Encephalitis usually affects the meninges as well as the brain, the term meningoencephalitis is more accurate. Bickerstaff s encephalitis be is an acute postinfectious demyelinating disease with albuminocytological dissociation. They are the most commonly identified cause of bacterial intestinal disease in the uk and elsewhere in the developed world. Bickerstaffs brainstem encephalitis bbe is a rare diagnosis different from meningoencephalitis. Miller fisher syndrome mfs and bickerstaff s brainstem encephalitis bbe share some clinical features and a common immunological profile characterized by antigq1b antibodies. Bickerstaff and cloake published a report of three cases they called mesencephalitis and rhombencephalitis bickerstaff and cloake, 1951. According to my doc, if it was caught in the first six months, antivirals and blood cleaning could have minimized nerve damage. A 33yearold woman three weeks after a febrile illness presented with a syndrome of ophthalmoplegia, ataxia and areflexia soaa that characterizes clinically both bickerstaff and miller fisher syndromes. Pdf a pediatric case of bickerstaffs brainstem encephalitis. In the 1950s, bickerstaff and fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. Severe bickerstaffs encephalitis treated with rituximab. Bickerstaffs brainstem encephalitis bbe in childhood. How often and when fisher syndrome is overlapped by. Extensive vasogenic edema in bickerstaff brainstem encephalitis ten days after respiratory infection, a 32yearold woman presented with headaches, ataxia, and diplopia without encephalopathy.
Bickerstaff s brainstem encephalitis bbe is a rare immune disorder in children. Bickerstaffs brainstem encephalitis, miller fisher. Associate professor of pediatrics, oregon health and science university. Pdf bickerstaffs brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of. Bbe is characterized by progressive ophthalmoplegia, ataxia, disturbance of consciousness, pyramidal signs, extensor plantar responses, and long track sensory disturbance1. We report a patient with mfs, bbe, and axonal gbs overlap in whom serial electrophysiological studies showed persistent motor conduction. Vasogenic edema in bickerstaffs brainstem encephalitis. Bickerstaff s brainstem encephalitis is a rare syndrome defined by the triad of ophthalmoplegia, ataxia and decreased consciousness. Brain mri revealed extensive white matter, brainstem, and cerebellar vaso. High apparent diffusion coefficient values imply a vasogenic rather than cytotoxic edema as the cause of the hyperintense signal changes on t2weighted images.
Bbe typically follows an acute flulike infection, followed by progressive onset of ataxia, symmetrical external ophthalmoplegia, hyperreflexia. Pdf chronic bickerstaffs encephalitis with cognitive impairment, a. Bickerstaff brainstem encephalitis bbe is a rare demyelinating disease of the central nervous system, first described by bickerstaff. Prasad 92 bickerstaff brainstem encephalitisbickerstaff brainstem encephalitis symptoms of progressive symmetrical external ophthalmoplegia suggest bickerstaff brainstem encephalitis in association with m. Bickerstaff encephalitis radiology reference article. Medical officer, viral and rickettsial disease laboratory, state of california, richmond, calif after completing this article, readers should be able to. An elderly patient with bickerstaff brainstem encephalitis. Our aim was to elucidate the frequency of this overlap and the. Bickerstaff brainstem encephalitis and fisher syndrome. Campylobacter species are microaerophilic spiralshaped gramnegative bacilli which cause diarrhoeal and systemic illness in humans and animals. Bickerstaff s brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute. Meningitis inflammation of meninges encephalitis an acute inflammatory process affecting the brain parenchyma. This study evaluated longterm neurologic and immune sequelae of bickerstaff brainstem encephalitis in children.
Pdf bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar. Several entities, including listeria rhombencephalitis, bickerstaff s brainstem encephalitis, and ma2associated paraneoplastic encephalitis, have been described in case reports and series 15, but the spectrum of causes and outcomes of be have not been systematically studied. Fisher syndrome and bickerstaff brainstem encephalitis. This condition has been named bickerstaff s brainstem encephalitis bbe. A case report article pdf available in the italian journal of neurological sciences 2. Both presented with acute febrile illness, progressive encephalopathy, and marked cerebrospinal fluid pleocytosis. Published knowledge of pediatric bickerstaff brainstem encephalitis focuses on the acute phase of the disease process. Later, bickerstaff added five more cases to the original study and named the condition brain stem encephalitis bickerstaff. Three young patients with bickerstaff s brainstem encephalitis bbe are reported. To better characterize etiologies and prognosis of be and to. Neuropsychological evaluation showed slowing, memory and executive function. Bickerstaff and cloake first described the entity known as bickerstaff brainstem encephalitis bbe 1.
Bickerstaff brainstem encephalitis bbe is a rare neurological disease that. A chronic form has rarely been described previously. T 2weighted mr imaging showed increased signal intensity in the medulla oblongata, upper pons, pendunculi cerebelli and the cerebellum. Its been almost six months since jy was admitted to the hospital, 102315. Miller fisher syndrome, bickerstaff brainstem encephalitis. A 56yearold man presented with a 3day history of progressive tingling of the hands, unsteadiness, and diplopia. Symptoms typically develop following a respiratory or diarrheal illness and serum antigq1b antibodies are present in approximately two. 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.
Silent aspiration and recovery from dysphagia in a case of. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. Viruses cause most cases of infectious encephalitis, but bacteria especially intracellular organisms such as rickettsiae, fungi and parasites are also important. It is rare, yet the most severe variant of guillainbarre syndrome gbs in terms of initial presentation. The normality of the electrophysiological tests performed, the csf findings and the magnetic resonance images proved that the syndrome stemmed from brainstem pathology. These two conditions may represent a single autoimmune disease. An overlapping case of miller fisher syndrome, bickerstaff. Unlimited viewing of the articlechapter pdf and any associated supplements and figures. Miller fisher syndrome, bickerstaff brainstem encephalitis and guillainbarre syndrome. Herein, we report a rare case that fits the diagnoses of both mfs and bbe. 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. Longterm sequelae of pediatric bickerstaff brainstem. A 44yearold man was hospitalized for drowsiness, cognitive complaint limb weakness, ataxia and sensory disturbance after diarrhea. Moreover, the coexistence of bbe and acute lymphoblastic leukaemia all is distinctly uncommon.
Bickerstaff s brainstem encephalitis is a rare inflammatory disorder of the central nervous system. Pdf fisher syndrome or bickerstaff brainstem encephalitis. Unfortunately in our case we didnt have facilities to measure antiganglioside antibodies. We present a patient with bickerstaff s brainstem encephalitis in whom mr imaging correlated with the clinical findings during the course of the disease. Pdf whether bickerstaffs brainstem encephalitis bbe is a distinct disease or a subtype of fisher syndrome fs is. An antigq1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders. Public health physician, oregon department of human services, portland, ore 2. Bickerstaff brainstem encephalitis bbe and millerfisher syndrome mfs might be a pedigree disease. Miller fisher syndrome, bickerstaff brainstem encephalitis and. A case of bickerstaff brainstem encephalitis and acute. Bickerstaff brainstem encephalitis is a rare, immunemediated disorder of the brainstem and peripheral nervous system. 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 bickerstaff s brainstem encephalitis bbe.
These features are in conjunction with the miller fisher syndrome, so there must be other features the properly diagnose the disorder. Withneyupdate of practice guidelines for the management of. Pdf background bickerstaffs encephalitis be is an acute postinfectious demyelinating disease with albuminocytological. Case one shared some of the clinical features that have been seen in previously reported cases of brainstem encephalitis, such as ophthalmoplegia, ataxia, and progressive encephalopathy. Rehabilitation of a patient with overlap of acute transverse myelitis and bickerstaff s brainstem encephalitis. The bacterial cell wall of the organism contains an endotoxin which, when released, can result in acute enteritis and. Pdf bickerstaffs brainstem encephalitis and fisher syndrome. Fisher syndrome or bickerstaff brainstem encephalitis. 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. Bickerstaff s brainstem encephalitis is a clinical syndrome defined by ophthalmoplegia, ataxia, and altered consciousness although other brainstem and long tract signs are often associated bickerstaff and cloake, 1951, odaka et al. The authors report serial mri of a patient with bickerstaff s brainstem encephalitis bbe, disclosing caudal migration of an initial upper midbrain lesion. Download fulltext pdf download fulltext pdf a pediatric case of bickerstaff s brainstem encephalitis article pdf available in korean journal of pediatrics 5712.
717 395 272 1431 91 1163 1238 831 800 644 983 1604 456 783 476 1116 1083 980 1160 120 1618 484 1284 1458 641 1236 83 132 807 1117 702 885 1375 298 393 240 764 663 954 147 498 1333 396 180 599 636 428 908 674