Bickerstaffs brainstem encephalitis, miller fisher syndrome and. Professional reference articles are designed for health professionals to use. In the 1950s, bickerstaff and fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. Neuropsychological evaluation showed slowing, memory and executive function. Brain mri revealed extensive white matter, brainstem, and cerebellar vasogenic edema, without gadolinium enhancement, partially regressive during followup figure, a. In 1951, bickerstaff and cloake described three patients in their original report entitled mesencephalitis and rhomboencephalitis, and in 1957 bickerstaff expanded his series to eight patients and proposed the. Bickerstaffs encephalitis be is an acute postinfectious demyelinating disease with albuminocytological dissociation.
Paraneoplastic brain stem encephalitis can occur as an isolated clinical syndrome or, more often, may be part of a more widespread encephalitis. They are written by uk doctors and based on research evidence, uk and european guidelines. Brain mri revealed extensive white matter, brainstem, and cerebellar vaso. Treatment strategies tried have been immunotherapies such as plasma exchange or intravenous immunoglobulin which are used in. Ten days after respiratory infection, a 32yearold woman presented with headaches, ataxia, and diplopia without encephalopathy. We sought to characterize clinical presentations, etiologies, response to treatment, and predictors of outcome. Chronic brainstem encephalitis with mental symptoms and. Since the first description of a characteristic clinical neuropsychiatric picture in a patient with hashimoto thyroiditis by brain et al.
Bickerstaff encephalitis is a rare immunemediated condition believed to be one of a number of conditions sharing a similar immunological mechanism, and collectively termed antigq1b igg antibody syndrome. Progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks and disturbance of consciousness or hyperreflexia are both required8. The incidence of herpes simplex encephalitis is 1 per 250 000 to 500 000 population per year. Bickerstaffs brainstem encephalitis is a rare syndrome defined by the triad of ophthalmoplegia, ataxia and decreased consciousness. Atypical brainstem encephalitis caused by herpes simplex. Electroencephalography pattern from 20yearold patient with overlapping bickerstaffs brainstem encephalitis, miller fisher syndrome, and guillainbarre syndrome. An overlapping case of miller fisher syndrome, bickerstaff.
Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by edwin bickerstaff in 1951. Because of the apparent similarities in the clinical presentations of bbe and fishers syndrome fs, opinions differ as to whether these conditions are distinct or related. Since then, such a condition has been called bickerstaffs brainstem encephalitis bbe. Brainstem encephalitis is a medical condition that causes swelling and inflammation of the brainstem, the area of the brain that connects to the spinal column. Viruses cause most cases of infectious encephalitis, but bacteria especially intracellular organisms such as rickettsiae, fungi and parasites are also important. A case of bickerstaff brainstem encephalitis and acute. Compared to cases previously reported as brainstem encephalitis, the clinical and pathological findings observed in these cases have rather peculiar characteristics. Backgroud information regarding the epidemiological background of bickerstaff brainstem encephalitis bbe is limited. You may find one of our health articles more useful.
Bickerstaffs brain stem encephalitis shares many clinical features but also includes altered consciousness and signs of central nervous system inflammation. A case of tick borne encephalitis tbe is reported, with simultaneous brain stem, spinal cord, and bilateral thalamic involvement confirmed by magnetic resonance imaging mri. Rhombencephalitis re is a syndrome of multiple causes and multiple outcomes. High apparent diffusion coefficient values imply a vasogenic rather than cytotoxic edema as the cause of the hyperintense signal changes on t2weighted images.
A pediatric case of bickerstaffs brainstem encephalitis ncbi. Bickerstaffs brainstem encephalitis bbe is a very uncommon central nervous system disease with unknown etiology. Nationwide survey of patients in japan with bickerstaff. Other patients may have mild to severe residual findings. Full text get a printable copy pdf file of the complete article 2. Pdf bickerstaffs brainstem encephalitis, miller fisher.
Ideal sources for wikipedia s health content are defined in the guideline wikipedia. A chronic form has rarely been described previously. Herpes simplex encephalitis hse is one of the most common and serious sporadic encephalitides of immunocompetent adults. A 56yearold man presented with a 3day history of progressive tingling of the hands, unsteadiness, and diplopia. The authors report serial mri of a patient with bickerstaffs brainstem encephalitis bbe, disclosing caudal migration of an initial upper midbrain lesion. Bickerstaff encephalitis radiology reference article. Rhombencephalitis, brainstem encephalitis, brain biopsy, autoimmune. Ito m, kuwabara s, odaka m, misawa s, koga m, hirata k, yuki n. Aan members 800 8791960 or 612 9286000 international nonaan member subscribers 800 6383030 or 301 2232300 option 3, select 1 international. It can present at any age and has an estimated incidence of between 1 in 250 000 and 1 in 1 million persons a year. We characterized clinical and paraclinical features, and used regression models to. Severe tick borne encephalitis with simultaneous brain.
When evaluating a patient with suspected central nervous system cns infection, it is essential to determine why this individual, in this place, has developed this disease at this time2. Aan members must change their passwords on the aan site. Treatment for fisher syndrome, bickerstaffs brainstem. 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. Bickerstaffs brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. As it is usually responsive to treatment, the diagnosis this disease. A 44yearold man was hospitalized for drowsiness, cognitive complaint limb weakness, ataxia and sensory disturbance after diarrhea. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some. An atypical bickerstaffs brainstem encephalitis with involvement of. Some of the most frequently reported symptoms include visual. Odaka m1, yuki n, yamada m, koga m, takemi t, hirata k, kuwabara s.
Guidelines on when acyclovir treatment can be suspended in adults exists and. Extensive vasogenic edema in bickerstaff brainstem encephalitis ten days after respiratory infection, a 32yearold woman presented with headaches, ataxia, and diplopia without encephalopathy. Receipt of free study medication from teva neurological. 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. The etiologic categories of re include infections, autoimmune diseases, and paraneoplastic syndromes pns. An atypical bickerstaffs brainstem encephalitis with. A case report article pdf available in the italian journal of neurological sciences 2. After exposure to a tbe endemic region, the patient developed a biphasic clinical course with initial flulike symptoms followed by a severe brain stem syndrome. Successful plasmapheresis in bickerstaffs brain stem.
Request pdf an atypical bickerstaffs brainstem encephalitis with involvement of spinal cord bickerstaff brainstem encephalitis bbe is a rare neurological disease that generally has a good. Bickerstaff brainstem encephalitis and fisher syndrome. Panayiotopoulos 1 division of neurology, king khalid university. Sharif3, abdul rahman shamena1, mohammed a1jaberi2, tahir obeid and chrysostomos c. A case of overlapping bickerstaffs brainstem encephalitis. Different antineuronal autoantibodies, such as antihu, antiri, and antima2 can be associated with the syndrome, and the most frequent tumors are lung and testicular cancer. Bickerstaff brainstem encephalitis bbe is a rare neurological disease that. A pediatric case of bickerstaffs brainstem encephalitis. Bickerstaffs brainstem encephalitis and fisher syndrome form a continuous spectrum.
This disease is notifiable in the uk see noids article for more details. Rhombencephalitis brainstem encephalitis springerlink. Brainstem encephalitis be is an uncommon condition. A case of bickerstaff brainstem encephalitis and acute lymphoblastic leukaemia hasriza hashim1, narisa sulaiman sahari2, sazlyna mohd sazlly lim2, shariful hasan2, fan kee hoo2 summary bickerstaff brainstem encephalitis bbe is a postinfectious immune disorder. Bickerstaff brainstem encephalitis bbe is characterised by acute ophthalmoplegia, ataxia, and some features suggesting cns involvement such as consciousness disturbance. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support. Area postrema syndrome as frequent feature of bickerstaff brainstem encephalitis. Wed like to understand how you use our websites in order to improve them. Brain stem encephalitis caused by primary herpes simplex 2. Diagnostic criteria of bickerstaffs brainstem encephalitis were described as follows4,7. The most common cause of this condition is the presence of cancerous tumors in the brain, although cancer in other parts of the body may cause this inflammation as well. Vasogenic edema in bickerstaffs brainstem encephalitis.