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 .
|Published (Last):||16 February 2012|
|PDF File Size:||1.39 Mb|
|ePub File Size:||16.27 Mb|
|Price:||Free* [*Free Regsitration Required]|
Almost all the patients had a monophasic remitting course and generally a good outcome.
Bickerstaff brainstem encephalitis – Wikipedia
In the nerve conduction studies, most patients with BBE without limb weakness were normal, whereas half of those who had BBE and overlapping GBS showed indications of motor nerve dysfunction, predominantly axonal dysfunction. Foci can be seen in the brainstem, which is expected, however thalamic and basal ganglia lesions have been documented as well.
Neurological examination revealed mild bilateral blepharoptosis, limitation of horizontal gaze and incomplete convergence. The dramatic MRI abnormalities in our patient assisted us in making an early diagnosis, which allowed prompt treatment of BBE. MRI on days 6 and 21, with and without enhancement, detected no abnormalities in the brainstem and brain.
Results of the sensory examination were normal.
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 system and predominantly axonal involvement. Common seropositivity between these two conditions highlights their pathophysiological and clinical similarities.
The patient’s speech became clearer and her reflexes more normal. Plantar responses were indifferent. Formal tests of limb coordination brainsteem decomposition and dysmetria. Vibration sense was mildly decreased in the lower limbs. D ICD – Radiology Interventional radiology Nuclear medicine Pathology Anatomical pathology Clinical pathology Clinical chemistry Clinical immunology Cytopathology Medical microbiology Transfusion medicine.
Over the next few weeks, the episodes disappeared and the patient returned to her normal baseline condition.
One of the present authors M. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. He considered that six of the 18 cases were typical FS, and the other 12 represented obscure brainstem lesions without peripheral polyneuropathy. However, each of these criteria fails to fit a substantial encephalitos of patients, and there is no single test or feature which is diagnostic of Bickerstaff brainstem encephalitis.
There was no significant difference in the clinical profiles of the two subgroups. Views Read Edit View history. Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system first described by Edwin Bickerstaff in Sign in to make a comment Sign encephalitos to your personal account. Electrodiagnostic study results suggested peripheal motor axonal degeneration. Detailed information Professionals Clinical practice guidelines Deutsch These cases with severe brainstem involvement should be diagnosed as having BBE.
Bickerstaff brainstem encephalitis
On day 7, he was drowsy but easily aroused by stimulation. Receive exclusive offers and updates from Oxford Academic. There was proximal upper limb weakness, of 4 on the MRC scale. Bickerstaff considered these changes to be caused by cerebral oedema secondary to viral infection or to be the 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.
Results of a second lumbar puncture on April 7 were normal. BrainVolumeIssue 10, 1 OctoberPages —, https: There was also very encephalifis 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. Slurred speech was present, but there was no facial or oropharyngeal palsy. Sign In or Create an Account.
Orphanet: Bickerstaff brainstem encephalitis
Ital J Neurol Sci. There is a lack of similar large-scale BBE epidemiological studies in Japan and in the rest of the world. Electrodiagnostic study results suggested peripheral motor axonal degeneration.