Bickerstaff's Encephalitis Research Articles

Immunotherapy‐responsive brainstem lesion accompanied with anti‐galactocerebroside antibody

AbstractBackgroundBrainstem lesions are a diagnostic challenge and clinicians have to differentiate between many diseases including acute disseminated encephalomyelitis, multiple sclerosis (MS), Bickerstaff brainstem encephalitis (BBE) and brain tumors. The anti‐galactocerebroside (Gal‐C) antibody is an anti‐glycolipid antibody that is often detected in autoimmune peripheral neuropathy. However, anti‐Gal‐C antibody is rarely detected in autoimmune central nervous system diseases. Here, we report the case of an anti‐Gal‐C antibody‐positive patient who had a brainstem lesion that mimicked MS and responded to immunotherapy.Case presentationWe report a 32‐year‐old woman who presented with subacute numbness of the right lower extremities and hemiataxia. Her cerebrospinal fluid examination showed an elevated myelin basic protein level and an oligoclonal band. Brain magnetic resonance imaging showed a T2 high‐intensity lesion from the left brainstem to the cerebellum. She was treated with steroid pulse therapy, but internuclear ophthalmoplegia and left peripheral facial palsy developed. Her brain magnetic resonance imaging showed an enlargement of the brainstem lesion. Therefore, plasma exchange therapy and high‐dose immunoglobulin therapy were administered, and her symptoms eventually ameliorated. First, we diagnosed her with MS and started dimethyl fumarate. After anti‐GM2 and anti‐Gal‐C antibody positivity was revealed, we diagnosed an anti‐Gal‐C antibody‐related brainstem lesion. There have been few reports of anti‐Gal‐C antibody‐positive central nervous system diseases.ConclusionsThe present case highlights that an anti‐Gal‐C positivity can cause brainstem inflammatory lesions, which should be differentiated from acute disseminated encephalomyelitis, Bickerstaff brainstem encephalitis and MS. The patient’s symptoms improved with intense immunotherapy, and antibody measurement led to the discontinuation of unnecessary disease‐modifying drugs.

Clinical Characterization of Anti-GQ1b Antibody Syndrome in Childhood.

Objective: To delineate the comprehensive clinical features of anti-GQ1b antibody syndrome in childhood.Methods: The clinical data of children diagnosed with anti-GQ1b antibody syndrome at two Chinese tertiary pediatric neurology centers were collected and analyzed. We also conducted a systematic literature review on anti-GQ1b antibody syndrome in children.Results: This study included 78 children with anti-GQ1b antibody syndrome, consisting of 12 previously unreported cases from the two Chinese centers. The median onset age was 10 years (range, 2–18 years). The most common phenotype was acute ophthalmoparesis (32%), followed by classic Miller Fisher syndrome (15%), and Bickerstaff brainstem encephalitis (12%). External ophthalmoplegia (48%), sensory disturbance (9%), and bulbar palsy (9%) were the three most frequent onset symptom manifestations. Brain or spinal lesions on MRI and abnormal recordings by nerve conduction study were present in 18% (12/68) and 60% (27/45) of cases, respectively. There was CSF albuminocytologic dissociation in 34% of the patients (23/68). IV immunoglobulin alone or combined with steroids or plasma exchange was administered to 58% of patients (42/72). We did not find a significant correlation between early improvement up to 3 months and age onset and phenotype. All patients showed different degrees of recovery, and 81% (57/70) had complete recovery within 1 year.Conclusions: Acute ophthalmoparesis and classic Miller Fisher syndrome are the most common phenotypes of anti-GQ1b antibody syndrome in childhood. The majority of patients show good response to immunotherapy and have favorable prognosis.

Cholesterol-added antigens can enhance antiglycolipid antibody activity: Application to antibody testing

We analysed the effect of adding cholesterol to glycolipid antigens on antibody activity with enzyme-linked immunosorbent assay in 123 subjects consisting of 96 patients with Guillain-Barré syndrome, 25 Miller Fisher syndrome, and two Bickerstaff brainstem encephalitis. The use of cholesterol-added GM1 antigens increased anti-GM1 activity in 11 out of 23 anti-GM1-positive patients and resulted in six out of 100 anti-GM1-negative patients becoming anti-GM1-positive. Enhancement of anti-GM1 activity by cholesterol addition was significantly associated with antecedent gastrointestinal infection. The use of cholesterol-added glycolipid antigens can increase the detection rate of anti-glycolipid antibodies and accurately evaluate the anti-glycolipid antibody activity in vivo.

Nationwide Survey of Childhood Guillain-Barré Syndrome, Fisher Syndrome, and Bickerstaff Brainstem Encephalitis in Japan

Background: Guillain-Barre syndrome (GBS), Fisher syndrome (FS), and Bickerstaff brainstem encephalitis (BBE) are immune-mediated neuropathies presenting with symptoms such as muscle weakness, ophthalmoplegia, ataxia, and consciousness disturbances. Although the epidemiology of GBS and BBE in patients of all ages has been reported, childhood data have not been well-investigated. We aimed to determine the clinical features, therapeutics, and prognoses of childhood GBS, FS, and BBE in Japan. Methods: We sent questionnaires to 1068 pediatric neurologists in Japan from 2014 to 2016 to determine the number of children less than 15 years old with GBS, FS, or BBE and their age and sex. We subsequently performed a secondary survey to investigate the clinical features, laboratory data, treatment, and prognosis. This study was approved by the local ethics committee of the Graduate School of Medicine, Chiba University. Findings: Five-hundred thirty-eight pediatric neurology specialists (50·4%) responded to the first survey. The total number of children with GBS, FS, and BBE in Japan from 2014 to 2016 were 87, 10, and 6, respectively, suggesting annual incidence rates of was 0·19, 0·023, and 0·0071 per 100,000 children, respectively, in the pediatric population (0-14 years). GBS was classified as acute inflammatory demyelinating neuropathy (39·7%), acute motor axonal neuropathy (24·4%), or acute motor-sensory axonal neuropathy (11·5%), with a male-to-female ratio of 1·29:1·0 and a wide distribution of onset ages. The disease severities of GBS, FS, and BBE were variable, but all children were able to walk within one year, indicating an excellent childhood prognosis. No in-hospital deaths occurred in this study. Interpretation: The prognoses of childhood GBS, FS, and BBE were generally favorable, as long as the patient was promptly treated with either intravenous immunoglobulin or plasma exchange. Clinical Trial Registration Details: The research scheme was registered at the UMIN Clinical Trials Registry (UMIN-CTR) as UMI18739. Funding Information: This work was supported by the institutional budget of the Chiba University Graduate School of Medicine (grant reference J09KF00284). Declaration of Interests: The authors declare no conflict of interest. Ethics Approval Statement: The study protocol was approved by the institutional ethics committee of the Graduate School of Medicine, Chiba University (No. 319), and the Collaborative Research Committee of the Japanese Society of Child Neurology (No. 13-04).

Fırtına Sonrası Sessizlik; Bickerstaff Beyin Sapı Ensefalitli Bir Olgu

A pediatric case with Bickerstaff’s brainstem encephalitis (BBE), which is a very rare monophasic post-infectious condition characterized by central nervous system involvement, unconsciousness, ophthalmoplegia and ataxia, is presented. Twelve years old patient was brought with difficulty in eye movements and ataxia. On the second day, she became agitated and lethargic, and then bulbar palsy and whole body paralysis developed. Upper motor neuron involvement was evident. Routine biochemical parameters and serologic tests, cranial magnetic resonance imaging, lumbar puncture, autoimmune, paraneoplastic, and electrophysiological studies were evaluated. All were normal except for the encephalopathic first electroencephalography (EEG) and the EEG repeated on the 25th day was reported to be normal. Anti-ganglioside antibody, anti-GQ1b was found positive. Intravenous immunoglobulin (IVIG) started on the fourth day. A very rapid improvement was seen in the first week of IVIG treatment. She was able to walk in the second week. She was completely normal in 3 months. Although seven years passed, our patient has not had any relapse or neurological deficit. We presented a case diagnosed as BBE treated successfully with single dose intravenous Immunoglobulin. We wanted to emphasize that BBE has a good prognosis even though it is an acutely developing severe condition.

Bickerstaff's brainstem encephalitis in childhood: a literature overview.

This is a review on clinical presentation, diagnosis, and treatment of reported cases of Bickerstaff brain encephalitis. Cases of pediatric Bickerstaff's brainstem encephalitis collected from PubMed, Cochrane Library and Scopus Web of Science databases were reviewed. The inclusion criteria of the cases were based on age ≤ 18 years and the clinical characteristics of the disorder. Twenty-seven articles on Bickerstaff's brainstem encephalitis, including 236 children from a total of 42 from January 1990 to January 2020, were selected. The phenotype of the pediatric cases confirmed those described in the previously published articles. Almost half of the cases demonstrated the positivity of anti-GQ1b antibody titers, but the antibodies' presence was not linked to longer healing time. However, it was found that individuals with neuroimaging changes needed a longer time to recovery. Overall, patients treated with any type of immunotherapy (intravenous immunoglobulins, steroid or plasmapheresis) demonstrated faster resolution of symptoms than supportive care. Bickerstaff's brainstem encephalitis is an uncommon disorder, the short-term and long-term prognoses depend on the clinical presentation of the disorder, co-morbidity, instrumental investigations, and precocity of treatment.

On clinical findings of Bickerstaff's brainstem encephalitis in childhood.

A short review on the clinical presentation of pediatrics cases of Bickerstaff brain encephalitis emphasizing the broad clinical spectrum of the disease. Cases of pediatric Bickerstaff's brainstem encephalitis collected on three electronic medical databases (PubMed, Cochrane Library and Scopus Web of Science) are reviewed. The inclusion criteria of the cases were based on the clinical characteristics of the disorder in the pediatric age. We reviewed 20 articles on Bickerstaff's brainstem encephalitis, identifying 40 pediatric cases focused on the clinical symptoms. We saw that the prevalence was higher in male subjects, and the median age at diagnosis was 8 years. The phenotype of pediatrics patients was similar to previously published literature. We identify three cases of overlapping forms between Bickerstaff brain encephalitis and Guillain-Barré Syndrome in patients with lower limbs weakness and typical signs of Bickerstaff brain encephalitis, suggesting a combined involvement of the central and peripheral nervous system. Although there is no defined data on incidence and prevalence in the literature, Bickerstaff's brainstem encephalitis appears to be a rare disorder, especially in children. The incidence of Bickerstaff brain encephalitis and Guillain-Barré Syndrome, and Miller Fisher Syndrome has been underrated in the past, primarily due to an underestimation of the forms with a Peripheral Nervous System involvement. Bickerstaff brain encephalitis usually has a rapid and acute onset within 2-4 weeks, characterized by a typical picture of ophthalmoplegia, hyperreflexia, cerebellar symptoms as ataxia. The subsequent manifestations of hyperreflexia or consciousness disturbances as drowsiness, sleepiness, or coma, indicative of central involvement, suggest a Bickerstaff brain encephalitis clinical diagnosis.

Bickerstaff's brainstem encephalitis with monospecific anti-GT1a antibody: A subtype of Guillain–Barre syndrome

Bickerstaff's brainstem encephalitis is an uncommon post-infectious causing autoimmune neurological disease, which is usually associated with the GQ1b antibody. We described a young man with recent upper respiratory tract infection, who presented with sudden onset of left eye ptosis with internuclear ophthalmoplegia, staccato speech with left-sided hemiplegia but with normal deep tendon reflexes. The cerebral imaging was normal and cerebrospinal fluid analysis produced no cyto-albuminologic dissociation, negative result of tuberculosis culture and virus PCR. He was tested positive for serum GT1a IgG antibody. We discussed this case as the neurological presentation was severe, in the presence of GT1a IgG antibody. La encefalitis troncoencefálica de Bickerstaff es una enfermedad neurológica postinfecciosa autoinmune rara, que está normalmente asociada al anticuerpo GQ1b. Describimos el caso de un varón joven con infección reciente del tracto respiratorio superior, que acudió con aparición súbita de ptosis en el ojo izquierdo con oftalmoplejía internuclear, habla entrecortada con hemiplejía izquierda, aunque con reflejos tendinosos profundos normales. La imagen cerebral fue normal, y el análisis del líquido cefalorraquídeo no produjo disociación cito-albuminológica, siendo negativo el resultado del cultivo para la detección de tuberculosis y la PCR para la presencia de virus. El resultado de anticuerpos séricos GT1a IgG fue positivo. Debatimos este caso, dado que la presentación neurológica fue grave, en presencia de anticuerpos GT1a IgG.

Miller Fisher Syndrome Variant: The Incomplete Triad

Miller Fisher Syndrome (MFS) is a rare inflammatory peripheral neuropathy where the diagnosis is made based on the clinical triad of ophthalmoplegia, ataxia, and areflexia. It is considered a variant of Guillain-Barre Syndrome (GBS) and associated with antiGQ1b IgG positive serology. Reports on cerebellar ataxia and supranuclear gaze palsy in MFS suggested an additional involvement of the central nervous system, encompassing Bickerstaff's Brainstem Encephalitis (BBE) spectrum.

Determining the Utility of the Guillain-Barré Syndrome Classification Criteria.

Background and PurposeSeveral variants of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) exist, but their frequencies vary in different populations and do not always meet the inclusion criteria of the existing diagnostic criteria. However, the GBS classification criteria by Wakerley and colleagues recognize and define the clinical characteristics of each variant. We applied these criteria to a GBS and MFS cohort with the aim of determining their utility.MethodsConsecutive GBS and MFS patients presenting to our center between 2010 and 2020 were analyzed. The clinical characteristics, electrophysiological data, and antiganglioside antibody profiles of the patients were utilized in determining the clinical classification.ResultsThis study classified 132 patients with GBS and its related disorders according to the new classification criteria as follows: 64 (48.5%) as classic GBS, 2 (1.5%) as pharyngeal-cervical-brachial (PCB) variant, 7 (5.3%) as paraparetic GBS, 29 (22%) as classic MFS, 3 (2.3%) as acute ophthalmoparesis, 2 (1.5%) as acute ataxic neuropathy, 2 (1.5%) as Bickerstaff brainstem encephalitis (BBE), 17 (12.9%) as GBS/MFS overlap, 4 (3%) as GBS/BBE overlap, 1 (0.8%) as MFS/PCB overlap, and 1 (0.8%) as polyneuritis cranialis. The electrodiagnosis was demyelinating in 55% of classic GBS patients but unclassified in 79% of classic MFS patients. Anti-GM1, anti-GD1a, anti-GalNAc-GD1a, and anti-GD1b IgG ganglioside antibodies were more commonly detected in the axonal GBS subtype, whereas the anti-GQ1b and anti-GT1a IgG ganglioside antibodies were more common in classic MFS and its subtypes.ConclusionsMost of the patients in the present cohort met the criteria of either classic GBS or MFS, but variants were seen in one-third of patients. These findings support the need to recognize variants of both syndromes in order to achieve a more-complete case ascertainment in GBS.

Encefalitis de bickerstaff

Introduction: Bickerstaff encephalitis is a rare acute neurological disease, characterized clinically by relatively symmetric ophthalmoplegia, ataxia, altered level of consciousness and hyperreflexia, belonging to a continuous pathological spectrum that includes acute ophthalmoplegia, Miller Fisher syndrome and Guillain Barre syndrome. Case report: Female, 26 years old. He started with blurred vision, holocranean headache, generalized myalgias and a 38.5 ° C fever of 7 days of evolution. He found amnesia, dysarthria, ataxia and generalized hyperreflexia, left facio-brachio-crural paresis and left palpebral ptosis. Faced with the deterioration of the level of progressive consciousness, advanced airway management began. Computed tomography (CT) of the skull with severe cerebral edema and magnetic resonance imaging (MRI) of the skull with reduced caliber of posterior cortical vessels. Lumbar puncture with cytological albumine dissociation Electroencephalogram with encephalopathy with tetha-based activity reactive to activation maneuvers and visual evoked potentials with bilateral optic neuropathy of demyelinating type. IgG antigangglioside antibodies with Anti-GQ1b positive. Immunoglobulin treatment was started at a dose of 0.4 g / kg / day for five days with subsequent satisfactory clinical response. Conclussion: The illustrated case has multiple characteristics of those described by Bickerstaff in its initial series, such as ophthalmoplegia, hyperreflexia and altered state of consciousness. The controversy as to whether Bickerstaff’s encephalitis and Guillain-Barré syndrome are different entities, is already raised, as well as the alternative hypothesis that it can be diseases within the same spectrum.

Neurological manifestations associated with SARS-CoV-2 and other coronaviruses: A narrative review for clinicians.

IntroductionThe past two decades have been marked by three epidemics linked to emerging coronaviruses. The COVID-19 pandemic highlighted the existence of neurological manifestations associated with SARS-CoV-2 infection and raised the question of the neuropathogenicity of coronaviruses. The aim of this review was to summarize the current data about neurological manifestations and diseases linked to human coronaviruses.Material and methodsArticles have been identified by searches of PubMed and Google scholar up to September 25, 2020, using a combination of coronavirus and neurology search terms and adding relevant references in the articles.ResultsWe found five cohorts providing prevalence data of neurological symptoms among a total of 2533 hospitalized COVID-19 patients, and articles focusing on COVID-19 patients with neurological manifestations including a total of 580 patients. Neurological symptoms involved up to 73% of COVID-19 hospitalized patients, and were mostly headache, myalgias and impaired consciousness. Central nervous system (CNS) manifestations reported in COVID-19 were mostly non-specific encephalopathies that represented between 13% and 40% of all neurological manifestations; post-infectious syndromes including acute demyelinating encephalomyelitis (ADEM, n = 13), acute necrotizing encephalopathy (ANE, n = 4), Bickerstaff's encephalitis (n = 5), generalized myoclonus (n = 3) and acute transverse myelitis (n = 7); other encephalitis including limbic encephalitis (n = 9) and miscellaneous encephalitis with variable radiologic findings (n = 26); acute cerebrovascular diseases including ischemic strokes (between 1.3% and 4.7% of COVID-19 patients), hemorrhagic strokes (n = 17), cerebral venous thrombosis (n = 8) and posterior reversible encephalopathy (n = 5). Peripheral nervous system (PNS) manifestations reported in COVID-19 were the following: Guillain–Barré syndrome (n = 31) and variants including Miller Fisher syndrome (n = 3), polyneuritis cranialis (n = 2) and facial diplegia (n = 2); isolated oculomotor neuropathy (n = 6); critical illness myopathy (n = 6). Neuropathological studies in COVID-19 patients demonstrated different patterns of CNS damage, mostly ischemic and hemorrhagic changes with few cases of inflammatory injuries. Only one case suggested SARS-CoV-2 infiltration in endothelial and neural cells. We found 10 case reports or case series describing 22 patients with neurological manifestations associated with other human coronaviruses. Among them we found four MERS patients with ADEM or Bickerstaff's encephalitis, two SARS patients with encephalitis who had a positive SARS-CoV PCR in cerebrospinal fluid, five patients with ischemic strokes associated with SARS, eight MERS patients with critical illness neuromyopathy and one MERS patient with Guillain–Barré Syndrome. An autopsy study on SARS-CoV patients demonstrated the presence of the virus in the brain of eight patients.ConclusionThe wide range of neurological manifestations and diseases associated with SARS-CoV-2 is consistent with multiple pathogenic pathways including post-infectious mechanisms, septic-associated encephalopathies, coagulopathy or endothelitis. There was no definite evidence to support direct neuropathogenicity of SARS-CoV-2.

Anti-GQ1b Antibody Syndrome Presenting with Severe Headache

Anti-GQ1b antibody syndrome, including Miller Fisher syndrome, Guillain-Barré syndrome with ophthalmoplegia, Bickerstaff's brainstem encephalitis, and acute ophthalmoplegia without ataxia, has overlapped clinical symptoms and mostly associated with anti-GQ1b immunoglobulin (Ig) G antibody. We report two cases of anti-GQ1b antibody syndrome mainly presenting with a severe headache. The 60-year-old man was admitted for severe headache and gait disturbance. Neurological examination revealed limb and truncal ataxia, areflexia, nystagmus, and ophthalmoplegia. Serum IgG anti-GQ1b antibody was positive. He recovered after intravenous (IV) immunoglobulin and steroid. The 23-year-old man suffered from severe headache (visual analogue scale=10) within the periorbital area. Ophthalmoplegia with gaze-evoked nystagmus were revealed. Serum IgG anti-GQ1b and anti-GT1a antibodies were positive. Headache was improved by IV immunoglobulin and steroid. The pathophysiology of headache in anti-GQ1b antibody syndrome is largely unknown. The affected nerve or structures in the brainstem including the trigeminovascular system may induce intractable severe headache. Keywords: Anti-GQ1b antibody syndrome; Headache; Miller Fisher syndrome 중심 단어: 항GQ1b 항체 증후군, 두통, 밀러 í”¼ì ” 증후군

Plasma C3d levels as a diagnostic marker for complete complement factor I deficiency

Plasma C3d levels as a diagnostic marker for complete complement factor I deficiency

A brain-heart interaction: Bickerstaff's brainstem encephalitis with takotsubo cardiomyopathy.

Bickerstaff's brainstem encephalitis (BBE) is a neurological condition characterised by ataxia, opthalmoplegia and altered sensorium. Neurological diseases, such as Guillain-Barre syndrome, have previously been reported in association with Takotsubo cardiomyopathy. We describe a case of Takotsubo cardiomyopathy associated with BBE. A 62-year-old Chinese female with no significant medical history was admitted after a fall due to transient bilateral lower limb numbness. A day after her initial presentation, she developed altered sensorium and further deteriorated within hours - with bulbar weakness, increasing oxygen requirements requiring intubation and cardiogenic shock needing noradrenaline support. She also had rapid atrial fibrillation treated with amiodarone. Her cardiac enzymes were elevated (high sensitivity troponin I 2137pg/ml; upper limit 15.6pg/ml) and echocardiography demonstrated apical ballooning and dyskinesis with hypercontractility of the base in the left ventricle consistent with Takotsubo cardiomyopathy. Examination revealed bulbar weakness with poor gag and cough reflexes, ataxia and complex opthalmoplegia. Her reflexes were brisk and pupils mydriatic with sluggish pupillary response. Magnetic resonance imaging revealed a gadolinium-enhancing lesion in the brainstem on T1 sequence and anti-GQ1b antibody was positive. She was diagnosed with BBE and treated with intravenous immunoglobulin with good functional recovery. Repeat echocardiography demonstrated interval improvement in apical ballooning. This is the first described case of Takotsubo cardiomyopathy associated with BBE. The pathogenesis of Takotsubo cardiomyopathy in relation to BBE is postulated to be related to dysautonomia with sympathetic overactivity and excessive catecholamine release. In cases of hemodynamic instability in BBE, Takotsubo cardiomyopathy should be considered.

Clinical Characteristic Analysis of Seven Children With Bickerstaff Brainstem Encephalitis in China.

Objective: To summarize the clinical, electrophysiological, neuroimaging, and immunological characteristics of seven cases of Bickerstaff brainstem encephalitis (BBE) in China and to determine whether certain clinical features suggestive of BBE can facilitate diagnosis and treatment.Patients and Methods: The clinical data of seven BBE patients treated at the Children's Hospital of Fudan University between 2016 and 2019 were retrospectively analyzed. The clinical and laboratory characteristics of the BBE patients were studied.Results: The seven patients in this study included four females and three males, and the median age at diagnosis was 5.3 years (interquartile range: 3.0–7.1 years). All seven patients had an acute onset with a preinfection history. Seven cases of acute extraocular paralysis, ataxia, and an impaired level of consciousness, two cases of tendon hyperreflexia, one case of positive pathology, and five cases of cranial nerve involvement (the facial nerve and oculomotor nerve) were noted. Cerebrospinal fluid (CSF) examination of five patients showed albuminocytologic dissociation. Electromyography (EMG) was used to examine seven patients; the results were normal in four patients, showed axonal involvement in two patients, and showed demyelination in one patient. The head magnetic resonance imaging (MRI) results of all seven patients were normal. Electroencephalogram (EEG) background activity in the five monitored patients was slowed down. Seven patients underwent serum antibody testing, three of whom were positive for anti-GQ1b antibody, while one was positive for anti-GM1 antibody. Three patients received glucocorticoid combined with intravenous immunoglobulin (IVIG) therapy, and four received only IVIG therapy. One patient required a nasal catheter for oxygen during the disease course, and left upper limb muscle dysfunction (grade III muscle strength of the left upper limb) was observed during the 6-month follow-up. The other six patients had a good prognosis and no dysfunction.Conclusion: Our study identified clinical, imaging, and treatment characteristics that may have prognostic value for pediatric BBE. The positive rate of head MRI was low, the positive rate of serum anti-GQ1b ganglioside antibody was low, and the therapeutic effect of IVIG therapy was good.

Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies.

ObjectiveTo describe the main syndrome and clinical course in a large cohort of patients with anti–Ri-associated paraneoplastic neurologic syndrome (Ri-PNS).MethodsTwenty-year retrospective nationwide study and systematic review of the literature.ResultsThirty-six patients with complete clinical information were identified (median age 66 years, range: 47–87 years). In this French cohort, the majority were women (78%). At onset, 4 main patterns were observed: cerebellar syndrome (39%), isolated tremor (24%), oculomotor disturbances (17%), and other symptoms (19%). Course was multistep for 78% of cases. At the time the disease reached the plateau phase (median 12 weeks, range: 1–64 weeks; 28% >3 months), 24 (67%) showed an overt cerebellar syndrome, which was isolated in 3 patients, and was most frequently (21/24 cases) part of a multisystem neurologic disease. Patients manifested a variety of movement disorders, including myoclonus (33%), dystonia (17%), either cervical or oromandibular, and parkinsonism (17%). Most patients had cancer (92%), mainly breast cancer (n = 22). Misdiagnoses concerned 22% of patients (n = 8) and included atypical parkinsonism (n = 2), MS (n = 2), Bickerstaff encephalitis (n = 1), hyperekplexia (n = 1), vestibular neuritis (n = 1), and functional neurologic disorder (n = 1). Survival at 12 months was 73% (95% CI [0.54–0.85]), at 24 months 62% (95% CI [0.41–0.78]), and at 36 months 47% (95% CI [0.25–0.65]). There was no major clinical difference between cases retrieved from the systematic review of the literature (n = 55) and the French cohort.ConclusionsRi-PNS is a multisystem neurologic syndrome with prominent cerebellum/brainstem involvement. Opsoclonus-myoclonus is less common than expected, and the disorder can mimic neurodegenerative diseases.

Interesting Case Presentation of Bickerstaff Brainstem Encephalitis with Overlapping Guillain Barre Syndrome

Interesting Case Presentation of Bickerstaff Brainstem Encephalitis with Overlapping Guillain Barre Syndrome

Bickerstaff Brainstem Encephalitis with Isolated Acute Bilateral Ophthalmoplegia: An Unusual Presentation.

Bickerstaff Brainstem Encephalitis with Isolated Acute Bilateral Ophthalmoplegia: An Unusual Presentation.

Locked-in on Bora Bora—Chikungunya triggered Bickerstaff’s encephalitis mimicking brain death in French Polynesia

Introduction: This is a case report about a severe neurological manifestation of a tropical disease that resulted in an overlap syndrome mimicking brain death. We are showing a Chikungunya (CHIKV)-associated encephalitis triggering an overlapping autoimmunological disease [antibody negative Bickerstaff’s brainstem encephalitis (BBE) with Miller Fisher syndrome (MFS) and Guillain–Barré syndrome (GBS)] and the resulting complications. Until recently, CHIKV was considered to cause a relatively harmless self-limiting disease with Dengue-like symptoms (DENV). This case supports the idea of likely neuroinvasiveness of CHIKV, suggesting a potential high severity of this infection. Chikungunya shows a global development and evolution from a rarely seen pathogen toward an epidemic causing disease within only a few decades. Case Report: A 74-year-old man fell sick during his holidays in French Polynesia. He initially presented with fever and a rash, which slowly progressed to hoarseness and back pain. Within one week, the patient developed numbness of the fingertips and difficulties breathing, finally resulting in a complete irresponsive neurological state. Local physicians assumed brain death caused by a cardiac event and voted for termination of mechanical ventilatory support. However, the patient′s wife perceived responses of her husband and initiated extensive repatriation by the Swiss Air Rescue, Rega. Strikingly, the patient recovered subsequently and is wheelchair mobile today. Conclusion: This case demonstrates how tropical airborne virus diseases become directly visible effects of global warming and globalization, as the disease recently seems to appear in Europe and America. It exemplifies how a broader knowledge of parainfectious neurologic syndromes might help to save lives by avoiding misdiagnosis or potentially fatal therapeutic decisions.