Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today
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 leve...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2024-03-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2024.1387505/full |
| _version_ | 1797265539954900992 |
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| author | Luca Gregorio Giaccari Donatella Mastria Rosella Barbieri Rossella De Maglio Francesca Madaro Gianfranco Paiano Maria Caterina Pace Pasquale Sansone Giuseppe Pulito Luciana Mascia |
| author_facet | Luca Gregorio Giaccari Donatella Mastria Rosella Barbieri Rossella De Maglio Francesca Madaro Gianfranco Paiano Maria Caterina Pace Pasquale Sansone Giuseppe Pulito Luciana Mascia |
| author_sort | Luca Gregorio Giaccari |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-25T00:46:25Z |
| format | Article |
| id | doaj.art-6bb4cead56894134a6803110999ef672 |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-25T00:46:25Z |
| publishDate | 2024-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-6bb4cead56894134a6803110999ef6722024-03-12T04:38:16ZengFrontiers Media S.A.Frontiers in Neurology1664-22952024-03-011510.3389/fneur.2024.13875051387505Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to todayLuca Gregorio Giaccari0Donatella Mastria1Rosella Barbieri2Rossella De Maglio3Francesca Madaro4Gianfranco Paiano5Maria Caterina Pace6Pasquale Sansone7Giuseppe Pulito8Luciana Mascia9Department of Anesthesia and Intensive Care, “Vito Fazzi” Hospital, Lecce, ItalyDepartment of Anesthesia and Intensive Care, “Vito Fazzi” Hospital, Lecce, ItalyDepartment of Anesthesia and Intensive Care, “Vito Fazzi” Hospital, Lecce, ItalyDepartment of Anesthesia and Intensive Care, “Vito Fazzi” Hospital, Lecce, ItalyDepartment of Anesthesia and Intensive Care, “Vito Fazzi” Hospital, Lecce, ItalyDepartment of Anesthesia and Intensive Care, “Vito Fazzi” Hospital, Lecce, ItalyDepartment of Women, Child, General and Specialist Surgery, University of Campania “L. Vanvitelli”, Naples, ItalyDepartment of Women, Child, General and Specialist Surgery, University of Campania “L. Vanvitelli”, Naples, ItalyDepartment of Anesthesia and Intensive Care, “Vito Fazzi” Hospital, Lecce, ItalyDepartment of Experimental Medicine, University of Salento, Lecce, ItalyBickerstaff 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.https://www.frontiersin.org/articles/10.3389/fneur.2024.1387505/fullBickerstaff brainstem encephalitisBickerstaff’s encephalitisBickerstaff’s syndromechildrenpediatric |
| spellingShingle | Luca Gregorio Giaccari Donatella Mastria Rosella Barbieri Rossella De Maglio Francesca Madaro Gianfranco Paiano Maria Caterina Pace Pasquale Sansone Giuseppe Pulito Luciana Mascia Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today Frontiers in Neurology Bickerstaff brainstem encephalitis Bickerstaff’s encephalitis Bickerstaff’s syndrome children pediatric |
| title | Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today |
| title_full | Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today |
| title_fullStr | Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today |
| title_full_unstemmed | Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today |
| title_short | Bickerstaff encephalitis in childhood: a review of 74 cases in the literature from 1951 to today |
| title_sort | bickerstaff encephalitis in childhood a review of 74 cases in the literature from 1951 to today |
| topic | Bickerstaff brainstem encephalitis Bickerstaff’s encephalitis Bickerstaff’s syndrome children pediatric |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2024.1387505/full |
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