Bickerstaff brainstem encephalitis: A case report

Abide, Zakaria; Nasr, Khosrow; Kaddouri, Soufiane; Edderai, M.; Elfenni, J.; Salaheddine, T.

doi:10.1016/j.radcr.2023.04.038

Cited by 3 publications

(4 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…We included 41 studies ( 20 – 60 ), for a total of 74 cases. 73% of cases were among males (54 males, 20 females).…”

Section: Resultsmentioning

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.

View full text Add to dashboard Cite

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.

show abstract

“…We included 41 studies ( 20 – 60 ), for a total of 74 cases. 73% of cases were among males (54 males, 20 females).…”

Section: Resultsmentioning

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.

View full text Add to dashboard Cite

show abstract

“…He came to the conclusion that BBE was comprised of both typical and atypical cases. The diagnosis of the BBE and MFS overlap (Fisher-Bickerstaff overlap syndrome) is made using a mix of anamnestic, clinical, and radiological findings because there is no particular biological marker for the condition [ 19 ]. BBE does not currently have a proven treatment.…”

Section: Discussionmentioning

confidence: 99%

Bickerstaff’s Brainstem Encephalitis and Miller Fisher Syndrome: A Rare Overlap

Pantbalekundri,

Acharya,

Shukla

et al. 2024

Cureus

View full text Add to dashboard Cite

A rare illness known as "Bickerstaff's brainstem encephalitis" (BBE) is characterized by an abrupt brainstem dysfunction and includes the triad of diminished consciousness, ataxia, and ophthalmoplegia. It differs from the Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) by involving the central nervous system (CNS) and frequently manifesting as reduced consciousness. Here, we describe a rare instance of Bickerstaff's encephalitis coexisting with MFS, where the patient had rapidly progressing quadriplegia, VII cranial nerve palsy, and episodes of unconsciousness.

show abstract

“…GBS has multiple variants that may have an overlapping clinical presentation. For instance, in both MFS and BBE, patients often develop ataxia and ophthalmoplegia, but impaired consciousness is only observed in BBE [ 6 , 7 ]. In our case, the mental status was intact, excluding the BBE possibility.…”

Section: Discussionmentioning

confidence: 99%

“…However, MFS variants including weakness of the respiratory system and limbs have been described. Another variant of MFS is Bickerstaff brainstem encephalitis (BBE) and involves altered consciousness, ataxia, ophthalmoparesis, and paradoxical hyperreflexia [ 6 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

Miller-Fisher Syndrome With Initial Manifestation of Rhinolalia Aperta: A Case Report and Literature Review

Pacut,

Han,

Ghasemi

2023

Cureus

View full text Add to dashboard Cite

Rhinolalia aperta (hypernasal speech) is rarely reported in patients with Miller-Fisher syndrome (MFS). Here, we report a patient with MFS who presented with rhinolalia aperta. A 35-year-old man with a history of alcohol abuse and hepatic cirrhosis presented with a three-day acute hypernasal voice change and numbness of both hands/thighs. After admission, the exam also revealed palatal hypomobility, decreased bilateral hand/thigh sensation, ataxic gait, dysmetria, areflexia, and bilateral abducens palsy. Serum immunoglobulin G (IgG) anti-GQ1b antibody titer was elevated (1:6400). A five-day intravenous IgG was administered with a robust clinical response. Oropharyngeal involvement in MFS can initially manifest with isolated hypernasal speech.

show abstract

Bickerstaff brainstem encephalitis: A case report

Cited by 3 publications

References 15 publications

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

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

Bickerstaff’s Brainstem Encephalitis and Miller Fisher Syndrome: A Rare Overlap

Miller-Fisher Syndrome With Initial Manifestation of Rhinolalia Aperta: A Case Report and Literature Review

Contact Info

Product

Resources

About