It typically develops in women with ovarian teratoma, and its characteristic clinical symptoms include acute behavioural change, psychosis, movement disorder, seizure and autonomic dysfunction. The autopsy showed a diffuse nodular activation of microglia in the whole encephalon with prominent intraparenchymal and perivascular lymphocytic infiltration of the brainstem. Background herpes simplex encephalitis is one of the most common and serious sporadic encephalitides of immunocompetent adults. To our knowledge, the term rhombencephalitis re was first used in 1951 to refer to inflammatory diseases affecting the hindbrain brainstem and the cerebellum. Listerial brainstem encephalitis treatable, but easily missed. Bickerstaff encephalitis radiology reference article. Bickerstaff brainstem encephalitis journal of the neurological. Paediatric brainstem encephalitis associated with glial and neuronal. Backgroundbickerstaff brainstem encephalitis bbe is a rare inflammatory, demyelinating disease that generally has a good prognosis. Pdf brainstem encephalitis be is an uncommon condition. We included children with a clinical diagnosis of meningitis, encephalitis, brainstem encephalitis, encephalomyelitis with or without autonomic dysfunction, or flaccid. Introduction query encephalitis is a common reason for neurological consultation in hospitalised patients. Antinmethyldaspartate receptor antinmdar encephalitis is an autoimmune disorder involving igg antibody reaction against nmdar. Brain stem encephalitis caused by primary herpes simplex 2 infection in a young.
Paediatric brainstem encephalitis associated with glial and. 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. Bickerstaff brainstem encephalitis bbe and millerfisher syndrome mfs might be a pedigree disease. Brainstem encephalitis resulting from epsteinbarr virus. Health, general blood analysis health aspects medical examination blood tests diagnostic errors encephalitis care and treatment diagnosis research. Encephalitis often causes only mild flulike signs and symptoms such as a fever or headache or no symptoms at all. Compared to cases previously reported as brainstem encephalitis, the clinical and pathological findings observed in these cases have rather peculiar characteristics. We sought to characterize clinical presentations, etiologies, response to treatment, and predictors of outcome. Genomic and serologic characterization of enterovirus a71. Encephalitis is an acute inflammation of the brain. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support. A grave nondemyelinating disease with benign prognosis.
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. Other patients may have mild to severe residual findings. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some. Disease localized to the brainstem was seen in 19 patients. Conclusions bickerstaff brainstem encephalitis is a potentially reversible syndrome that needs early diagnosis facilitated by magnetic resonance imaging and prompt aggressive and supportive treatment.
Enterovirus a71 infection and neurologic disease, madrid. We report a unique case of brainstem encephalitis with a sudden lethal outcome following ipilimumab and pembrolizumab therapy in a patient with malignant melanoma. This syndrome, characterized by a subacute course, usually occurs in adolescents and young adults. Brainstem encephalitis resulting from mumps has rarely been reported 16.
Brainstem encephalitis be is a rare, severe, and potentially lifethreatening inflammation of the central nervous system. Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by edwin bickerstaff in 1951. Aan members 800 8791960 or 612 9286000 international nonaan member subscribers 800 6383030 or 301 2232300 option 3, select 1 international. Brainstem encephalitis is characterized by impaired consciousness, ophthalmoplegia, and cranial nerve involvement 14 and is often associated with an antiganglioside antibody, gq1b 15.
One patient had gross flaccid weakness in the four limbs. Brainstem encephalitis, which characterized the neurologic presentation of the reported child, has been described as being caused by either true viral infection or an immunomediated process. 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. Rhombencephalitis brainstem encephalitis request pdf. Ramsay hunt syndrome complicated by brainstem encephalitis. Clinically, infectious encephalitis is characterised by acute onset of fever, altered mental status, focal neurological deficits and generalised or focal seizures. Brainstem encephalitis be is an uncommon condition. In bickerstaff brainstem encephalitis, hyperreflexia was noted which support of a central. Brainstem disorders 2011th edition pdf, brainstem disorders 2011th edition free download, brainstem disorders 2011th edition ebook, free medical books, medical, medical books free download, medical books, free medical, medical dental books, nursing books, free ebook download, medical textbook, books for medical students. The hospital is a 230bed reference medical center for patients with highcomplexity pathologic conditions.
Herpes simplex virus 2 hsv2 infections of the central nervous system usually manifest as subacute encephalitis, recurrent meningitis, myelitis, and forms resembling psychiatric syndromes. Imaging findings included t2 signal abnormalities in the brain stem and cerebellum with mild patchy enhancement and mass effect. Brainstem encephalitis bbe is an uncommon nervous system disorder characterized by clinical signs and symptoms including acute ophthalmoplegia, ataxia, and impaired consciousness. Atypical brainstem encephalitis caused by herpes simplex. Longterm cognitive and motor deficits after enterovirus 71. Rhombencephalitis brainstem encephalitis springerlink. Brainstem encephalitis definition of brainstem encephalitis.
Encephalitis ensefuhlietis is inflammation of the brain. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. May 30, 2017 we report a unique case of brainstem encephalitis with a sudden lethal outcome following ipilimumab and pembrolizumab therapy in a patient with malignant melanoma. An elderly patient with bickerstaff brainstem encephalitis. Bickerstaffs brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. However, the specific trigger for encephalitis is still obscure 16. Listerial brainstem encephalitistreatable, but easily.
Clinical alert, report by south african medical journal. Overlap of bickerstaff brainstem encephalitisguillainbarre. Viruses cause most cases of infectious encephalitis, but bacteria especially intracellular organisms such as rickettsiae, fungi and parasites are also important. Apr 26, 2019 bickerstaff brainstem encephalitis pdf posted on april 26, 2019 by admin bickerstaffs brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute. Bickerstaffs brainstem encephalitis n14 and clinically isolated syndrome. Brain stem encephalitis caused by primary herpes simplex 2. Herpes simplex virus 2 hsv2 infections of the central nervous system usually manifest as subacute encephalitis, recurrent meningitis, myelitis, and forms. Bickerstaffs brainstem encephalitis is a rare syndrome defined by the triad of ophthalmoplegia, ataxia and decreased consciousness. This article cites 2 articles, 0 of which you can access for free at.
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. It is usually caused by a viral infection, or by the immune system attacking brain tissue, and it is rarely lifethreatening. In addition this patient demonstrated symptoms consistent with brainstem dysfunction, including aspiration and apnea. In particular there are reports detailing headache, anosmia, altered mental staus, seizures, myelitis, neuromuscular disorders, muscle injury and unfortunately. In the 1950s, bickerstaff and fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. Brain mri revealed extensive white matter, brainstem, and cerebellar vasogenic edema, without gadolinium enhancement, partially regressive during followup figure, a. The pathogenesis of secondary fms can be clarified by using neurological examination methods and auxiliary strategies, including tumors in the cerebellopontine angle, inflammation, arterial aneurysm, brainstem encephalitis, syringobulbia, craniocerebral injury and symptoms of. 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. Guillainbarre syndrome, fisher syndrome and bickerstaff. Frequent episodes of transient brainstem dysfunction occurred in our patient during recovery, possibly due to ephaptic transmission. However, the clinical presentation of gbs is diverse. Brainstem encephalitis has multiple etiologies, which vary in treatment. Read article for free, from open access legal sources, via unpaywall. Some of the most frequently reported symptoms include visual.
Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system1 first described by edwin bickerstaff in antigq1b antibody is one that interacts with the peripheral nerve ganglioside, gq1b. This study was designed to determine the longterm cognitive and motor outcomes of children with enterovirus 71 brainstem encephalitis. An elderly patient with bickerstaff brainstem encephalitis and. Treatment for fisher syndrome, bickerstaffs brainstem encephalitis. Acute encephalitis in immunocompetent adults the lancet. Brainstem encephalitis was diagnosed for patients with encephalopathy depressed or altered level of consciousness, lasting more than 24 hours, lethargy, or change in personality or behaviour 8 and 1 predominant brainstem signs cranial neuropathies or ataxia and neuroimaging findings of brainstem abnormalities, or 2 a clinical syndrome. 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. There are several causes, but the most common is a viral infection. The incidence of herpes simplex encephalitis is 1 per 250 000 to 500 000 population per year. 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.
The pathogenesis of secondary fms can be clarified by using neurological examination methods and auxiliary strategies, including tumors in the cerebellopontine angle, inflammation, arterial aneurysm, brainstem encephalitis, syringobulbia, craniocerebral injury and symptoms of laterstage facial nerve paralysis. It is similar to miller fisher syndrome, a variant of guillainbarre syndrome, in that they share features such as ophthalmoplegia and ataxia. Prevalent brainstem and cerebellar signs together with almost exclusive involvement as seen on mri of posterior fossa structures at the onset of the symptoms were the main features of our case. We report the mr imaging findings of a patient with rhombencephalitis caused by adenovirus. It is considered to be a variant of miller fisher syndrome and guillainbarre syndrome but is differentiated from the two by the presence of central nervous system involvement, commonly in the form of impaired consciousness. The difference is that patients with bickerstaffs brainstem encephalitis have impaired consciousness, whereas patients with miller. Rhombencephalitis carries significant morbidity and mortality. Herpes simplex encephalitis is a type of infectious encephalitis which happens when herpes simplex virus hsv enters the brain. Rhombencephalitis re is a syndrome of multiple causes and multiple outcomes. Antinmethyldaspartate receptor encephalitis relapse in. Usually, it begins with flulike symptoms followed by neurological deterioration, which may include personality and behavioural changes, seizures, weakness and difficulties in communication. Enterovirus encephalitis eve can present as meningoencephalitis, an illness similar to herpes simplex encephalitis, or a severe brainstem encephalitis. Miller fisher syndrome mfs, the most recognizable gbs variant, is characterized by an acute onset of ataxia, areflexia, and ophthalmoplegia, and when there is associated disturbance of consciousness, the condition is referred to as bickerstaff brainstem encephalitis bbe. Fisher and bickerstaff, in the 1950s, reported some cases with distinctive features of ophthalmoplegia and ataxia.
Receipt of free study medication from teva neurological. 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. We characterized clinical and paraclinical features, and used regression models to. A statement on covid19 from the encephalitis society updated tuesday, may 5 there are confirmed reports of an association between covid19 and central nervous system cns damage. Varicellazoster virus vzv infection can lead to zoster or cranial nerve palsy such as ramsay hunt syndrome rhs, or complications of central nervous system cns such as myelitis, cerebellitis, encephalitis, and stroke syndrome. Full text get a printable copy pdf file of the complete article 2. Brainstem encephalitis was the most common neurologic complication. Brainstem disorders 2011th edition pdf download medical. Extensive vasogenic edema in bickerstaff brainstem encephalitis. This condition has been named bickerstaffs brainstem encephalitis bbe. Bickerstaffs brainstem encephalitis bbe is an uncommon nervous system disorder characterized by clinical signs and symptoms. What are synonyms for bickerstaff brainstem encephalitis. 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. Aan members must change their passwords on the aan site.
We characterized clinical and paraclinical features, and used regression models to assess. The clinical details of 18 cases of brainstem encephalitis are presented. The etiologic categories of re include infections, autoimmune diseases, and paraneoplastic syndromes pns. Bickerstaffs brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute. We conducted a prospective followup study of children who met the case definition for enterovirus 71 brainstem encephalitis. Bickerstaff brainstem encephalitis and fisher syndrome.
Article abstractwe describe the clinical case and mri findings of a patient with acquired immune deficiency syndrome and pathologically confirmed cytomegalovirus encephalitis. Pdf bickerstaff reported eight patients who, in addition to acute. Bickerstaff brainstem encephalitis synonyms, bickerstaff. Most authors now use the terms rhombencephalitis and brainstem encephalitis interchangeably even though anatomically they are slightly different. Herein, we report a rare case that fits the diagnoses of both mfs and bbe. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in. Patients may show lethargy, somnolence, altered consciousness, personality change, hallucinations, stiff neck, ataxia, seizures or coma. Encephalitis is a rare manifestation of adenovirus infection. Antihuassociated brainstem encephalitis journal of. Paraneoplastic encephalitis, which can affect any part of the nervous system, has been reported to accompany a variety of systemic neoplasms 18. Encephalitis is a condition of inflammation of the brain parenchyma, occurs as a result of infectious or autoimmune causes, and can lead to encephalopathy, seizures, focal neurological deficits, neurological disability, and death.