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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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Light reflexes were prompt. The episodes of brainstem dysfunction in our patient resembled similar events reported by Andermann et al 10 and Espir et al. There is also a need for randomized controlled trials to evaluate if certain therapeutic agents or procedures may hasten recovery for patients with BBE.

Privacy Policy Terms of Use. EEG findings that can be seen include slow-wave activity encephalitiw the to range, consistent with central nervous system involvement.

Detailed clinical and laboratory features of BBE were documented in a large number of patients. The brainstem had perivascular lymphocytic infiltration with perivascular oedema Fig. Nerve conduction studies may show an axonal emcephalitis. Retrieved from ” https: A recent Cochrane review was not able to give any specific recommendations for the treatment of both BBE and MFS because of an apparent lack of randomized trials evaluating treatment in such clinical settings.

Get free access to newly published articles Create a personal account or sign in to: Bickerstaff’s brainstem bickersgaff 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.

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. Intravenous immunoglobulin IVIg and plasma exchange are often used as treatments in this patient group.


Anti-Gq1b seropositivity has been witnessed in up to 83 percent of MFS cases 9 whereas only about 66 percent of patients with BBE have demonstrated anti-Gq1b seropositivity.

A few days later, she noticed very frequent, stereotypic episodes lasting 1 to 10 seconds and occurring throughout the day; these episodes consisted of transient diplopia accompanied by dysarthria if talking and gait imbalance if walking. An autopsy was performed. 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.

Comparisons between groups of clinical features were made by post hoc. In this report, we describe a patient who had BBE with a number of remarkable features.

Presumably, because of its rarity, there has been no study of a bickedstaff number of BBE patients.

Bickerstaff brainstem encephalitis – Wikipedia

B A glial nodule composed of microglia in the medulla oblongata. Although the exact pathological mechanism is not fully understood, BBE is associated with the presence of the antiganglioside antibody, anti-GQ1b. Controlled clinical trials are needed to test this proposal. Surgical operation for Bell’s palsy idiopathic facial paralysis Interventions for preventing nerve damage caused by cisplatin and other tumour-inhibiting platinum drugs Immune treatments for peripheral neuropathy caused by an IgM paraprotein antibody, which may bind to MAG, a protein on the myelin sheath of nerves Corticosteroids for treating nerve damage in leprosy Intravenous immunoglobulin treatment improves strength significantly more often than placebo.

Interestingly, axons that are encephalitix demyelinated develop spontaneous activity with a lowered threshold for firing bickerstsff week or more after the insult, possibly explaining the onset of these episodes during the patient’s recovery.

Roos, Soliven, Goldenberg, Badruddin, and Baron. We found no randomised or non-randomised prospective controlled trials of immunotherapy in Fisher Syndrome or bickerstaft disorders. In encephalitix 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’. The spectrum of IgG GQ1b syndrome: A chest radiograph showed an increase in interstitial markings and small right-sided effusion.


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

The discovery that a large number of affected patients tested positive for the Anti-Gq1b antibody encephslitis to a greater understanding of BBE. Miller Fisher described cases of ophthalmoplegia and ataxia in a edition of. 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.

There was no significant difference in frequency of positive C. Clinical description Patients usually present with onset of diplopia and gait disturbance following upper respiratory or gastrointestinal tract infections. Abstract Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss.

The nosological position of the ophthalmoplegia, ataxia and areflexia syndrome: The frequency of disturbance of consciousness in patients with BBE with overlapping GBS was almost the same for drowsiness and for stupor, semicoma and coma. Fisher syndrome is an uncommon paralysing illness, usually caused by autoimmune inflammation of nrainstem following an infection.

Although the clinical picture is severe, the disease course is generally monophasic brxinstem complete remission of symptoms within 6 months in over half of the patients. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. The patient had dysmetria and slowed rapid alternating movements in the upper extremities.

Bickerstaff brainstem encephalitis

Between episodes, results of an examination showed minimal dysarthria, full eye movements, no ptosis, a questionable right Babinski sign, slight clumsiness, and a slow gait with a negative Romberg sign. Miller Fisher syndrome is associated with serum antibodies to GQ1b ganglioside.

Active denervation potentials positive sharp waves or fibrillation potentials were present in three, with limb weakness on days 21—