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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Migraine Familial hemiplegic Cluster Tension. Our present day resources bear no clinical guidelines that describe specific diagnostic criteria for BBE.

Bickerstaff brainstem encephalitis – Wikipedia

About Contact Subscriptions Bickerstxff Privacy. Copyright American Medical Association. Sign in to customize your interests Sign in to your personal account. Diagnostic methods Diagnosis is based on the clinical findings, patient history, cerebrospinal fluid CSF analysis bickertsaff 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.

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Episodes that involve more complex abnormalities are not common but have been described. 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.

Miller Fisher syndrome is associated with serum antibodies to GQ1b ganglioside. Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed encephalitks, extensor plantar responses or hemisensory loss.


Foci can be seen in the brainstem, which is expected, however thalamic and basal ganglia lesions have been documented as well. Electrophysiological examination on day 9 showed reduced amplitudes of CMAPs with normal motor conduction velocities in the median, ulnar and tibial nerves, indicative of bicoerstaff axonal degeneration Table 2.

Over the next few weeks, the episodes disappeared and the patient returned to her normal baseline condition.

Orphanet: Bickerstaff brainstem encephalitis

In this condition, neuronal excitability can spread laterally to adjacent axons that may have an abnormally lowered threshold for firing as a result of demyelination. The episodes of brainstem dysfunction in our patient resembled similar events reported by Andermann et al 10 and Espir et al.

Active denervation potentials positive sharp waves or fibrillation potentials were present in three, with limb weakness on days 21— Yet it should be noted that negative antibody testing does not preclude the diagnosis: All of these variants are associated with anti-GQ1b IgG antibodies.

Acute axonal neuropathy is characterized by an acute onset brxinstem symmetrical motor weakness, areflexia, and facial and oropharyngeal weakness. In the 37 patients who had BBE with limb weakness, muscle weakness was symmetrical and flaccid.

For a diagnosis of BBE, the following diseases must be excludable: One month before admission, the patient had developed a severe nonproductive cough that was diagnosed as interstitial pneumonitis and treated bicjerstaff moxifloxacin hydrochloride. 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.


On day encephalitsi he remained alert. For all other comments, please send your remarks via contact us. These inflammatory processes probably have a pivotal role in the development of the characteristic phenotypes of BBE.

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

The clinical, electrophysiological and immunological findings reported in our study support the original hypothesis of Bickerstaff and Cloakethat BBE is closely related to GBS and that they form a continuous spectrum. Because coma continued, he was given 0. In addition to complete ophthalmoplegia, there were mild bifacial palsy, severe oropharyngeal palsy and slightly explosive speech. We wish to thank Dr K. The median CSF protein concentration after the second week was higher than that during the first week.

Table 5 bickefstaff CSF findings during the first week, the second rbainstem, and the third and fourth weeks. This page was last edited on 17 Marchat MFS usually presents with areflexia, consistent with a peripheral pathology. In addition to the episodes, the patient had minimal slurring of words when she first began to speak and a slight imbalance when she began to walk. Table brainsetm shows abnormal MRI and neurophysiological findings.

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