Distinct phenotypes of multisystem inflammatory syndrome in children: a cohort study

To characterize the clinical features and outcomes of childhood-onset primary Sjögren’s syndrome (pSS). Patients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria. The electronic medical records of patients with pSS from 2013 to 2020 were collected and analysed. Thirty-nine patients were included. Of them, 27 (69.2%), 38 (97.4%) and 35 (89.7%) patients fulfilled the AECG criteria, ACR/EULAR criteria and proposed juvenile pSS criteria, respectively. The female:male ratio was 3.9:1. The median ages at first signs or symptoms and at diagnosis were 9.2 (4.7, 14.5) years and 10.9 (6.3, 15.0) years, respectively. The main clinical manifestations were rash or purpura (20, 51.3%), followed by fever (12, 30.8%), glandular enlargement/recurrent parotitis (10, 25.6%), and dry mouth and/or dry eyes (9, 23.1%). Twenty-eight (56.4%) patients had systemic damage, the most common of which was haematological involvement (14, 35.9%), followed by hepatic (13, 33.3%) and renal involvement (8, 20.5%). Thirty-eight (97.4%) patients underwent labial minor salivary gland biopsy, and all exhibited focal lymphocytic sialadenitis. All patients had a global ESSDAI score ≥ 1 at diagnosis, and the median total score at diagnosis was 8 (2, 31). Thirty-six (92.3%) patients were followed up for a median time of 23.6 (7.9, 79.5) months, and three patients developed systemic lupus erythematosus (SLE) at follow-up times of 13.3, 38.8 and 63.8 months. The presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS. Labial salivary gland biopsy is vital for patients with probable pSS. Some patients may develop SLE over time.     

Sleep disturbances in children with epilepsy compared with their nearest-aged siblings

The aim of the study was to compare sleep patterns in children with epilepsy with those of their non-epileptic siblings and to determine which epilepsy-specific factors predict greater sleep disturbance. We conducted a case-control study of 55 children with epilepsy (mean age 10y, range 4 to 16y; 27 males, 28 females) and their nearest-aged non-epileptic sibling (mean age 10y, range 4 to 18y; 26 males, 29 females). Epilepsy was idiopathic generalized in eight children (15%), symptomatic generalized in seven (13%), and focal in 40 (73%); the mean duration was 5 years 8 months. Parents or caregivers completed the Sleep Behavior Questionnaire (SBQ) and Child Behavior Checklist (CBCL) for patients and controls, and the Quality of Life in Childhood Epilepsy (QOLCE) for patients. Patients had a higher (more adverse) Total Sleep score (p<0.001) and scored worse than controls on nearly all subscales of the SBQ. In patients, higher Total Sleep scores were correlated with higher scores on the Withdrawn, Somatic complaints, Social problems, and Attention subscales of the CBCL, and significantly lower Total Quality of Life Scores. Refractory epilepsy, mental retardation, and remote symptomatic etiology predicted greater sleep problems in those with epilepsy. We conclude that children with epilepsy in this current study had significantly greater sleep problems than their non-epileptic siblings.     

Seizure-alerting and -response behaviors in dogs living with epileptic children

There is little evidence for the ability of dogs to anticipate human seizures. Families of epileptic children were surveyed to investigate seizure-related behaviors in dogs. Approximately 40% of families owned a dog, about 40% of these had seizure-specific behavior, and about 40% of these (approximately 15% overall) showed anticipatory ability. Anticipation occurred early and was both sensitive and specific. Quality of life was higher in families with a dog that responded to seizures.     

Family function in cognitively normal children with epilepsy: impact on competence and problem behaviors

A cross-sectional cohort of 82 cognitively normal children with epilepsy attending the pediatric neurology clinic, who were aged 6-17 years and who had a similarly aged sibling without seizures or cognitive delay, were identified. The parent was asked to complete the Family Assessment Measure III (FAM-III) as well as the Child Behavior Checklist (CBCL) for both the child with epilepsy and his or her sibling. The Overall Rating Score on FAM-III did not differ significantly from the normative mean, although families scored significantly better on the Involvement subscale and significantly worse on the Role Performance subscale. Moderate correlations were found between competence and behavior problems and family function in both children with epilepsy and their siblings. In families functioning at the highest level, both cohorts did well. In those at the lowest level, approximately half of the children in either cohort had problems. For average functioning families, behavior and competence issues were more frequent in children with epilepsy than their siblings.     

Depression, anxiety, and quality of life in siblings of children with intractable epilepsy

Thirty-seven siblings (aged 6-18) of children with intractable epilepsy were surveyed regarding their anxiety, depression, and quality of life, by both self-report (Revised Children's Manifest Anxiety Scale, RCMAS; Children's Depression Inventory, CDI; and Peds QL) and parental report (Child Behavior Checklist, CBCL). Completed forms were returned by 37 of 58 (64%) eligible families. No sibling had a score in the clinical range on the CDI, and only 2 of 37 (6%) scored in the clinical range on the RCMAS, a proportion similar to the normative population. In contrast, 25% were rated by their parents to have elevated Internalizing Behaviors scores on the CBCL. There was a trend for PedsQL scores to be lower than the normative mean. Comorbid behavior and attention problems in the child with epilepsy and sibling CDI and RCMAS scores correlated significantly with self-reported quality of life. However, other epilepsy, child, and family variables (income, parental education, family function, maternal depression) did not correlate. We conclude that siblings of children with intractable epilepsy are functioning well overall and have a good quality of life.     

Deterioration in cognitive function in children with benign epilepsy of childhood with central temporal spikes treated with sulthiame

The purpose of this study was to determine if reduction in spike frequency in children with benign epilepsy of childhood with centrotemporal spikes treated with sulthiame correlates with improved neuropsychologic function. Six untreated children (5 boys, 1 girl) with a mean (standard deviation) age of 9.1 years (1.5 years) underwent overnight ambulatory electroencephalograph recording and neuropsychologic evaluation at baseline and after 6 months of sulthiame monotherapy. The Reliable Change Index was used to determine whether a statistically reliable change in neuropsychologic function occurred. All children continued receiving sulthiame monotherapy (dose range, 8.0-9.3 mg/kg per day) for the study period without further seizures. Although spike frequency was reduced in all 6 children (3 showing a decrease to less than 10% of baseline), they all demonstrated significant deterioration in their reading ability, 5 had significant declines in general memory, and more than half had reduction in attention skills and mathematics ability. Although sulthiame was effective at seizure control and significantly reduced spike frequency in children, it was also associated with deterioration in cognitive function.     

Peri-ictal headache in children: prevalence and character

Structured interviews regarding peri-ictal headaches and personal or family histories of interictal headaches were conducted on 101 children (aged 5-18 years), with generalized tonic-clonic or partial seizures. Epilepsy-specific details were collected by interviews and reviews of neurology clinic charts. Peri-ictal headaches were reported by 41% (29%, postictal only; 5%, preictal only; 7%, both). Clear migrainous features were present in 50% of preictal and 58% of postictal headaches. Most children described bilateral headaches. No demographic or epilepsy-specific correlates were identified that predicted peri-ictal headaches. Interictal headaches occurred in 24%, with 14% of children meeting criteria for migraines. However, neither interictal migraines nor a positive family history of migraines was significantly predictive of either peri-ictal headaches or migrainous peri-ictal headaches. Postictal headaches occurred reliably after most seizures in predisposed children, and interrupted activities in the majority. Whereas only half of children received abortive medications for these headaches, simple analgesics were effective in most cases. We conclude that peri-ictal headaches are common, affecting 41% of children with epilepsy. Their presence should be routinely queried, and if documented, treatment with simple analgesics appears beneficial and should be considered.