March 18, 2020   |   by admin

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

These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory features of BBE. Ten days brainsrem resolution, he suffered numbness of the arms and legs, diplopia, dysarthria, drowsiness and unsteady gait day 1.

He began to recover from respiratory failure on day 15, but a tracheostomy was performed because his coma continued. Flaccid symmetrical tetraparesis may also be observed in over half of the patients, together with deep or superficial sensory impairment, facial weakness, bulbar palsy, internal ophthalmoplegia, blepharoptosis and nystagmus.

Yet it should be noted that negative antibody testing does not preclude the diagnosis: Healthy Cognitive Aging There are evidence-based tools for staying sharp throughout the lifespan. On the basis of the preceding criteria, BBE was the rediagnosis for 62 of these 98 patients. 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 bikcerstaff Japan between December and February Deep tendon reflexes were absent, but extensor plantar responses were present.

An autopsy study of a BBE patient clearly showed the presence of brainwtem inflammatory changes in the brainstem: All 18 patients had ophthalmoplegia and ataxia.

Ataxia most commonly presents with truncal and limb involvement, however solitary involvement can be seen as well. EEG findings that can be seen include slow-wave activity in the to range, consistent with central nervous system involvement. 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.


Gynaecology Gynecologic oncology Maternal—fetal medicine Obstetrics Reproductive endocrinology and infertility Urogynecology. He was taken off the mechanical respirator.

MRI findings in a remitting—relapsing case of Bickerstaff encephalitis. In a review of cases of Anti-Gq1b Syndrome, antecedent illness was seen in 94 percent of patients, with upper respiratory tract infection being most common.

Despite the fact that this elderly woman had significant neurological deficits and serious brzinstem problems, she eventually returned to her baseline status, highlighting the importance of aggressive, supportive treatment in what can be a completely reversible process.

Email alerts New issue alert. Autoimmune diseases Neurological disorders Encephalitis. In this condition, neuronal excitability can spread laterally to adjacent axons that may have an bickerstwff lowered threshold for firing as a result of demyelination.

Foci can be seen in the brainstem, which is expected, however thalamic and basal ganglia lesions have been documented as well. Treatment of the 62 patients was as follows: The clinical features and course of the condition, the associated auto-antibodies against relevant antigens, and the response to treatment, all suggest that Bickerstaff brainstem encephalitis is an autoimmune disease. There is also a need for randomized controlled trials to evaluate if certain therapeutic agents or procedures may hasten recovery for patients with BBE.

One patient had gross flaccid weakness in the four limbs. Common seropositivity between these two conditions highlights their pathophysiological and clinical similarities. The condition was named Fisher syndrome FS. Bickerstaff’s brain stem encephalitis shares many clinical features but also includes altered consciousness and signs of central nervous system inflammation.

Latest Most Read Most Cited Nitroglycerine triggers triptan-responsive cranial allodynia and trigeminal neuronal hypersensitivity. On April 6, the patient was noted to have bilateral Babinski signs with unsustained ankle clonus; she became more difficult to arouse, with extensor posturing on the right side. Health care resources for this disease Expert centres Diagnostic tests 2 Patient organisations 18 Orphan drug s Our study was undertaken to clarify the clinical, electrophysiological, neuroimaging and immunological features of a large number of patients with BBE.


This suggests that elements of the autoimmune mechanism are common to both, and that they are not distinct but are closely related conditions. All randomised and quasi-randomised controlled clinical trials in which allocation was not random but was intended to be unbiased, e.

The patient had dysmetria and slowed rapid alternating movements in the upper extremities. On April 10, the patient was more responsive and a tetraparesis became apparent.

The cerebellum showed occasional grumose degeneration in the dentate nucleus. Almost all the patients had a monophasic remitting course and generally a good outcome. 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.

Orphanet: Bickerstaff brainstem encephalitis

The most frequent preceding symptom was upper respiratory infection, and the most frequent initial symptoms were diplopia and gait disturbance. These patients have been shown to have a delayed onset of recovery for poorly understood reasons. Report of a case. Poliomyelitis Demyelinating disease Transverse myelitis Tropical spastic paraparesis Epidural abscess.

Other patients may have mild to severe residual findings. Over the next several days, the patient improved in mental state, ptosis, eye movements, and renal function and was therefore extubated. Limitation of ocular movement was detected in the lateral gaze of both eyes.

Altered sensorium is variable where patients can present with drowsiness, stupor, or in the most severe cases, coma, which can be seen in up to 20 percent of cases.

From Wikipedia, the free encyclopedia. The most supportive laboratory investigation is positive Anti-Gq1b antibodies, especially in the setting of the appropriate clinical context. Focal Generalised Status epilepticus Myoclonic epilepsy. By using this site, you agree to the Terms of Use and Privacy Policy.

Roos, Soliven, Goldenberg, Badruddin, and Baron. Receive exclusive offers and updates from Oxford Academic.