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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Bickefstaff of serological studies on a variety of respiratory pathogens showed no elevations that would suggest a recent infection. MRI findings in a remitting—relapsing case of Bickerstaff encephalitis.

Handbook of clinical neurology, Vol. Almost all the patients had a monophasic remitting course and generally a good outcome. These inflammatory processes probably have a pivotal role in the development of the characteristic phenotypes of BBE.

An increased incidence in Japan and other Asian countries is observed in the summer months. Articles which use infobox templates with no data rows Infobox medical condition new All articles with unsourced statements Articles with unsourced statements encehpalitis April Articles with unsourced statements from February On day 29 there was no extraocular muscles paresis, except for limitation of abduction.

Wendy Terry Publisher wterry bmctoday. An electroencephalogram showed marked generalized slowing in the theta range, suggestive of an encephalopathy.

Bickerstaff brainstem encephalitis

Table 5 shows CSF findings during the first week, the second week, and the third and fourth weeks. Degenerative SA Friedreich’s ataxia Ataxia-telangiectasia.

The episodes increased in frequency so that they occurred up to hundreds of times a day. CSF samples were tested at the various hospitals. Dissecting out migraine complexity through comprehensive analysis of allodynia. Check this box if you bickefstaff to receive a copy of your message. Gynaecology Gynecologic oncology Maternal—fetal medicine Obstetrics Reproductive endocrinology and infertility Urogynecology. The brainstem had perivascular lymphocytic infiltration with perivascular oedema Fig.


B A glial nodule composed of microglia in the medulla oblongata. One of the three patients encephaliis by Fisher in showed mild drowsiness Fisher, On day 7, auditory evoked brainstem responses showed the absence of waves II—V on both sides Fig. These are also diagnostic features of Miller Fisher syndromeand so Bickerstaff’s is only diagnosed if other features are present which exclude Miller Fisher syndrome.

Only comments written in English can be processed. 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.

Miller Fisher syndrome is associated with serum antibodies to GQ1b ganglioside. By day 73, nystagmus had disappeared.

He was taken off the mechanical respirator. Several days after their resolution, he experienced diplopia in the morning day 1. Insomnia Hypersomnia Sleep apnea Obstructive Congenital central hypoventilation syndrome Narcolepsy Cataplexy Kleine—Levin Circadian rhythm sleep disorder Advanced sleep phase disorder Delayed sleep phase disorder Nonhour sleep—wake disorder Jet lag.

Bickerstaff brainstem encephalitis – Wikipedia

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

On April 6, the patient was noted to have bilateral Babinski signs with unsustained ankle clonus; she became more difficult to arouse, with extensor posturing on the right side.


He underwent four sessions of immunoadsorption, on days 2, 5, 7 and Similarly, raised CSF protein levels and pleocytosis are frequent but non-specific. About Contact Subscriptions Videos Privacy.

The age of the patient was not at all typical for patients with BBE and contrasts with previously presented cases. Despite improvements in the understanding of BBE, much more work remains to be done. Electrophysiological examination on day 9 showed reduced amplitudes of CMAPs with normal motor conduction velocities in the median, ulnar and tibial nerves, indicative bickerrstaff primary axonal degeneration Table 2.

Poliomyelitis Demyelinating disease Transverse myelitis Tropical spastic paraparesis Epidural abscess. The diagnosis was made quickly, partly through the use of magnetic resonance imaging MRI studies.

Orphanet: Bickerstaff brainstem encephalitis

Active denervation potentials positive sharp waves or fibrillation potentials were present in three, with limb weakness on days 21— Anti- Gq1b antibody seropositivity has also suggested that BBE is most likely part of a spectrum of diseases under the umbrella known as Anti-Gq1b antibody syndrome. However, each of these criteria fails to fit a substantial brainatem of bickerstagf, and there is no single test or feature which is diagnostic of Bickerstaff brainstem encephalitis.

There is a lack of similar large-scale BBE epidemiological studies in Japan and in the rest of the world.

Detailed clinical and laboratory features of BBE were documented in a large number of patients.