Electroencephalographic findings in Bickerstaff’s brainstem encephalitis: A possible reflection of the dysfunction of the ascending reticular activating system

Yoshimura, Hajime; Togo, Masaya; Ishii, Junko; Ishiyama, Hiroyuki; Tamura, Ryota; Kimura, Masamune; Kuroda, Takehito; Kusunoki, Susumu; Kawamoto, Manabu; Kohara, Nobuo

doi:10.1016/j.cnp.2020.11.004

Cited by 5 publications

(3 citation statements)

References 20 publications

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“…Actually, the present study revealed that diffuse beta activity occurred more frequently in initial EEGs from NMDARE patients (5 vs. 1 patient, p = 0.046) than those with other AEs. On the other hand, a recent study more commonly detected a slow component, such as delta activity, in patients with AEs (28)(29)(30)(31)(32)(33): 51% in total AEs, 56% in NMDARE, and 40% in other AEs (33). In addition, GRDA with fast activity is more common in NMDARE than in other AEs (34).…”

Section: Discussionmentioning

confidence: 94%

Novel qEEG Biomarker to Distinguish Anti-NMDAR Encephalitis From Other Types of Autoimmune Encephalitis

et al. 2022

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ObjectiveTo establish the diagnostic biomarker of electroencephalogram (EEG) to distinguish between anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) and other types of autoimmune encephalitis (other AEs).MethodsWe reviewed the clinical records of 90 patients with acute encephalitis who were treated in our institution between January 2014 and October 2020. We enrolled the patients who fulfilled the diagnostic criteria for possible AE (pAE) defined by Graus et al. (pAE criteria) and then classified into definite NMDARE and other AEs. We investigated the main syndrome and analyzed all admission EEGs using EEG power value (PV). Statistical significance was tested using the Mann–Whitney U test or Fisher’s exact test.ResultsTwenty-five patients fulfilled the pAE criteria and were classified into 9 with definite NMDARE (median age: 21 years; 8 women) and 12 with other AEs (median age: 37.5 years; 6 women). Four were eventually excluded. Speech dysfunction (9/9 vs. 4/12, p = 0.005) and movement disorders (6/9 vs. 1/12, p = 0.016) were more frequent in NMDARE than in other AEs. The PV analyses revealed the novel quantitative EEG (qEEG) index, namely, fast slow ratio (FSR) (PV of total beta/PV of total theta + delta). The median FSR (0.139 vs. 0.029, p = 0.004) was higher for NMDARE than other AEs, and the receiver operating characteristic curve area of FSR was 0.86 (95% CI 0.70–1.00). A cutoff value of 0.047 yielded a specificity of 0.75 and a sensitivity of 1.00. Focusing on patients who did not meet the “probable NMDARE criteria” in Graus 2016 (proNMDARE criteria) (n = 10), the pretest probability of NMDAR antibody test was 0.30 (3/10), which increased in patients with an FSR greater than the cutoff (n = 5) to 0.60 (3/5).ConclusionsThe NMDARE group highlighted speech dysfunction and movement disorders, and a novel qEEG index FSR accurately distinguished the NMDARE patients from other AEs. The FSR is a promising diagnostic marker for NMDARE that indicates the positive results of NMDAR antibodies in patients with AE when combined with the proNMDARE criteria.

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Section: Discussionmentioning

confidence: 94%

Novel qEEG Biomarker to Distinguish Anti-NMDAR Encephalitis From Other Types of Autoimmune Encephalitis

et al. 2022

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“…The frequency of abnormal EEG findings indicated CNS involvement, consistent with disturbance of consciousness. EEG changes correlate with the level of consciousness in patients with BBE and often show predominant N1 and/or N2 sleep patterns, even with external stimuli (“unarousable sleep-like” EEG), including the fusiform coma pattern ( 11 ). The deterioration of consciousness in BBE may be caused by a dysfunction of the brainstem reticular formation ( 11 ).…”

Section: Discussionmentioning

confidence: 99%

“…EEG and EMG are indicative of central nervous system (CNS) impairment and predominantly in the brainstem ( 9 ). Patients with BBE showed a characteristic “unarousable sleep-like” EEG ( 11 ). In about one-third of the BBE patients, MRI shows high-intensity areas on T2-weighted images of the brainstem, thalamus, cerebellum and cerebrum ( 12 ).…”

Section: Introductionmentioning

confidence: 99%

Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today

Giaccari,

Mastria,

Barbieri

et al. 2024

Front. Neurol.

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Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune disease characterized by the subacute onset of bilateral external ophthalmoplegia, ataxia, and decreased level of consciousness. BBE is part of a group of rare autoimmune diseases in children that can affect the nervous system at any level. The onset of neurological deficits is often sudden and nonspecific. The diagnosis is based on clinical findings and abnormal findings on cerebrospinal fluid (CSF), electroencephalography (EEG), electromyography (EMG), and magnetic resonance imaging (MRI). BBE is associated with the presence of the antiganglioside antibody, anti-GQ1b and anti-GM1. Intravenous immunoglobulin (IVIg) and plasma exchange are often used as treatments for these patients. We conducted a review on clinical presentation, diagnosis, treatment and outcome of reported cases of BBE. 74 cases are reported in the literature from the first cases described in 1951 to today. The prevalence is unknown while the incidence is higher in males. In 50% of cases, BBE occurs following respiratory or gastrointestinal tract infections. The most frequent initial symptoms were consciousness disturbance, headache, vomiting, diplopia, gait disturbance, dysarthria and fever. During illness course, almost all the patients developed consciousness disturbance, external ophthalmoplegia, and ataxia. Lumbar puncture showed pleocytosis or cytoalbuminological dissociation. Abnormal EEG and MRI studies revealed abnormalities in most cases. Anti-GQ1b antibodies were detected in more than half of the patients; anti-GM1 antibodies were detected in almost 40% of patients. Treatment guidelines are missing. In our analysis, steroids and IVIg were administered alone or in combination; as last option, plasmapheresis was used. BBE has a good prognosis and recovery in childhood is faster than in adulthood; 70% of patients reported no sequelae in our analysis. Future studies need to investigate pathogenesis and possible triggers, and therapeutic possibilities.

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Etiology matters for neuroprognostication: A multimodal electrophysiological investigation in a case of Bickerstaff's brainstem encephalitis

Cluse

Pégat

Ritzenthaler

et al. 2022

Neurophysiologie Clinique

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Electroencephalographic findings in Bickerstaff’s brainstem encephalitis: A possible reflection of the dysfunction of the ascending reticular activating system

Cited by 5 publications

References 20 publications

Novel qEEG Biomarker to Distinguish Anti-NMDAR Encephalitis From Other Types of Autoimmune Encephalitis

Novel qEEG Biomarker to Distinguish Anti-NMDAR Encephalitis From Other Types of Autoimmune Encephalitis

Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today

Etiology matters for neuroprognostication: A multimodal electrophysiological investigation in a case of Bickerstaff's brainstem encephalitis

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