Current state of hemispherectomy and callosotomy for pediatric refractory epilepsy in Denmark.

OBJECTIVE: To evaluate outcomes from hemispherectomy and callosotomy related to the need for anti-seizure medication (ASM), seizure frequency, and cognition. METHODS: A review of the medical charts of all Danish pediatric patients who underwent hemispherectomy or callosotomy from January 1996 to December 2019 for preoperative and postoperative ASM use, seizure frequency, and cognitive data. RESULTS: The median age of epilepsy onset was two years (interquartile range (IQR): 0.0-5.3) for the hemispherectomy patients (n = 16) and one year (IQR: 0.6-1.7) for callosotomy patients (n = 5). Median time from onset to final surgery was 3.4 years for hemispherectomy and 10.2 years for callosotomy, while the median follow-up time was 6.9 years and 9.0 years, respectively. Preoperatively, all patients had daily seizures and were treated with ≥ 2 ASM. Hemispherectomy resulted in a reduction in seizure frequency in 87.5 % of patients, with 78.6 % achieving seizure freedom. Furthermore, 81.3 % experienced a reduction in ASM use and 56.3 % stopped all ASM. Median IQ/developmental quotient (IQ/DQ) was low preoperatively (44.0 [IQR: 40.0-55.0]) and remained unchanged postoperatively (IQ change: 0.0 [IQR: -10.0-+4.0]). Callosotomy resulted in a seizure reduction of 86-99 % in four patients, and ASM could be reduced in three patients. Median IQ/DQ was 20.0 preoperatively (IQR: 20.0-30.0) and remained unchanged postoperatively (IQ change: 0.0 [IQR: 0.0]). CONCLUSION: Hemispherectomy and callosotomy result in a substantial reduction in seizure frequency and ASM use without deterioration of IQ. Extensive epilepsy surgery should be considered early in children with drug-resistant epilepsy.

Cerebellar mutism syndrome of non-tumour surgical aetiology-a case report and literature review.

Cerebellar mutism syndrome (CMS) is a well-known complication of posterior fossa (PF) tumour surgery. CMS has previously been reported in cases of non-tumour surgical aetiology in a limited number of publications. We report a case of a 10-year-old girl who suffered a cerebellar haemorrhage and subsequent CMS following surgical treatment of a ruptured arteriovenous malformation (AVM) in the cerebellar vermis. The AVM was removed acutely through a transvermian access, and hydrocephalus was treated with temporary external drainage. In the postoperative period, she suffered diffuse vasospasms of the anterior cerebral circulation and had a permanent shunt placed for hydrocephalus. Her mutism resolved after 45 days but severe ataxia persisted. To our knowledge, this is the first reported case of CMS related to a vermian haemorrhagic stroke with postoperative diffuse vasospasms. Based on this case, we present a literature review on CMS of non-tumour surgical origin in children.

School performance and psychiatric comorbidity in childhood absence epilepsy: A Danish cohort study.

The aim was to determine school performance and psychiatric comorbidity in children with childhood absence epilepsy (CAE). We reviewed the medical records in children with ICD-10 codes for idiopathic generalized epilepsy before 18 years of age, and pediatric neurologists confirmed the International League Against Epilepsy criteria for CAE were met. Control groups were the general pediatric population or children with non-neurological chronic disease. Outcomes were from nationwide and population-based registers on school performance and psychiatric comorbidity. We compared the mean grade point average using linear regression and estimated hazard ratios (HR) using Cox regression for the other outcomes. Analyses were adjusted for the child's sex, and year of birth, and parental highest education, receipt of cash benefits or early disability pension. We included 114 children with CAE with a median age at onset of 5.9 years (interquartile range = 4.5-7.3 years). Compared with both population controls and non-neurological chronically ill children, children with CAE had increased hazard of special needs education (HR = 2.7, 95% confidence interval (CI) = 1.8-4.1, p < 0.0001), lower grade point average at 9th grade by 1.7 grade points (95% CI = -2.5 to -1.0, p < 0.001), increased ADHD medicine use (HR = 4.4, 95% CI = 2.7-7.2, p < 0.001), increased sleep medicine use (HR = 2.7, 95% CI = 1.7-4.3, p < 0.001), and increased psychiatry visits (HR = 2.1, 95% CI = 1.1-4.0, p = 0.03). In conclusion, children with CAE have increased psychiatric comorbidity and a considerable proportion of these children receive special needs education in primary/secondary school, albeit insufficient to normalize their considerably lower grade point average in the 9th grade.

School performance and psychiatric comorbidity in juvenile absence epilepsy and juvenile myoclonic epilepsy: a Danish population-based cohort study.

BACKGROUND: We aimed to determine school performance and psychiatric comorbidity in juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and generalized tonic-clonic seizures (GTCS) alone. METHODS: All children (< 18 years) fulfilled International League Against Epilepsy criteria after review of their medical records. Control groups were the pediatric background population or children with non-neurological chronic disease. Outcomes were on school performance and psychiatric comorbidity. We compared mean grade point averages using linear regression and estimated hazard ratios using Cox regression in the remaining analyses. We adjusted for the child's sex, age, and year of birth; and parental highest education, receipt of cash benefits or early retirement. RESULTS: We included 92 JAE, 190 JME, 27 GTCS alone, 15,084 non-neurological chronic disease controls, and population controls. JAE had two times increased hazard for special needs education compared with age-matched population controls (hazard ratio 2.2, 95% CI = 1.1‒4.6, p = 0.03); this was not seen in JME. Compared with population controls, both JAE and JME had lower grade point average in secondary and high school (JME: 9th grade: - 0.5 points, 95% CI = -0.9 to -0.06, p = 0.03; high school: - 0.6 points, 95% CI = -1.3 to -0.1, p = 0.04), and 8% fewer JME and 15% fewer JAE attended high school. Both JME and JAE had higher hazard for redeeming sleep medication compared with non-neurological chronic disease; additionally, JAE had increased hazard for ADHD medicine redemptions. CONCLUSIONS: Both JAE and JME had marginally poorer school performance; performance seemed worse in JAE than in JME. Both JAE and JME had increased use of sleep medication.

Validation of Pediatric Idiopathic Generalized Epilepsy Diagnoses from the Danish National Patient Register During 1994‒2019.

Objective: To identify pediatric idiopathic generalized epilepsy (IGE) during 1994-2019 using ICD-10 codes in the Danish National Patient Register and anti-seizure prescriptions in the Danish Prescription Database. Study Design and Setting: We reviewed the medical records in children with ICD-10 codes for IGE before 18 years of age, and pediatric neurologists confirmed that the International League Against Epilepsy criteria were met. We estimated positive predictive values (PPV) and sensitivity for ICD-10 alone, including combinations of codes, anti-seizure prescription, and age at first code registration using medical record-validated diagnoses as gold standard. Results: We validated the medical record in 969 children with an ICD-10 code of IGE, and 431 children had IGE (115 childhood absence epilepsy, 97 juvenile absence epilepsy, 192 juvenile myoclonic epilepsy, 27 generalized tonic-clonic seizures alone). By combining ICD-10 codes with antiseizure prescription and age at epilepsy code registration, we found a PPV for childhood absence epilepsy at 44% (95% confidence interval [CI]=34%‒54%) and for juvenile absence epilepsy at 44% (95% CI=36%-52%). However, ethosuximide prescription, age at ethosuximide code registration before age 8 years and a combination of ICD-10 codes yielded a PPV of 59% (95% CI=42%‒75%) for childhood absence epilepsy but the sensitivity was only 17% (20/115 children identified). For juvenile myoclonic epilepsy the highest PPV was 68% (95% CI=62%‒74%) using the code G40.3F plus antiseizure prescription and age at epilepsy code registration after age 8 years, with sensitivity of 85% (164/192 children identified). For generalized tonic-clonic seizures alone the highest PPV was 31% (95% CI=15%‒51%) using G40.3G during 2006-2019 plus antiseizure prescription and age at code registration after age 5 years. Conclusion: The Danish National Patient Register and the Danish Prescription Database are not suitable for identifying children with IGE subtypes, except for juvenile myoclonic epilepsy which can be identified with caution.

Acute flaccid rhombencephalomyelitis with radiculitis in a child with an enterovirus A71 infection seen for the first time in Denmark: a case report.

BACKGROUND: Acute flaccid myelitis is a serious condition of the spinal cord. More than 80% of patients experience a mild respiratory illness or fever consistent with a viral infection prior to acute flaccid myelitis development. Enterovirus A71 is known to circulate in Denmark, and has previously been associated with severe neurological symptoms. In this case report we describe acute flaccid rhombencephalomyelitis with radiculitis in an infant with an enterovirus infection. CASE PRESENTATION: The 8-month-old male of Asian origin presented with fever and gastrointestinal symptoms, followed by severe neurological deficits such as flaccid paralysis of the neck and upper extremities. An initial magnetic resonance imaging scan of the brain was normal, and the boy was treated for encephalitis. A follow-up magnetic resonance imaging scan of the brain and spinal cord 1 week later showed the development of pathological symmetrical gray matter hyperintensity lesions on T2-weighted images in the brainstem and upper medulla spinalis, and nerve enhancement in the terminal thread of the spinal cord and the cervical roots; findings consistent with rhombencephalomyelitis with radiculitis causing flaccid paralysis. Enterovirus A71 was detected in both nasopharyngeal and fecal specimens. Other differential diagnostic etiologies of viral and bacterial encephalitis, including poliovirus, were excluded. CONCLUSIONS: This is the first case in Denmark of a patient diagnosed with acute flaccid rhombencephalomyelitis strongly linked to an enterovirus A71 infection. This case emphasizes the diagnostic importance of combining a history of respiratory and/or gastrointestinal illness, fever, and delayed onset of varying degrees of paralysis with progressive characteristic spinal and brain lesions. Analysis of respiratory, fecal, and cerebrospinal samples for the presence of enterovirus, and eliminating other differential pathogens, is essential to confirm the diagnosis.

Causes of poor eye contact in infants: a population-based study.

BACKGROUND: Establishing eye contact between infants and parents is important for early parent-child bonding and lack of eye contact may be a sign of severe underlying disease. The aim of the study was to evaluate the causes of poor or lacking eye contact in infants. METHODS: Cross-sectional study reviewing all referrals of infants ≤1 year of age from January 1rst, 2016 to December 31rst, 2018. Medical information was retrieved from patient files covering pregnancy, birth, diagnostic work-up and ocular parameters such as refraction, visual acuity and structural findings. RESULTS: We identified 99 infants with poor or lacking eye contact. The relative frequency of causes was neurologic disease 36.4% (36/99), delayed visual maturation 24.2% (24/99), ocular disease 21.2% (21/99) and idiopathic infantile nystagmus 4.0% (4/99). Fourteen infants had a visual function within age-related norms at first examination despite poor eye contact at the time of referral. Of the infants with available data, 18/27 (33.3%) with neurologic cause, 15/23 (65.2%) with delayed visual maturation and 9/21 (42.9%) with ocular cause had visual acuity within the age-related norm at latest follow-up (0-41 months). In 23 infants, a genetic cause was found. CONCLUSION: Poor eye contact in infants may be a sign of severe underlying disease, such as neurological or ocular disease. Close collaboration between pediatric ophthalmologists and neuro-pediatricians are warranted in the management of these infants.

Characteristics of Danish children registered with and pharmacologically treated for hypertension.

INTRODUCTION: A previous study found that 0.04% of Danish children were registered with hypertension, among whom 54% were treated pharmacologically. Our study describes pharmacologically treated cases at the onset of antihypertensive therapy, noting whether the evaluation of target-organ damage could be improved. METHODS: Our review of the medical records of children under 16 years living in Central and Eastern Denmark from April 2014 to May 2015 found that 119 children were registered with an International Classification of Diseases, tenth edition diagnosis of hypertension and treated with antihypertensive medicine. RESULTS: The cohort consisted of 61% boys and 39% girls (p = 0.01). The majority of patients (80%) had secondary hypertension. Renal aetiology was found in 52%. Echocardiography, retinal examination and examination for proteinuria were undertaken in 77%, 74% and 100%, respectively. Both echocardiography and retinal examination were undertaken in 61% of patients with renal aetiology. Among the remaining patients, 95% and 89% underwent these examinations, respectively (p less than 0.001 and p less than 0.001). Abnormal echocardiography, abnormal retinal examination and proteinuria were found in 39%, 16% and 66%, respectively, of patients with renal aetiology and in 30%, 24% and 35% of the remaining patients (p = 0.3, p = 0.4 and p less than 0.001). CONCLUSIONS: Examination for target-organ damage was performed less often in patients with hypertension of renal aetiology than in the remaining patients. Examination for target-organ damage is recommended in all hypertensive children to determine whether treatment is indicated to reduce long-term morbidity. FUNDING: The study received funding from the Novo Nordisk Foundation, grant number NNF15OC0015702 and from Amager-Hvidovre Hospital Foundation. TRIAL REGISTRATION: not relevant.

Poor Head Growth Is Associated with Later Mental Delay among Vietnamese Preterm Infants: A Follow-up Study.

BACKGROUND: Preterm infants in low- and middle-income countries are at high risk of poor physical growth, but their growth data are still scarce. OBJECTIVES: To describe the growth of Vietnamese preterm infants in the first 2 years, and to compare with references: World Health Organization (WHO) child growth standards, and healthy Southeast Asian (SEA) infants. Further, to assess the association between growth in the first year and neurodevelopment at 2 years corrected age (CA). METHODS: We conducted a cohort study to follow up preterm infants discharged from a neonatal intensive care unit for 2 years. Weight, length and head circumference (HC) were measured at 3, 12 and 24 months CA. Neurodevelopment was assessed using Bayley Scales of Infant and Toddler Development-3rd Edition at 24 months CA. RESULTS: Over 90% of the cohort showed catch-up weight at 3 months CA. Weight and length were comparable to healthy SEA but were lower than WHO standards. HC was significantly smaller than those of WHO standards with HC Z-scores steadily decreasing from -0.95 at 3 months CA to -1.50 at 24 months CA. Each one decrement of HC Z-score from 3 to 12 months CA was associated with nearly twice an increase in odds of mental delay at 24 months CA (odds ratio 1.89; 95% confidence interval 1.02-3.50). CONCLUSION: Vietnamese preterm infants exhibited early catch-up weight but poor head growth, which was associated with later delays in mental development. Our findings support the importance of HC measures in follow-up for preterm infants.

Rates of rehospitalisation in the first 2 years among preterm infants discharged from the NICU of a tertiary children hospital in Vietnam: a follow-up study.

OBJECTIVES: To describe the characteristics of rehospitalisation in Vietnamese preterm infants and to examine the time-to-first-readmission between two gestational age (GA) groups (extremely/very preterm (EVP) vs moderate/late preterm (MLP)); and further to compare rehospitalisation rates according to GA and corrected age (CA), and to examine the association between potential risk factors and rehospitalisation rates. DESIGN AND SETTING: A cohort study to follow up preterm infants discharged from a neonatal intensive care unit (NICU) of a tertiary children's hospital in Vietnam. PARTICIPANTS: All preterm newborns admitted to the NICU from July 2013 to September 2014. MAIN OUTCOMES: Rates, durations and causes of hospital admission during the first 2 years. RESULTS: Of 294 preterm infants admitted to NICU (all outborn, GA ranged from 26 to 36 weeks), 255 were discharged alive, and 211 (83%) NICU graduates were followed up at least once during the first 2 years CA, of whom 56% were hospital readmitted. The median (IQR) of hospital stay was 7 (6-10) days. Respiratory diseases were the major cause (70%). Compared with MLP infants, EVP infants had a higher risk of first rehospitalisation within the first 6 months of age (p=0.01). However, the difference in risk declined thereafter and was similar from 20 months of age. There was an interaction in rehospitalisation rates between GA and CA. Longer duration of neonatal respiratory support and having older siblings were associated with higher rehospitalisation rates. Lower rates of rehospitalisation were seen in infants with higher cognitive and motor scores (not statistically significant in cognitive scores). CONCLUSIONS: Hospital readmission of Vietnamese preterm infants discharged from NICU was frequent during their first 2 years, mainly due to respiratory diseases. Scale-up of follow-up programmes for preterm infants is needed in low-income and middle-income countries and attempts to prevent respiratory diseases should be considered.

Increase in cognitive function is seen in many single-operated pediatric patients after epilepsy surgery.

PURPOSE: The recurrent seizures of pediatric drug-resistant epilepsy (DRE) are known to impair brain development and can lead to a loss in cognitive functioning. Surgery is increasingly being used to treat children with DRE. This study investigates the pre- and postoperative cognitive function in a pediatric epilepsy surgery cohort as well as predictive determinants of change in intelligence quotient (IQ) following surgery. METHODS: A consecutive series of 91 Danish children who underwent focal resective epilepsy surgery between January 1996 and December 2016 were included. All underwent preoperative cognitive evaluation and were reevaluated at 1-year and/or 2-year follow-up. Single-operated and multi-operated patients were examined separately. RESULTS: 79 of 91 patients were single-operated. Single-operated patients received less anti-epileptic drugs (AED) and experienced a decrease in seizure frequency postoperatively, p < 0.001. IQ increased postoperatively (IQ change ± standard deviation: 3.3 ± 14.0), p < 0.05. High preoperative seizure frequency was a significant predictor for decreased IQ, p < 0.01. Multi-operated patients did not experience a reduction in AED treatment. Surgery and continued AED treatment did, however, result in significantly better seizure control, p < 0.01. IQ remained unchanged in multi-operated patients. CONCLUSION: Epilepsy surgery allowed for IQ gains in single-operated patients. Preoperative seizure frequency was a significant predictor of IQ change following surgery. Interactions between other, not included, possible predictors remain to be examined. Single-operated patients had the best cognitive outcome. The inclusion of a non-surgical control group is needed to assess the extent of the beneficial effects of surgery on cognitive ability.

Neurodevelopment at 2 years corrected age among Vietnamese preterm infants.

BACKGROUND: Preterm infants are at risk of neurodevelopmental delay, but data on long-term outcomes in low-income and middle-income countries remain scarce. OBJECTIVES: To examine neurodevelopment using Bayley Scales of Infant and Toddler Development-3rd edition (Bayley-III) and neurological findings in 2-year-old preterm infants, and to compare with healthy Vietnamese infants. Further, to assess factors associated with neurodevelopmental impairment. DESIGN AND SETTING: Cohort study to follow up preterm infants discharged from a neonatal intensive care unit (NICU) of a tertiary children's hospital in Vietnam. PARTICIPANTS: Infants born at <37 weeks of gestational age. MAIN OUTCOMES: Bayley-III assessment and neurological examination at 2-year corrected age (CA) compared with healthy Vietnamese infants. RESULTS: Of 294 NICU preterm infants, Bayley-III scores of all 184/243 (76%) survivors at 2 years CA were significantly lower than those of healthy Vietnamese peers in all three domains: cognition (mean (SD): 84.5 (8.6) vs 91.4 (7.5), p<0.001), language (mean (SD): 88.7 (12.5) vs 95.9 (11.9), p<0.001) and motor (mean (SD): 93.1 (9.0) vs 96.8 (9.3), p=0.003). The mean differences in Bayley-III scores between preterm and healthy Vietnamese infants were -6.9 (-9.1 to -4.7), -7.2 (-10.5 to -3.8) and -3.7 (-6.1 to -1.2) for cognitive, language and motor scores, respectively. The prevalence of neurodevelopmental impairment was 17% for cognitive, 8% for language and 4% for motor performance. In total, 7% were diagnosed with cerebral palsy. Higher maternal education was positively associated with infant neurodevelopment (OR 0.32, 95% CI 0.11 to 0.94). CONCLUSIONS: Vietnamese preterm infants in need of neonatal intensive care showed poor neurodevelopment at 2 years. Higher maternal education was positively associated with infant neurodevelopment. Standard follow-up programmes for preterm infants should be considered in low-resource settings.

National data with high validity and completeness showed that only 0.04% of Danish children had been registered with diagnosed hypertension.

AIM: Hypertension has been reported in up to 4.0% of American children, but no national data exist in Europe. We studied the frequency of registered hypertension in Danish children and evaluated the data. METHODS: This 2014-2015 study focused on patients under the age of 16 registered with hypertension in the Danish National Patient Register and 10 paediatric departments in central and eastern Denmark. The diagnoses were coded according to the International Classification of Diseases, 10th revision. All the subjects' medical records were reviewed, including any confirmed diagnoses. RESULTS: There were 55 784 children under 16 in the study regions at 30 April 2014. By May 2015, 222 of those had been registered with hypertension: 200 in the Danish National Patient Register and 191 in the 10 paediatric departments. Their medical records confirmed hypertension in 187 and 185 cases, respectively. The prevalence of registered and confirmed hypertension was 222/553 784 (0.04%). The positive predictive values of hypertension were 187/200 (94%) and 185/191 (97%), and the completeness of diagnosed hypertension was 187/222 (84%) and 185/222 (83%). CONCLUSION: The registers had high-quality positive predictive values and completeness, but only 0.04% of the Danish children were registered with a verified diagnosis of hypertension.

Sodium oxybate (Xyrem) treatment in severely sleep-deprived child with Epstein-Barr virus encephalitis with lesion of sleep-wake regulation system: a case report.

We present the effect of exogenous sodium oxybate (GHB) in a severely tormented boy unable to sleep and unable to be anesthetized due to a lesion in the sleep initiation system involving the tracks between the ventrolateral preoptic nucleus and the reticular system. We bypassed the system by using sodium oxybate's effect on the cortical GHB and GABAB receptors involved in the initiation and maintenance of sleep.

Diagnostic Value of Oligoclonal Bands in Children: A Nationwide Population-Based Cohort Study.

OBJECTIVE: We evaluated the diagnostic value of cerebrospinal fluid oligoclonal bands in individuals less than 18 years of age. METHODS: In a nationwide population-based setting, we retrieved data on 2055 children's oligoclonal band examination, including concordant cerebrospinal fluid biomarkers, during 1994 to 2017. Case ascertainment was by review of medical records and diagnostic codes. We used Fisher's exact test to explore distribution differences of oligoclonal band positivity in acquired demyelinating syndromes (ADS) before and after age 12 years and calculated the sensitivity, specificity, positive predictive value, and negative predictive value of oligoclonal bands to distinguish ADS from the other diagnostic groups. RESULTS: Median age at oligoclonal band examination was 15.2 years (range = 1.8 to 18.0), and 10% had presence of cerebrospinal fluid oligoclonal bands. Oligoclonal band positivity was the highest in ADS (52%), but it was age dependent: 21% in children with ADS before age 12 years and 68% in children aged 12 through 17 years (P < 0.0001) owing to the higher incidence of multiple sclerosis in the latter. Cerebrospinal fluid oligoclonal bands were not predictive of ADS before age 12 years compared with the other diagnostic groups. However, cerebrospinal fluid oligoclonal bands in children aged 12 through 17 years were highly predictive of ADS compared with central nervous system infections and non-ADS immune-mediated central nervous system diseases (positive predictive value: 0.89; 95% confidence interval = 0.82 to 0.94; P < 0.0001), but negative oligoclonal bands were not discriminatory (negative predictive value: P = 0.17). CONCLUSIONS: In a clinical setting, cerebrospinal fluid oligoclonal band examination may be of higher yield in children aged 12 through 17 years if there is clinical suspicion of multiple sclerosis, and in such circumstances a positive test supports a diagnosis of multiple sclerosis.

Pediatric autoimmune encephalitis in Denmark during 2011-17: A nationwide multicenter population-based cohort study.

BACKGROUND: The incidence of pediatric autoimmune encephalitis (AIE) is unknown. Our aim was to assess the incidence of pediatric AIE in Denmark 2011-17. METHODS: In a nationwide population-based setting, we retrieved data on all children tested for AIE before age 18 years. We reviewed medical records in a) children with AIE antibodies (n = 18) to assess whether children fulfilled the AIE consensus criteria, b) children tested negative for AIE antibodies who were registered with an AIE diagnostic code to estimate the incidence of "antibody negative but probable AIE", and c) a reference cohort (n = 596) to determine the positive predictive value of International Classification of Diseases (ICD) codes used for anti-NMDAR encephalitis. RESULTS: 375 children were tested for AIE 2011-17 (median age 11.1 years; 54% girls); 18 children (5%) had AIE antibodies (percentage tested positive): CSF GAD65-IgG (3.1%), plasma NMDAR-IgG (2.8%), CSF NMDAR-IgG (1.8%), plasma GAD65-IgG (1.0%), and plasma CASPR2-IgG (0.4%). Five children fulfilled the criteria for probably/definite anti-NMDAR encephalitis (incidence: 0.07/100,000 person-years; 95% CI = 0.03-0.17), and 4 children with anti-GAD65 associated AIE (incidence = 0.055/100,000 person-years, 95% CI = 0.021-0.15). The incidence of "antibody negative but probable AIE" was 0.055/100,000 person-years (95% CI = 0.021-0.15). The positive predictive value of ICD diagnostic codes used for anti-NMDAR encephalitis was 8%. CONCLUSIONS: We diagnosed only children with anti-NMDAR, anti-GAD65, and "antibody negative but probable AIE". Before examining AIE antibodies, clinical presentation, paraclinical studies (CSF, EEG, and MRI), and incidence of pediatric AIEs should be considered. Updating the ICD to include AIE codes is warranted.

Atrial standstill presenting as cerebral infarction in a 7-year-old girl.

Atrial standstill is a rare arrhythmia defined by the absence of mechanical and electrical activity in the atria. Few cases of atrial standstill have been described in children, none of which have presented with cerebral infarction confirmed by imaging. We report a unique case of a 7-year-old girl presenting with expressive aphasia, central facial palsy and irregular pulse with cerebral infarction secondary to atrial standstill. This case illustrates that cardiogenic cerebral embolism in children can be caused by rare conditions like atrial standstill and should be considered in paediatric patients undergoing evaluation for stroke. There are no established treatment guidelines for atrial standstill. We recommend that treatment be directed towards any potential underlying cause. All patients with atrial standstill should receive long-term oral anticoagulation treatment and a permanent cardiac pacemaker implant to reduce the risk of further strokes or other cardiac events.

Infant botulism in Denmark from 1995 to 2015.

INTRODUCTION: Infant botulism is a rare, probably underdiagnosed, life-threatening disease caused by the toxin-producing bacterium Clostridium botulinum. METHODS: We investigated reported cases of infant botulism in Denmark from 1995 to 2015, and compared the incidence with that of other western countries. RESULTS: We found nine cases of infant botulism in Denmark from 1995 to 2015. The incidence of infant botulism in Denmark was similar to that seen in other western countries, but likely underestimated. The source of infection was only confirmed in a single case. Four cases received botulism immune globulin, intravenous (BIG-IV), which was administered within 7-10 days from admission. CONCLUSIONS: Infant botulism is probably underdiagnosed. Increased awareness is crucial to be able to recognise the condition. The time delay from hospital admission to administration of BIG-IV is challenging. Studies have shown that BIG-IV reduces both mean duration of intensive care and mean length of hospital stay; however, this was not confirmed in our work due to the limited number of patients. FUNDING: none. TRIAL REGISTRATION: not relevant.