Pdf brainstem encephalitis be is an uncommon condition. This condition has been named bickerstaffs brainstem encephalitis bbe. This article cites 2 articles, 0 of which you can access for free at. All of these variants are associated with antigq1b igg antibodies. Bickerstaffs brainstem encephalitis, miller fisher. Frequent episodes of transient brainstem dysfunction occurred in our patient during recovery, possibly due to ephaptic transmission. This condition has been named bickerstaffs brainstem ence phalitis bbe.
In one large series, csf protein was raised in 49% of patients on day 1 and 88% after 2 weeks. Summary bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. Bickerstaffs encephalitis be is an acute postinfectious demyelinating disease with albuminocytological dissociation. Counter to previous studies with bbe which indicate weakness due to guillainbarre. A 48yearold woman was hospitalized due to blurred vision and unsteady gait lasting for 9 days, and numbness of the limbs lasting for 6 days. A chronic form has rarely been described previously. It is similar to miller fisher syndrome, a variant of guillainbarre syndrome. Intravenous immunoglobulin ivig and plasma exchange are often used as treatments in this patient group. Bickerstaffs brainstem encephalitis bbe is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or babinskis sign bickerstaff, 1957. Bickerstaffs brainstem encephalitis bbe is characterized by acute ophthalmoplegia, ataxia and impaired consciousness. This disease is notifiable in the uk see noids article for more details. Pdf a pediatric case of bickerstaffs brainstem encephalitis.
We report results of the autopsy of a patient for whom overlapping bbe and gbs was diagnosed clinically. Serum antigq1b antibody in the acute phase has been detected in many bbe cases. Bickerstaff brainstem encephalitis bbe and millerfisher syndrome mfs might be a pedigree disease. Backgroundbickerstaff brainstem encephalitis bbe is a rare inflammatory, demyelinating disease that generally has a good prognosis. The authors proposed that bickerstaffs encephalitis and miller fisher syndrome represent a single autoimmune disease with a wide range. An elderly patient with bickerstaff brainstem encephalitis and. Extensive vasogenic edema in bickerstaff brainstem encephalitis. Bickerstaff encephalitis radiology reference article. Bickerstaffs brainstem encephalitis bbe is a very rare disease of the central nervous system. Bickerstaff migraine definition of bickerstaff migraine. A pediatric case of bickerstaffs brainstem encephalitis ncbi. Treatment for fisher syndrome, bickerstaffs brainstem. The clinical details of 18 cases of brainstem encephalitis are presented.
Bickerstaffs brainstem encephalitis bbe is a rare condition that must be suspected in patients presenting with ataxia, ophthalmoplegia and central nervous system involvement, which may take the form of pseudobulbar affect, decreased consciousness andor pyramidal signs. Bickerstaffs brainstem encephalitis and fisher syndrome. This may not be the complete list of references from this article. Bickerstaffs brainstem encephalitis in children a case series from. Bickerstaffs brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute. Bickerstaffs brainstem encephalitis is a rare syndrome defined by the triad of ophthalmoplegia, ataxia and decreased consciousness. Bickerstaffs brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. An antigq1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders. Its been almost six months since jy was admitted to the hospital, 102315. Disease bioinformatics bickerstaffs brainstem encephalitis is a rare inflammatory disorder of the central nervous system.
Encephalitis is a rare manifestation of adenovirus infection. Disease localized to the brainstem was seen in 19 patients. Causes of bickerstaffs brainstem encephalitis bbe including triggers, hidden medical causes of bickerstaffs brainstem encephalitis bbe, risk factors, and what causes bickerstaffs brainstem encephalitis bbe. An inservice on bickerstaffs brainstem encephalitis. There is no report on bickerstaffs brainstem encephalitis bbe patients in china. Always seek professional medical advice about any treatment or change in treatment plans.
Professional reference articles are designed for health professionals to use. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support. Neuropsychological evaluation showed slowing, memory and executive function. Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by edwin bickerstaff in 1951. He was initially diagnosed clinically with miller fisher syndrome mfs but later developed limb weakness consistent with guillainbarre syndrome gbs and subsequently reduced consciousness consistent with bickerstaffs brainstem encephalitis bbe. This is the first case report of this syndrome after s. A 44yearold man was hospitalized for drowsiness, cognitive complaint limb weakness, ataxia and sensory disturbance after diarrhea.
The cerebellum was moderately depleted of purkinje cells and there were necrotic foci in the cerebral cortices. Pdf bickerstaff reported eight patients who, in addition to acute. Posterior reversible encephalopathy syndrome in a patient. An atypical bickerstaffs brainstem encephalitis with involvement of. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some. Treatment list for bickerstaffs brainstem encephalitis bbe the list of treatments mentioned in various sources for bickerstaffs brainstem encephalitis bbe includes the following list. We report on one patient with bickerstaffs brainstem encephalitis bbe and associated flaccid weakness. Conclusions bickerstaff brainstem encephalitis is a potentially reversible syndrome that needs early diagnosis facilitated by magnetic resonance imaging and prompt aggressive and supportive treatment. In the 1950s, bickerstaff and fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia.
In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in. Paediatric brainstem encephalitis associated with glial and neuronal. Treatments for bickerstaffs brainstem encephalitis bbe. The difference is that patients with bickerstaffs brainstem encephalitis have impaired consciousness, whereas patients with miller.
The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. It may also affect the peripheral nervous system, and has features in common with both miller fisher syndrome and guillainbarre syndrome. Relapsingremitting severe bickerstaffs brainstem encephalitis. A 56yearold man presented with a 3day history of progressive tingling of the hands, unsteadiness, and diplopia. High apparent diffusion coefficient values imply a vasogenic rather than cytotoxic edema as the cause of the hyperintense signal changes on t2weighted images. Three young patients with bickerstaffs brainstem encephalitis bbe are reported.
We report a patient with mfs, bbe, and axonal gbs overlap in whom serial electrophysiological studies showed persistent motor conduction. Whether bickerstaffs brainstem encephalitis bbe is a distinct disease or a subtype of fisher syndrome fs is unclear as there have been no clinical studies with sufficiently large numbers of patients with fs or bbe. In order to diagnose bickerstaffs brainstem encephalitis, ataxia and ophthalmoplegia must be present. Pdf bickerstaffs brainstem encephalitis and fisher. Some weeks following an upper tract infection, the children after a short period of recovery, showed acute onset of symmetric weakness of the lower limbs with difficulty in standing by and walking. Most of the patients generally have a good outcome. Bickerstaffs brainstem encephalitis bbe is an uncommon nervous system disorder characterized by clinical signs and symptoms. Antigq1b antibody status, magnetic resonance imaging, and the nosology of bickerstaffs brainstem encephalitis. G led to the diagnosis of bickerstaff brainstem encephalitis. It is similar to miller fisher syndrome, a variant of guillainbarre syndrome, in that they share features such as ophthalmoplegia and ataxia. Miller fisher syndrome, bickerstaff brainstem encephalitis. Our aim was to clarify the nosological relationship. An elderly patient with bickerstaff brainstem encephalitis. Paratyphi a infection, and it is the second case of bickerstaffs brainstem encephalitis complicating enteric fever reported in the literature.
Background bickerstaff brainstem encephalitis bbe is a rare inflammatory, demyelinating disease that generally has a good prognosis objective to describe the course of a patient with severe bbe and multiple medical complications design case report setting academic medical center patient an 81yearold woman with bbe who fully recovered. Bickerstaffs brainstem encephalitis bbe in childhood. They are written by uk doctors and based on research evidence, uk and european guidelines. The authors report serial mri of a patient with bickerstaffs brainstem encephalitis bbe, disclosing caudal migration of an initial upper midbrain lesion. Anti gq1b antibody syndrome basal ganglia and brainstem encephalitis, optic neuritis, and radiculomyelitis in epsteinbarr virus infection. This case also demonstrated, for the first time, a positive antigq1b igg response in a patient with bickerstaffs brainstem encephalitis and. Pdf bickerstaffs brainstem encephalitis and fisher syndrome.
Herein, we report a rare case that fits the diagnoses of both mfs and bbe. Bickerstaff migraine basilar migraine a type of classical migraine often associated with the menstrual cycle that first appears in. Miller fisher syndrome mfs and bickerstaffs brainstem encephalitis bbe share some clinical features and a common immunological profile characterized by antigq1b antibodies. Other patients may have mild to severe residual findings. Treatment for fisher syndrome, bickerstaffs brainstem encephalitis. Bickerstaff brainstem encephalitis and fisher syndrome. Bickerstaffs brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute ophthalmoplegia, ataxia, and altered sensorium. Treatment for fisher syndrome, bickerstaffs brain stem. A pediatric case of bickerstaffs brainstem encephalitis. We sought to characterize clinical presentations, etiologies, response to treatment, and predictors of outcome.
You may find one of our health articles more useful. Antigq1b antibody status, magnetic resonance imaging, and. A further variant is associated with upper motor neuron signs and disturbance of consciousness bickerstaffs brainstem encephalitis. Fulltext pdf bickerstaff encephalitis and atypical features bickerstaffs papers revisited. One patient had gross flaccid weakness in the four limbs. An overlapping case of miller fisher syndrome, bickerstaff. Bickerstaffs encephalitis and the miller fisher syndrome. Treatment for fisher syndrome, bickerstaffs brain stem encephalitis and related disorders. Fisher and bickerstaff, in the 1950s, reported some cases with distinctive features of ophthalmoplegia and ataxia. Bickerstaffs brainstem encephalitis n14 and clinically isolated syndrome.
Similar to bickerstaffs brainstem encephalitis, patients typically present with differing degrees of encephalopathy, ocular dysfunction and ataxia. An immunologic abnormality common to bickerstaffs brainstem encephalitis and fishers syndrome. A case of bickerstaffs brainstem encephalitis in childhood khalid hama salih submitted 3rd december 2014. Review bickerstaff brainstem encephalitis and fisher. Brainstem encephalitis be is an uncommon condition. A case of overlapping bickerstaffs brainstem encephalitis. Vasogenic edema in bickerstaffs brainstem encephalitis. Brainstem encephalitis and the syndrome of miller fisher.
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