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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Only comments written in English can be processed. The diagnosis of BBE is largely based on clinical features, though additional studies may aid in making an accurate diagnosis. These patients have been shown to have a delayed onset of recovery for poorly understood reasons. MRI findings in a remitting—relapsing case of Bickerstaff encephalitis. Laboratory studies of the identifiable causes of disorientation analysis of blood gas, encephaoitis sugar, sodium, creatinine, thiamin, renin activity and ammonia were all normal.

The aetiology of this syndrome was speculated to be similar to that of GBS because of evidence of prodromal upper respiratory infection, areflexia and CSF albuminocytological dissociation.

All 18 patients had ophthalmoplegia and ataxia.

Orphanet: Bickerstaff brainstem encephalitis

Because of frequent spontaneous recovery and rare occurrence, today there is a lack of generalized consensus on the role of specific treatments for BBE. Our bicerstaff was undertaken to clarify the clinical, electrophysiological, neuroimaging and immunological features of a large number of patients with BBE.

This page was last edited on 17 Marchat Table 4 shows abnormal MRI and neurophysiological findings.

Serological evidence of recent C. 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.


Light reflexes were prompt. An electroencephalogram showed marked generalized slowing in the theta range, suggestive of an encephalopathy. Slurred speech was present, but there was no facial or oropharyngeal palsy. Neurological examination revealed mild bilateral brainste, limitation of horizontal gaze and incomplete convergence.

Brain Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic. One month before admission, the patient had developed a severe nonproductive cough that was diagnosed as interstitial pneumonitis and treated with moxifloxacin hydrochloride.

Bickerstaff brainstem encephalitis – Wikipedia

Nerve conduction studies may show an axonal polyneuropathy. Bickerstaff reported eight patients who, in addition to acute bickersraff and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. Pathological reflexes were lacking.

The external ophthalmoplegia is progressive within 4 weeks of onset and relatively symmetrical. At about the brainstsm time, C. Electrodiagnostic study results suggested peripheal motor axonal degeneration. Therefore, patients are generally classified as having BBE rather than MFS if there is evidence of corticospinal tract abnormalities, a disturbed state of consciousness, or a significant brainstem lesion on MRI or at autopsy.

Bickerstaff’s brainstem encephalitis BBE is a rare post-infectious neurological disease characterized by the association of external ophthalmoplegia, ataxia, lower limb arreflexia, extensor plantar response and disturbance of consciousness drowsiness, stupor or coma. Disease definition Bickerstaff’s brainstem encephalitis BBE is a rare post-infectious neurological disease characterized by the association of external ophthalmoplegia, ataxia, lower limb arreflexia, extensor plantar response and disturbance of consciousness drowsiness, stupor or coma.

Recovery began on day Detailed information Professionals Clinical practice guidelines Deutsch The patient’s weakness presumably resulted from the extensive brainstem lesion involving the corticospinal tracts and was not related to an associated GBS. The association of BBE with Anti-Gq1b antibody syndrome is supported by infection being the mutual precipitant for a subsequent immune-mediated reaction. Several days after their resolution, he experienced diplopia in the morning day 1.


Five years later, Fisher described three cases of ophthalmoplegia, ataxia and areflexia. 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 MRI detected no abnormalities in the brainstem.

The brain weighed g. On day 7, auditory evoked brainstem responses showed the absence of waves II—V on both sides Fig. Foci can be seen in the brainstem, which is expected, however thalamic and basal ganglia lesions have been documented as well.

Bickerstaff brainstem encephalitis

BBE patients definitely have 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 lesions. Views Read Edit View history.

He underwent four sessions of immunoadsorption, on days 2, 5, 7 and Until this is available, your continued use of this site will be deemed as consent to use of cookies. The next day there was worsened diplopia and ataxic gait.

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

There was no weakness. Our website uses cookies to enhance your experience. A second MRI showed extension of the brainstem involvement without new lesions.