We conducted a prospective followup study of children who met the case definition for enterovirus 71 brainstem encephalitis. Here we describe a patient with breast cancer and an antineuromyelitis optica nmo limbic and brainstem encephalitis syndrome who improved. The etiologic categories of re include infections, autoimmune diseases, and paraneoplastic syndromes pns. We report a patient with mfs, bbe, and axonal gbs overlap in whom serial electrophysiological studies showed persistent motor conduction. This study was designed to determine the longterm cognitive and motor outcomes of children with enterovirus 71 brainstem encephalitis. Nationwide survey of patients in japan with bickerstaff. Ev71 infection is usually selflimited but may progress to cause aseptic meningitis, brainstem encephalitis be, and acute flaccid paralysis that is indistinguishable from poliomyelitis. We characterized clinical and paraclinical features, and used regression models to assess. 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. Pdf zet herpes simpleks ensefaliti tipik olarak medial temporal ve inferior frontal loblar etkilemekte, beyin sap lezyonlar oldukca nadir olarak.
Viruses cause most cases of infectious encephalitis, but bacteria especially intracellular organisms such as rickettsiae, fungi and parasites are also important. Sep 23, 2003 the authors report serial mri of a patient with bickerstaffs brainstem encephalitis bbe, disclosing caudal migration of an initial upper midbrain lesion. Pdf bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar. Bickerstaffs brainstem encephalitisbbe is rare postinfectious immunemediated disorder. Brainstem encephalitis is characterized by impaired consciousness, ophthalmoplegia, and cranial nerve involvement and is often associated with an antiganglioside antibody, gq1b. We conducted a prospective followup study of children who met. Hypertensive brainstem encephalopathy was the most pertinent diagnosis in view of the marked clinicalradiological dissociation. Enterovirus encephalitis eve can present as meningoencephalitis, an illness similar to herpes simplex encephalitis, or a severe brainstem encephalitis. Bickerstaffs brainstem encephalitis bbe is a rare diagnosis different from meningoencephalitis. Mri characteristics of brainstem encephalitis in handfoot. The clinical details of 18 cases of brainstem encephalitis are presented.
Bickerstaffs brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute. Encephalitis is a serious neurological condition and unfortunately, despite improvements in specific and more supportive treatments such as excellent intensive care management, it still has a high mortality death rate. They are autoimmune, postinfectious diseases and present common clinical features such as ophthalmoplegia and ataxia2. 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. Bickerstaff brainstem encephalitis bbe is a rare autoimmune encephalitis, characterized by acute ophthalmoplegia, ataxia and altered state. Most authors now use the terms rhombencephalitis and brainstem encephalitis interchangeably even though anatomically they are slightly different. Miller fisher syndrome, bickerstaff brainstem encephalitis. Review bickerstaff brainstem encephalitis and fisher syndrome. Acute hemorrhagic leukoencephalitis ahle, also known as westonhurst syndrome. Jul 21, 2018 miller fisher syndrome mfs and bickerstaffs brainstem encephalitis bbe share some clinical features and a common immunological profile characterized by antigq1b antibodies.
An atypical bickerstaffs brainstem encephalitis with involvement of. Atypical brainstem encephalitis caused by herpes simplex virus 2. Brainstem encephalitis resulting from mumps has rarely been reported. Brainstem encephalitis was the most common neurologic complication. Diagnostic criteria of bickerstaffs brainstem encephalitis were described as follows4,7. Type i cases exhibited unilateral or bilateral symmetrical patchlike hyperintense t 1 and t 2 signals from the posterior brainstem that were restricted mainly to gray matter nuclei fig. Herpes simplex encephalitis hse is one of the most common and serious sporadic encephalitides of immunocompetent adults. Bickerstaff brainstem encephalitis bbe is a rare demyelinating disease of the central nervous system, first described by bickerstaff. Brainstem encephalitis rhombencephalitis due to listeria. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some. Jan 01, 20 bickerstaff brainstem encephalitis bbe is a rare demyelinating disease of the central nervous system, first described by bickerstaff. The california encephalitis project cep was initiated in 1998 to elucidate encephalitis etiologies in california.
Bbe typically follows an acute flulike infection, followed by progressive onset of ataxia, symmetrical external ophthalmoplegia, hyperreflexia. A pediatric case of bickerstaffs brainstem encephalitis. High apparent diffusion coefficient values imply a vasogenic rather than cytotoxic edema as the cause of the hyperintense signal changes on t2weighted images. Correlation of the clinical course with radiological and pathological findings confirms that this is not a variant of polyradiculitis or the guillainbarre syndrome, but is a distinct clinical entity which carries a benign prognosis. Acute disseminated encephalomyelitis adem is a rare disease characterized by severe inflammation of the central nervous system cns with resultant destruction of myelin and white matter. Enterovirus a71 eva71 is known to cause cardiopulmonary failure due to brainstem encephalitis, but there have been few reports of these conditions being associated with evd68. Table 1 summary of csf findings and clinical courses. It is thought to be autoimmune related and possibly triggered by viral infection or even recent immunization.
Rhombencephalitis brainstem encephalitis request pdf. Data were collected from 14 patients diagnosed by members of the paraneoplastic neurological syndromes euronetwork, and eight patients from the literature who presented with isolated brainstem encephalitis and had antihu. Pdf brainstem encephalitis be is a rare, severe, and potentially lifethreatening inflammation of the central nervous system. Silent aspiration and recovery from dysphagia in a case of. Annari, anna2hu, yo, mata, cv2, and amphysin antibodies were not present in the serum. Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by edwin bickerstaff in 1951. By continuing to use our website, you are agreeing to our use of cookies. The ct findings in our patient can represent diffuse glioma, infectious brainstem encephalitis, wernickes encephalopathy and central pontine myelinolysis. Longterm cognitive and motor deficits after enterovirus. In the 1950s, bickerstaff and fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia.
Encephalitis with thalamic and basal ganglia abnormalities. Rhombencephalitis re is a syndrome of multiple causes and multiple outcomes. Fluorodeoxyglucosepositron emission tomography scan revealed increased uptake in the brainstem medulla, pons, and dorsal midbrain and left medial temporal lobe, consistent with brainstem and limbic encephalitis. One patient had gross flaccid weakness in the four limbs. Full text get a printable copy pdf file of the complete article 2. Paraneoplastic brainstem encephalitis and antiri antibodies 3 article pdf available in journal of neurology 24911.
This condition has been named bickerstaffs brainstem encephalitis bbe. Any information contained in this pdf file is automatically generated from digital material submitted to epos by third parties in the form of scientific presentations. We report a patient with mfs, bbe, and axonal gbs overlap in whom serial electrophysiological studies showed persistent motor conduction blocks. We characterized clinical and paraclinical features, and used regression models to.
The 21 cases of brainstem encephalitis were divided into two types, type i cases and type ii 8 cases based on lesion patterns on mri. Chronic brainstem encephalitis with mental symptoms and. Conclusions bickerstaff brainstem encephalitis is a potentially reversible syndrome that needs early diagnosis facilitated by magnetic resonance imaging and prompt aggressive and supportive treatment. Introduction query encephalitis is a common reason for neurological consultation in hospitalised patients. Antihuassociated brainstem encephalitis journal of. Bickerstaff encephalitis radiology reference article. Outbreaks of evd68 infection have occurred in the usa, canada, europe and. Frequent episodes of transient brainstem dysfunction occurred in our patient during recovery, possibly due to ephaptic transmission. Rehabilitation of a patient with overlap of acute transverse myelitis. Since then, such a condition has been called bickerstaffs brainstem encephalitis bbe. Methods we conducted a nationwide survey of bbe in the japanese population in two steps. Brain stem encephalitis caused by primary herpes simplex 2. Pdf adult coeliac disease and brainstem encephalitis. Data were collected from 14 patients diagnosed by members of the paraneoplastic neurological syndromes euronetwork, and eight patients from the literature who presented with isolated brainstem encephalitis and had anti.
Condition is relatively rare in children and there are very. 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. Enterovirus d68 evd68 causes respiratory illnesses such as pneumonia, and has been reported to cause acute flaccid myelitis. Brainstem encephalitis with kikuchifujimoto disease. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. Clinically, infectious encephalitis is characterised by acute onset of fever, altered mental status, focal neurological deficits and generalised or focal seizures. Bickerstaffs brainstem encephalitis bbe is a rare, autoimmune mediated inflammatory condition, which is associated with. Following this, other neurological presentations that share antigq1b antibodies emerged in the literature. The japanese national institute of infectious diseases analyzed serum obtained from the patient.
Longterm cognitive and motor deficits after enterovirus 71. Bickerstaff brainstem encephalitis and fisher syndrome. Brainstem encephalitis by contrast with limbic encephalitis, patients with brainstem encephalitis can have preserved consciousness and behaviour and thus might not strictly fulfil proposed diagnostic criteria for encephalitis. Dec 31, 2014 bickerstaffs brainstem encephalitis was defined as ophthalmoplegia, ataxia, and impaired consciousness1. However, the specific trigger for encephalitis is still obscure. Aan members 800 8791960 or 612 9286000 international nonaan member subscribers 800 6383030 or 301 2232300 option 3, select 1 international. Brainstem encephalitis be is an uncommon condition. 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. Some of the most frequently reported symptoms include visual. Listerial brainstem encephalitis is a rare disease. Bickerstaff brainstem encephalitis bbe is a rare neurological disease that.
Bickerstaffs brainstem encephalitis in children a case series from. Enterovirus 71induced brainstem encephalitis and myelitis epos. Pdf paraneoplastic brainstem encephalitis and antiri. Miller fisher syndrome, bickerstaff brainstem encephalitis and. The authors report serial mri of a patient with bickerstaffs brainstem encephalitis bbe, disclosing caudal migration of an initial upper midbrain lesion. Brainstem encephalitis has multiple etiologies, which vary in treatment. Aan members must change their passwords on the aan site. An elderly patient with bickerstaff brainstem encephalitis. However, there was no involvement of the medial temporal lobes. Compared to cases previously reported as brainstem encephalitis, the clinical and pathological findings observed in these cases have rather peculiar characteristics.
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. Rhombencephalitis refers to inflammatory diseases affecting the hindbrain brainstem and cerebellum and has a wide variety of etiologies including infection, autoimmune disease, and paraneoplastic syndrome, with listeria monocytogenes being cited as the most common cause. Bbe typically follows an acute flulike infection, followed by progressive onset of ataxia, symmetrical external. Apr 14, 2020 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. Rhombencephalitis carries significant morbidity and mortality. Outbreaks of evd68 infection have occurred in the usa, canada, europe and asia. The findings revealed evd68 infection, which was thus confirmed as the cause of brainstem encephalitis. Bickerstaffs brainstem encephalitis is in the same spectrum with miller fisher syndrome and guillainbarre syndrome. Progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks and disturbance of consciousness or hyperreflexia are both required8. Miller fisher syndrome, bickerstaff brainstem encephalitis and guillainbarre syndrome.
Recently, seropositive neuromyelitis optica spectrum disorders nmosd presenting with transverse myelitis or optic neuritis have been reported in association with various cancer types, raising the possibility of paraneoplastic nmosd. Paraneoplastic brainstem encephalitis manifests as a variety of gaze palsies or other ocular motor disturbance, possibly together with dysarthria, dysphagia, facial weakness, vertigo, central respiratory failure, or other signs and symptoms referable to the brainstem saiz et al. Background herpes simplex encephalitis is one of the most common and serious sporadic encephalitides of immunocompetent adults. Backgroud information regarding the epidemiological background of bickerstaff brainstem encephalitis bbe is limited. Bickerstaff brainstem encephalitis and overlapping guillainbarre. Other patients may have mild to severe residual findings. Taiwan experienced 3 major epidemics in 1998, 2000, and 2001. Brainstem encephalitis be is a rare, severe, and potentially lifethreatening inflammation of the central nervous system.
Herpes simplex virus 2 hsv2 infections of the central nervous system usually manifest as subacute encephalitis, recurrent meningitis, myelitis, and forms resembling psychiatric syndromes. Patients may show lethargy, somnolence, altered consciousness, personality change, hallucinations, stiff neck, ataxia, seizures or coma. Request pdf brainstem encephalitis with kikuchifujimoto disease a case of young woman is described who developed clinical and mri features of brainstem encephalitis in the setting of fever. Several large outbreaks of enterovirus 71 infections have occurred in taiwan during the past decade. Atypical brainstem encephalitis caused by herpes simplex. We sought to characterize clinical presentations, etiologies, response to treatment, and predictors of outcome. Pathogenesis of enterovirus 71 brainstem encephalitis in. Brainstem and limbic encephalitis with paraneoplastic. Withneyupdate of practice guidelines for the management of. Miller fisher syndrome mfs and bickerstaffs brainstem encephalitis bbe share some clinical features and a common immunological profile characterized by antigq1b antibodies.
Cardiopulmonary failure as a result of brainstem encephalitis. Among these cases, subsets exist with distinct clinical, laboratory, or neuroimaging features, including a unique subset with abnormalities involving the thalamus andor basal ganglia tbg. Paraneoplastic brain stem encephalitis can occur as an isolated clinical syndrome or, more often, may be part of a more widespread encephalitis. A 27 year old woman developed a vesicular genital rash and cerebellar dysfunction with progressive neurological deterioration suggesting brain stem encephalitis. A subsequent publication from a group of researchers, which included bickerstaff al.
In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support. Vasogenic edema in bickerstaffs brainstem encephalitis. 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. Acute disseminated encephalomyelitis presenting as a. 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. Some mfs patients overlap with guillainbarre syndrome gbs or bbe.
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