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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Bickerstaff brainstem encephalitis

Although the exact pathological mechanism is not fully understood, BBE is associated with the presence of the antiganglioside antibody, anti-GQ1b. Migraine Familial hemiplegic Cluster Tension. On the basis of radiological three patients and pathological one patient changes in the brainstem and abnormal EEG findings 12 patientsthey insisted that the condition represents a clinical entity distinct from GBS.

Hassan T, Mumford C. 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. The majority of patients achieved spontaneous recovery without the need for treatment despite the alarming initial presentation of BBE. Over the next several days, the patient improved in mental state, ptosis, eye movements, and renal function and was therefore extubated.

We wish to thank Dr K.

Ataxia most commonly presents with truncal and limb involvement, however solitary involvement can be seen as well. The bickerataff position of the ophthalmoplegia, ataxia and areflexia syndrome: However, each of these criteria fails to fit a substantial proportion of patients, and there is no single test or feature which is diagnostic of Bickerstaff brainstem encephalitis.

MRI on days 6 and 21, with and without enhancement, encephalits no abnormalities in the brainstem and brain. One patient had gross flaccid weakness in the four limbs.

Orphanet: Bickerstaff brainstem encephalitis

Recovery from coma began on day 23, and he was fully alert on day Retrieved from ” https: Additional examples of Anti-Gq1b syndrome include acute axonal neuropathy and the pharyngeal-cervical-brachial GBS variant. It should be noted not all cases present classically with this triad of features, and absence of one does not rule out the diagnosis. For Bickerstaff’s migraine, see basilar migraine. The patient had transient and very frequent episodes of brainstem dysfunction during the recovery phase.


In this report, we describe a patient who had BBE with a number of remarkable features. The precise incidence and prevalence of BBE in the United States and other Western nations is currently unknown, which can be attributed to the rarity of the disease and confusion and overlap with other Anti-Gq1b antibody syndromes.

Fisher syndrome is characterised by impairment of eye movements, abnormal co-ordination and loss of tendon reflexes. 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.

In the acute phase of disease, BBE may be so severe that there is complete ophthalmoplegia, facial diplegia and full paralysis of arms and legs, resembling ‘brain-death’. Acute axonal neuropathy is characterized by an acute onset of symmetrical motor weakness, areflexia, and facial and oropharyngeal weakness.

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 On April 5, acyclovir administration was discontinued and mechanical ventilation for encehpalitis protection and hemodynamic stabilization was started. Roos, Goldenberg, and Badruddin. Diagnostic methods 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.

Information encphalitis the outcome was then collated and summarised. An unusual variant of acute idiopathic polyneuritis: Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more. One patient had gross flaccid weakness in the four limbs. Views Read Edit View history.


Bickerstaff brainstem encephalitis – Wikipedia

Results of the motor examination were unremarkable except for a questionable right Babinski sign and unsteady gait. Sign in to save your search Sign in to your personal account. Create a free personal account to download free article PDFs, sign up for alerts, and more.

All 18 patients had ophthalmoplegia and ataxia.

In our case, the presence of Babinski signs, a disturbed state of consciousness, and the dramatic MRI signal abnormalities in the brainstem indicated a diagnosis of BBE. Histological examination results for the cerebrum were normal.

An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: Results of serological studies on a variety of respiratory pathogens showed no elevations that would suggest a recent infection. The findings confirmed that BBE can be defined as a syndrome presenting acute ophthalmoplegia, ataxia and disturbance of consciousness as its major manifestations, in association with certain symptoms and signs indicative of central involvement, and that it constitutes a clinical entity.

In addition to the episodes, the patient had minimal slurring of words when she first began to brainztem and a slight imbalance when she began to walk. Our findings have added information about the clinical features of BBE. Health care resources for this disease Expert bickersaff Diagnostic tests 2 Patient organisations 18 Orphan drug s Treatment for Fisher syndrome, Bickerstaff’s brain stem encephalitis and related disorders Fisher syndrome is an uncommon paralysing bifkerstaff, usually caused by autoimmune inflammation of nerves following an infection.