Bickerstaff brainstem encephalitis and overlapping guillainbarre. Encephalitis and postinfectious encephalitis represent two important conditions for the neurologist, both in terms of their presentations as neurologic emergencies and their potential to cause death or serious neurologic impairment. The term rhombencephalitis refers to inflammatory diseases affecting the hindbrain brainstem and cerebellum. One patient had gross flaccid weakness in the four limbs. Listerial brainstem encephalitis lbe is an uncommon form of listerial central nervous system infection that progresses rapidly and is invariably fatal unless detected and treated early. Conclusions bickerstaff brainstem encephalitis is a potentially reversible syndrome that needs early diagnosis facilitated by magnetic resonance imaging and prompt aggressive and supportive treatment.
This disease is notifiable in the uk see noids article for more details. Rearrange individual pages or entire files in the desired order. Pdf bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or. Chronic brainstem encephalitis with mental symptoms and. 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. The absence of prior infection, vaccination, external ophthalmoplegia, alteration of consciousness, and subcortical white matter change made these disease entities less likely17. Brainstem strokes are much less common and have a higher mortality rate than cortical strokes. Bickerstaffs brainstem encephalitis bbe is a rare postinfectious. The aim of this study was to assess the role of cerebrospinal fluid csf neopterin as a biomarker of disease severity in children with enterovirusrelated brainstem encephalitis. It may also affect the peripheral nervous system, and has features in common with both miller fisher syndrome and guillainbarre syndrome. Bickerstaff brainstem encephalitis bbe is a rare autoimmune encephalitis, characterized by acute ophthalmoplegia, ataxia and altered state.
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. Enterovirusa71 causes outbreaks of brainstem encephalitis, ranging from selflimited disease to acute flaccid paralysis. Clinical alert listerial brainstem encephalitis treatable. This syndrome, characterized by a subacute course, usually occurs in adolescents and young adults. Background herpes simplex encephalitis is one of the most common and serious sporadic encephalitides of immunocompetent adults. However, in clinical practice, some disorders encountered are multifocal, and atypical presentations which fall outside of the classic descriptions may occur tables. Encephalitis and postinfectiousencephalitis neuroscience. Acute encephalitis in immunocompetent adults the lancet. Brainstem encephalitis has multiple etiologies, which vary in treatment. Bickerstaff encephalitis radiology reference article.
Coronaviruses, including severe acute respiratory syndrome sars and middle east respiratory syndrome mers stanley perlman and kenneth mcintosh. Brainstem encephalitis the neurological manifestations of brainstem encephalitis are diverse, and may include one or more symptoms of subacute onset. Physical therapy management of a patient with chronic. Exclusion of well defined syndromes of autoimmune encephalitis eg, typical limbic encephalitis, bickerstaffs brainstem encephalitis, acute disseminated encephalomyelitis 3. You may find one of our health articles more useful. Pdf brainstem encephalitis be is an uncommon condition.
In the majority of cases, symptoms have an acute to subacute onset, and evaluation of the cerebrospinal fluid csf often shows abnormalities such as. Aug 21, 2014 professional reference articles are designed for health professionals to use. They are written by uk doctors and based on research evidence, uk and european guidelines. Listeria is the most common cause of infectious re. Mri signal intensity differentiation of brainstem encephalitis induced by enterovirus 71. Mri abnormalities suggestive of autoimmune encephalitis. We characterized clinical and paraclinical features, and used regression models to assess. Guillainbarre syndrome, fisher syndrome and bickerstaff. Get a printable copy pdf file of the complete article 2. An aura is a group of symptoms that generally serve as a warning sign that a bad headache is coming. Rhombencephalitis brainstem encephalitis request pdf. The documents contained in this web site are presented for information. Bickerstaffs brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute.
Most authors now use the terms rhombencephalitis and brainstem encephalitis interchangeably even though anatomically they are slightly different. A case of bickerstaff brainstem encephalitis and acute. Clinical features paraneoplastic encephalitis may manifest as limbic or brainstem encephalitis or be part of widespread involvement of the neuraxis, as in paraneoplastic encephalomyelitis. Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by edwin bickerstaff in 1951. This condition has been named bickerstaffs brainstem encephalitis bbe.
Brainstem encephalitis resulting from epsteinbarr virus. Paraneoplastic and autoimmune encephalitis uptodate. Frequent episodes of transient brainstem dysfunction occurred in our patient during recovery, possibly due to ephaptic transmission. Encephalitis and postinfectiousencephalitis john e. Bickerstaff s brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute ophthalmoplegia, ataxia, and altered sensorium. Be able to conduct a diagnostic investigation of a patient with encephalitis by using clinical and laboratory criteria. A case of a child with subacute neurologic features and imaging findings consistent with a brainstem encephalitis that was discovered to be related to a primary central nervous system infection caused by epsteinbarr virus is presented. Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor. Full text is available as a scanned copy of the original print version. An elderly patient with bickerstaff brainstem encephalitis. 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. We report on six adult patients with lbe, of whom five were managed or comanaged by. Jun 15, 2016 migraine with brainstem aura mba is a type of migraine headache with aura that is associated with pain at the back of the head on both sides.
Compared to cases previously reported as brainstem encephalitis, the clinical and pathological findings observed in these cases have rather peculiar characteristics. Herpes simplex virus 2 hsv2 infections of the central nervous system usually manifest as subacute encephalitis, recurrent meningitis, myelitis, and forms resembling psychiatric syndromes. 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. The etiologic categories of re include infections, autoimmune diseases, and paraneoplastic syndromes pns. Bilateral babinski signs were positive, but tests on the rest of the nervous system could not be performed. A brainstem tumor was initially suspected, but a correct diagnosis was formulated on the basis of the favorable clinical course and the detection of positive.
Absence of well characterized autoantibodies in serum and csf, and at least two of the following criteria. Know the anticipated clinical course of different types of encephalitis. A case of brainstem encephalitis treated with plasmapheresis. 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. Links to pubmed are also available for selected references. Open access mri signal intensity differentiation of brainstem. Antihuassociated brainstem encephalitis journal of. Be familiar with the modalities used to treat encephalitis. 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. Thickened air sacs, tracheal and pulmonary hemorrhages, brainstem encephalitis, myocardial, splenic, pancreatic necrosis virus isolation. 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. Viral causes account for the largest proportion, but in the last decade there has been growing recognition of antineuronal antibody syndromes. Isolated brainstem involvement in a patient with hypertensive. Peterson autoantibody testing in the diagnosis of autoimmune.
Rhombencephalitis brainstem encephalitis springerlink. Rhombencephalitis carries significant morbidity and mortality. 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. 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. A case of rhombencephalitis rebecca bystrom, md internal medicine, pgy2 acp maine chapter meeting 2018. Full text get a printable copy pdf file of the complete article 2. Neuroophthalmologic symptoms may include eyelid ptosis, anisocoria, diplopia due to skew deviation or ophthalmoparesis, oscillopsia due to nystagmus or opsoclonus and. 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.
Enterovirus 71induced brainstem encephalitis and myelitis epos. Atypical brainstem encephalitis caused by herpes simplex. 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. We sought to characterize clinical presentations, etiologies, response to treatment, and predictors of outcome. Brainstem encephalitis be is a rare, severe, and potentially lifethreatening inflammation of the central nervous system. Dec 04, 2016 strokes are a leading cause of disability, and the fifth leading cause of death in the u. Gold standard test positive hemagglutination activityhand or av. The clinical details of 18 cases of brainstem encephalitis are presented. 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. Brainstem encephalitis be is an uncommon condition.
Bickerstaff brainstem encephalitis and guillainbarre syndrome had low muscle tone and weak tendon reflexes in his left arm. Sep 29, 2011 rhombencephalitis re is a syndrome of multiple causes and multiple outcomes. Bickerstaffs brainstem encephalitis bbe, together with miller fisher syndrome mfs and guillainbarre syndrome gbs were considered to form a continuous clinical spectrum. Migraine with brainstem aura genetic and rare diseases.
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