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):||23 January 2008|
|PDF File Size:||16.2 Mb|
|ePub File Size:||8.34 Mb|
|Price:||Free* [*Free Regsitration Required]|
Vibration and position senses were intact.
Treatment for Fisher syndrome, Bickerstaff’s brain stem encephalitis and related disorders
The respiratory problems initially improved, but then the cough significantly worsened before the onset of neurological problems.
Bickertaff detected no abnormalities in the brainstem. 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. Sign in to save your search Sign in to your personal account.
BBE patients definitely have CNS involvement that is responsible for disturbance of consciousness, extensor plantar responses and hemisensory loss, brainsttem not all the features of BBE, including ophthalmoplegia and ataxia, are necessarily explained by central lesions. Two months later, he could climb stairs without support. Sign in to make a comment Sign in to your personal account. These encephalitid have been shown to have a delayed onset of recovery for poorly understood reasons.
Report of a case. On the morning after the sixth day day 1 he had dysaesthesiae, dysarthria and ataxic gait, but he was alert. Table 3 shows the clinical profiles of the 62 patients, 37 of whom were men and 25 women, aged 3—91 years median, 39 years. Our straightforward articles give neurologists tools they can immediately put into practice. Limb strength in the arms and legs was 4 on the MRC scale.
The examination on April 11 showed horizontal gaze abnormalities, ptosis, seventh nerve palsies, and increased strength. One patient had gross flaccid weakness in the four limbs. It is therefore possible that a proportion of cases are due to other causes, such as infection or lymphomabut remain undiagnosed.
In this condition, neuronal excitability can spread laterally to adjacent axons that may have an abnormally lowered threshold for firing as a result of demyelination. Deltoid and triceps muscle weakness registered 3 on the MRC scale.
The brainstem had perivascular lymphocytic infiltration with perivascular oedema Fig. For several weeks, the episodes occurred hundreds of times a day, lasting 1 to 10 seconds. The patient was witnessed to have multiple episodes of paroxysmal worsening of slurred speech and ataxia with diplopia.
Bickerstaff brainstem encephalitis
On day brainstemm he remained alert. There are no randomised controlled trials of immunomodulatory therapy in Fisher Syndrome or related disorders on which to base practice. No pulmonary embolism and coronary artery occlusion were present.
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. 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.
In addition to complete ophthalmoplegia, there were mild bifacial palsy, severe oropharyngeal palsy and slightly explosive speech. The association of BBE with Anti-Gq1b antibody syndrome is supported bickerstqff infection being the mutual precipitant for a subsequent immune-mediated reaction.
But in contradistinction to BBE, these patients had ejcephalitis change in sensorium and almost universally these patients were areflexic.
Hassan T, Mumford C. Roos, Soliven, Goldenberg, Bickersatff, and Baron. The episodes of brainstem dysfunction in our patient resembled similar events reported by Andermann et al 10 and Espir et al.
Orphanet: Bickerstaff brainstem encephalitis
The majority of patients achieved spontaneous recovery without the need for treatment despite the alarming initial presentation of BBE.
The discovery that a large number of affected patients tested positive for the Anti-Gq1b antibody led to a greater understanding of BBE. Treatment for Fisher syndrome, Bickerstaff’s brain stem encephalitis and related disorders Fisher syndrome is an uncommon paralysing illness, usually caused by autoimmune inflammation of nerves following an infection. The next day there was worsened diplopia and ataxic gait.
Despite improvements in the understanding of BBE, much more work remains to be done. All randomised and quasi-randomised controlled clinical trials in which allocation was not random but was intended to be unbiased, e. B A glial nodule composed of microglia in the medulla oblongata.