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 .

Author: Akinoll Nak
Country: Morocco
Language: English (Spanish)
Genre: Environment
Published (Last): 11 November 2013
Pages: 200
PDF File Size: 8.55 Mb
ePub File Size: 7.54 Mb
ISBN: 268-2-68444-518-1
Downloads: 50446
Price: Free* [*Free Regsitration Required]
Uploader: Mokree

Frequent episodes of transient brainstem dysfunction occurred in our patient during recovery, possibly due to ephaptic transmission. With these strict criteria, BBE, not FS, was diagnosed for patients with drowsiness, although in Case 2 in the original report by Fisher there was mild drowsiness.

Two months later, he could climb stairs without support. All randomised and quasi-randomised controlled clinical trials in which allocation was not random but was intended to be unbiased, e. Electrophysiological examination on day 9 showed reduced amplitudes of CMAPs with normal motor conduction velocities in the median, ulnar and tibial nerves, brinstem of primary axonal degeneration Table 2. On days 4, 5, 8, 9, 10, 11 and 12, he underwent seven sessions of immunoadsorption.

By the evening he could not swallow.

Orphanet: Bickerstaff brainstem encephalitis

Like some other autoimmune diseasesthe condition usually follows a minor infection, such as a respiratory tract infection or gastroenteritis. Prognosis Although the clinical picture is severe, the disease course is generally monophasic with complete remission of symptoms within 6 months in over half of the patients.

Bickerstaff considered these changes to be caused brainstwm cerebral oedema secondary to viral infection or to be the result of hypersensitivity to infection.


A biickerstaff diagnosis of BBE was made, and the patient was transferred to the neurological intensive care unit. The association of BBE with Anti-Gq1b antibody syndrome is supported by infection being the mutual precipitant for a encehpalitis immune-mediated reaction. Our patient’s episodes exhibited several brainstem abnormalities suggesting mass synchronous firing as a result of ephaptic transmission see Smith and McDonald 12 for a review.

Ataxic gait disappeared, and on day 35 he could stand on one foot.

On April 5, acyclovir administration was discontinued and mechanical ventilation for airway protection and hemodynamic stabilization was started. Vibration sense was mildly decreased in the lower limbs.

Bickerstaff brainstem encephalitis – Wikipedia

Paraffin sections stained with haematoxylin and eosin. MRI on days 6 and 21, with and without enhancement, detected no abnormalities in the brainstem and brain. Kufs or not Kufs: Evans, MD and Christina L. Migraine Familial hemiplegic Cluster Tension. Episodes that involve more complex abnormalities are not common but have been described.

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

Bickerstaff brainstem encephalitis

Study concept and design: Despite improvements in the understanding of BBE, much more work remains to be done. Needle electromyography showed positive sharp waves or fibrillation potentials in three patients on days 21— On April 10, the patient was more responsive and a tetraparesis became apparent.

Autoimmune diseases Neurological disorders Encephalitis. The cerebellum showed diffuse, slight loss of Purkinje cells, but some folia were completely lacking these cells.

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

A ibckerstaff MRI bicmerstaff increased signal on fluid-attenuated inversion recovery image sequences, with gadolinium enhancement in the brainstem Figure. Almost all the patients had a monophasic remitting course and generally a good outcome. Purchase access Subscribe to JN Learning for one year. The spectrum of IgG GQ1b syndrome: On the morning after the sixth day day 1 he had dysaesthesiae, dysarthria and ataxic gait, but he was alert.


BrainVolumeIssue 10, 1 OctoberPages —, https: In order to bickedstaff Bickerstaff brainstem encephalitis, ataxia and ophthalmoplegia must be present. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: Observational studies suggest that Fisher syndrome always and Bickerstaff’s brain stem encephalitis usually recovers completely. He was intubated and placed on a respirator as respiration was apneustic breathing and he was comatosed.

At about the same time, C. He was taken off the mechanical respirator.

One month before admission, the patient had developed a severe nonproductive cough that was diagnosed as interstitial pneumonitis and treated with moxifloxacin hydrochloride. Table 5 shows CSF findings during the first week, the second week, and the third and fourth weeks. Get free access to newly published articles.

Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic. There was also very marked perivascular round cell cuffing, sometimes with numerous macrophages.

Effective management and treatment of BBE and other variants of Anti-Gq1b syndrome requires prompt recognition and diagnosis. Clinical description Patients usually present with onset of diplopia and gait disturbance following upper respiratory or gastrointestinal tract infections.

Information on the following was obtained from each primary physician: