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

OBJECTIVE: This is a review on clinical presentation, diagnosis, and treatment of reported cases of Bickerstaff brain encephalitis. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Primary headache in childhood associated with psychiatric disturbances: an update.

OBJECTIVE: Primary headache disorders in children are one of the most prominent topics in the pediatric neurology literature. However, there are many unsolved aspects, including the conditions associated with migraine. The present study aims to report on the frequency of behavioral comorbidities in the setting of primary headache in childhood. PATIENTS AND METHODS: In this study, we enlisted 475 children (290 males and 185 females; ratio 1.6:1), aged 4 to 14 years, who were affected by primary headache. In direct interviews, children and parents gave information on the association of their headache with, attention-deficit/hyperactivity disorder, learning disabilities, tics, anxiety, depression, and obsessive-compulsive disorder. Other 475 children with no history of headache or recognized neurological conditions were matched for age, sex, race, and socioeconomic status and were used as controls. RESULTS: A significant association of primary headache was found with anxiety and depression (p-value <0.001); overall, behavioral disorders were more common in children who experienced headache than in controls (p-value <0.001). CONCLUSIONS: Primary headache in children is not associated with most of the common behavioral conditions. On the contrary, there was a significant association with anxiety and depression, as reported in adults.

The epidemiology of amyotrophic lateral sclerosis in the Mount Etna region: a possible pathogenic role of volcanogenic metals.

BACKGROUND AND PURPOSE: Trace elements (TEs) may play a role in the pathogenesis of amyotrophic lateral sclerosis (ALS) and volcanic degassing is the major natural source of TEs. Mount Etna, in the province of Catania, is the largest active volcano in Europe. Our aim was to assess the incidence of ALS in the province of Catania during 2005-2010 and its spatial distribution with respect to volcanic gas deposition. METHODS: Cases from all neurological centres of the province of Catania and of the boundary provinces were retrospectively collected. Patients who had onset during 2005-2010 and fulfilled the El Escorial revised diagnostic criteria were included. The incidence of ALS was estimated for the entire province and separately for the population living on the eastern and western flank of Mount Etna, respectively, the most and least exposed areas to volcanogenic TEs, considered as a possible risk factor for ALS. RESULTS: One hundred and twenty-six (57 men) ALS patients were enrolled. The mean annual crude incidence rate was 2.0/100 000 person-years (95% confidence interval 1.7-2.4). A higher incidence rate was found in the population living on the eastern flank compared to the western flank (2.4/100 000 and 0.9/100 000 respectively) with a relative risk of 2.75 (95% confidence interval 1.64-4.89; P < 0.001). CONCLUSIONS: The incidence of ALS in the province of Catania is close to those reported worldwide. The incidence was higher amongst the population living on the eastern flank of Mount Etna, which could be interpreted as a possible role of volcanogenic TEs. Further research on TEs and genetic factors is necessary to support this assumption.