Recurrent Diplopia in a Pediatric Patient with Bickerstaff Brainstem Encephalitis

McLeod, Scott A.; Wee, Wallace; Jacob, Francois Dominique; Chapados, Isabelle; Bolduc, François V.

doi:10.1155/2016/5240274

Cited by 3 publications

(3 citation statements)

References 15 publications

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“…Visual deficit including diplopia, esotropia, decreasing of visual acuity were reported in 65% of cases (in one case was reported anisocoria [16]). In one case, the patient had a reoccurrence of diplopia one month after his presentation [15]. Facial/bulbar weakness was reported in 16 cases (40%), limbs weakness in 14 cases (35%), no apparent weakness in 7 patients (17%), global weakness in 3 patients (8%).…”

Section: Resultsmentioning

confidence: 98%

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On clinical findings of Bickerstaff’s brainstem encephalitis in childhood

Messina¹,

Sciuto²,

Fontana³

et al. 2021

J. Integr. Neurosci.

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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.

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Section: Resultsmentioning

confidence: 98%

“…2). The most commonly reported finding was altered reflexes with 24 cases of hyperreflexia (60%), 8 cases of areflexia (20%) and 7 cases of hyporeflexia (18%), only in 1 case deep tendon reflexes remained normal (2%) [15]. Babinski reflex was present in 5 patients.…”

Section: Resultsmentioning

confidence: 99%

On clinical findings of Bickerstaff’s brainstem encephalitis in childhood

Messina¹,

Sciuto²,

Fontana³

et al. 2021

J. Integr. Neurosci.

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show abstract

“…Preceding Campylobacter infections in children with central nervous system (CNS) disorders reported in the literature are scant. 6 Case reports of Bickerstaff brainstem encephalitis associated with previous Campylobacter infection 7 as well as concomitant meningoencephalitis and GBS associated with previous Campylobacter infections have been described in pediatric patients. 8 A case report of a 10-year-old child with the presence of GBS concomitantly with ADEM was described recently, but no associated organism was identified.…”

Section: Discussionmentioning

confidence: 99%

Acute Disseminated Encephalomyelitis Following Campylobacter jejuni Infection in a 12-Year-Old Girl

Camelo

Rana

Cooper

2021

Journal of Pediatric Neurology

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We reported a case of acute disseminated encephalomyelitis (ADEM) in a 12-year-old girl shortly after developing fever and vomiting, and ultimately found to have Campylobacter jejuni by antigen detection and conventional stool culture. Campylobacter jejuni has been associated with peripheral demyelinating diseases including Guillain–Barre's syndrome, but it has not been previously implicated in central demyelination in children. The clinical description and review of the literature are included here.

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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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Recurrent Diplopia in a Pediatric Patient with Bickerstaff Brainstem Encephalitis

Cited by 3 publications

References 15 publications

On clinical findings of Bickerstaff’s brainstem encephalitis in childhood

On clinical findings of Bickerstaff’s brainstem encephalitis in childhood

Acute Disseminated Encephalomyelitis Following Campylobacter jejuni Infection in a 12-Year-Old Girl

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

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