigation Article navigation. Our present day resources bear no clinical guidelines that describe specific diagnostic criteria for BBE. According to our BBE diagnostic criteria, the condition was diagnosed for 62 of encepbalitis 98 patients.

Formal tests of limb coordination showed decomposition and dysmetria. Of special interest was the development of very brief transient episodes of brainstem dysfunction that occurred hundreds of times a day during the recovery phase. Slurred speech was present, but there was no facial or oropharyngeal palsy. Clinical features of BBE can at times overlap with GBS, with the characteristic triad of co-existent symmetrical limb weakness and flaccidity.

These cases with severe brainstem involvement should be diagnosed as having BBE. Limitation of ocular movement was detected in the lateral gaze of both eyes.

Bickerstaff brainstem encephalitis – Wikipedia

Four of the patients had been admitted to our hospital and the others were referred to our neuroimmunological laboratory for serum antiganglioside antibody testing from hospitals throughout Japan between December and February All randomised and quasi-randomised controlled clinical trials in which allocation was not random but was intended to be unbiased, e.

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

A further variant is associated with upper motor neuron signs and disturbance of consciousness Bickerstaff’s brainstem encephalitis. Our understanding of BBE has greatly evolved with the recognition of the role of Anti-Gq1b antibodies in its pathogenesis. Five years later, Fisher described three cases of ophthalmoplegia, ataxia and areflexia.

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Evans, MD and Christina L. Degenerative SA Friedreich’s ataxia Ataxia-telangiectasia. At about the same time, C. Latest Most Read Most Cited Nitroglycerine triggers triptan-responsive cranial allodynia and trigeminal neuronal hypersensitivity.

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

Electrodiagnostic study results suggested peripheal motor axonal degeneration. A recent Cochrane review was not able to give any specific recommendations for the treatment of both BBE and MFS because of an apparent lack of randomized trials evaluating treatment in such clinical settings.

Other patients may have mild to severe residual findings.

Health care resources for this disease Expert centres Diagnostic tests 2 Patient organisations 18 Orphan drug s Ataxic gait disappeared, and on day 35 he could stand on one foot. Encephalutis condition was named Fisher syndrome FS. For Bickerstaff’s migraine, see basilar migraine.

Clinical description Patients usually present with onset of diplopia and gait disturbance following upper respiratory or gastrointestinal tract infections. Healthy Cognitive Aging There are evidence-based tools for staying sharp throughout the lifespan.

B A glial nodule composed of microglia braiinstem the medulla oblongata. There was slight movement of the eyeballs in downward gaze. Bickerstaff considered these changes to be caused by cerebral oedema secondary to viral infection or to be bickerstxff result of hypersensitivity to infection.

Motor nerve conduction velocities were not reduced in the median, ulnar and tibial nerves, whereas CMAP amplitudes were decreased markedly Table 2.

Multimodal imaging in familial FTLD: