Comparing Seizure-Related Death and Suicide in Younger Adults with Epilepsy.

Younger adults with epilepsy have an increased mortality. Some deaths are seizure-related, for example, sudden unexpected death in epilepsy (SUDEP), whereas others, for example, suicide, have multiple causes, including adverse effects of the treatment on mood. In this retrospective population-based study of all Danish persons with epilepsy aged 18 to 49 years during 2007 to 2009 we evaluated the risk of death from seizures and suicide. SUDEP comprised 82.7% of all seizure-related death. Younger adults with epilepsy had an 8.3-fold increased risk of death from seizure-related causes compared with suicide. This underpins the importance of effective seizure control in preventing premature death. ANN NEUROL 2021;90:983-987.

Sudden unexpected death in epilepsy in persons younger than 50 years: A retrospective nationwide cohort study in Denmark.

OBJECTIVE: Persons with epilepsy have an increased mortality including a high risk of sudden unexplained death (SUD), also referred to as sudden unexpected death in epilepsy (SUDEP). We aimed to evaluate the risk of SUDEP in comparison to other causes of death and the risk of SUD in persons with and without epilepsy. METHODS: We undertook a retrospective population-based cohort study of all Danish citizens with and without epilepsy aged 1-49 years during 2007-2009. All deaths in the population were evaluated, and all cases of SUD identified. Primary causes of death in persons with epilepsy were evaluated independently by three neurologists and one neuropediatrician, using the unified SUDEP criteria. RESULTS: The three most frequent causes of death in persons with epilepsy were cancer (2.38 per 1000 person-years), SUDEP (1.65 per 1000 person-years), and pneumonia (1.09 per 1000 person-years) compared with cancer (.17 per 1000 person-years), accident-related deaths (.14 per 1000 person-years), and cardiovascular disease (.09 per 1000 person-years) in persons without epilepsy. Considering definite, definite plus, and probable cases, the SUDEP incidence was .27 per 1000 person-years (95% confidence interval [CI] = .11-.64) in children aged 1-17 years and 1.21 per 1000 person-years (95% CI = .96-1.51) in adults aged 18-49 years. Adjusted for age and sex, persons with epilepsy younger than 50 years had a 10.8-fold (95% CI = 9.97-11.64, p < .0001) increased all-cause mortality and a 34.4-fold (95% CI = 23.57-50.28, p < .0001) increased risk of SUD compared with persons without epilepsy. SUDEP accounted for 23.3% of all SUD. SIGNIFICANCE: This nationwide study of all deaths in persons with epilepsy younger than 50 years found a lower SUDEP risk in children compared with adults, and that epilepsy was a major risk factor for SUD in the background population. This underlines the importance of addressing risk factors for SUDEP to prevent premature death.

Implications of the International Paediatric Multiple Sclerosis Study Group consensus criteria for paediatric acute disseminated encephalomyelitis: a nationwide validation study.

AIM: The International Paediatric Multiple Sclerosis Study Group (IPMSSG) has proposed criteria for acute disseminated encephalomyelitis (ADEM) not evaluated in clinical practice. Our objective was to assess epidemiological implications of the IPMSSG criteria for ADEM in a cohort study using prospectively collected data. METHOD: We identified all diagnosed cases of ADEM in Denmark between 2008 and 2015 from the Danish National Patient Register by International Classification of Diseases 10 codes assigned to acute demyelinating episodes, and we reviewed all medical records to validate ADEM. RESULTS: We found 52 children up to the age of 18 years with a verified clinical diagnosis of ADEM (incidence rate 0.54/100 000 person-years; all had abnormal brain magnetic resonance imaging). Only 18 (35%) fulfilled the IPMSSG criteria regarding encephalopathy and polyfocal neurological deficits. Among all 52 children with ADEM, 33 per cent had clinical sequelae after a median follow-up of 4 years 6 months (range: 10mo-8y 3mo). Surprisingly, none progressed to multiphasic ADEM or multiple sclerosis, but median age at end of follow-up was only 10 years 9 months (range: 2y-24y 3mo). INTERPRETATION: Among 52 children with ADEM, none converted to multiphasic ADEM or multiple sclerosis (median follow-up: 4y 6mo; range: 10mo-8y 3mo). Applying the IPMSSG criteria to all children with a diagnosis of ADEM leaves 65 per cent of the cases without a diagnosis and lowers the incidence rate of paediatric ADEM. WHAT THIS PAPER ADDS: The incidence of paediatric acute disseminated encephalomyelitis (ADEM) was 0.54 per 100 000 person-years in children younger than 18 years. Only 35 per cent of children with ADEM fulfilled the International Paediatric Study Group consensus criteria. ADEM in clinical practice was primarily based on magnetic resonance imaging findings. Paediatric neurologists diagnosed ADEM in the absence of encephalopathy. None of the children with ADEM progressed to multiple sclerosis/multiphasic ADEM during follow-up.

Children and adolescents previously treated with glucocorticoids display lower verbal intellectual abilities.

AIM: Perinatal exposure to glucocorticoids has been associated with adverse cerebral effects, but little is known about their effect on cognitive development and exposure later in childhood. This study examined intellectual abilities, memory and behavioural problems in children previously treated with glucocorticoids. METHODS: We evaluated 38 children aged from seven to 16 years, who had been treated with glucocorticoids for rheumatic disease or nephrotic syndrome, together with 42 healthy controls matched for age, gender and parental education. The median cumulative dose of prednisolone equivalents was 158 mg/kg (range 21-723) and the mean time that had elapsed since treatment was three-and-a-half (standard deviation 2.2) years. Intellectual abilities were assessed with the Wechsler Intelligence Scale for Children and memory performance and behavioural problems with a pattern recognition memory task and the Child Behaviour Check List. RESULTS: There were no significant differences between the groups in pattern recognition memory, perceptual organisation index or behavioural problems, but patients had a significantly lower verbal comprehension index and this difference was present in both disease groups. There were no significant dose-response relationships regarding verbal intellectual abilities. CONCLUSION: Children and adolescents previously treated with glucocorticoids seemed to have lower intellectual verbal abilities than healthy controls.

[Genetic causes of infantile spasms--a systematic review]. / Genetisk udredning ved infantile spasmer.

Infantile spasms are a symptom of a severe epileptic encephalopathy. It is important to determine the aetiology for a child's disease. When a standard programme for evaluating the aetiology of the infantile spasms is unsuccessful genetic causes should be considered. We suggest array CGH as the first-line analysis and present an overview of relevant present possibilities for genetic testing.

Reliability of GMFCS family report questionnaire.

PURPOSE: To examine the reliability of the web-based GMFCS Family Report Questionnaire (GMFCS-FR) between 8 and 11 years old children, compared with the GMFCS-Expanded and Revised (GMFCS-E&R). METHOD: The GMFCS-FR was translated from the English GMFCS-FR into Danish after the CanChild guidelines; only the order of levels was chosen like in the GMFCS-E&R. Families of 30 children with spastic and dystonic cerebral palsy (age from 8 to 11 years, randomly selected from a cerebral palsy register) answered the GMFCS-FR and were later interviewed by two physiotherapists. Participants and non-responders were compared on basic parameters available from the Danish CP register. Inter-rater agreement and weighted κ was calculated in order to compare the translated GMFCS-FR with physiotherapist's applied GMFCS-E&R. RESULTS: The inter-rater agreement between the GMFCS-FR in Danish and the GMFCS-E&R was high (76%) and misclassification was minimal. There was a good agreement on the same or nearby levels (weighted κ = 0.76 and 0.81). The family rated the same or less ability, when compared with trained physiotherapists. CONCLUSION: The GMFCS-FR is a reliable tool for GMFCS evaluation among 8-11 years old Danish children with CP. The tendency for less-ability rating by families is important when performing and comparing results from epidemiological studies based on GMFCS-FR and GMFCS-E&R.

[Intrathecal baclofen in the treatment of severe spastic tetraplegia and dystonia in children and adolescents]. / Intratekal baclofenbehandling ved svaer spastisk tetraplegi og dystoni hos børn og unge.

INTRODUCTION: Continuous intrathecal baclofen has been used over the past years especially in adult patients with spasticity of spinal origin. Children and young adults with severe spasticity and dystonia of cerebral origin are difficult to treat in spite of optimal systemic antispasmotic therapy with baclofen, tizanidine, dantrolene and/or diazepam. Intrathecal baclofen has therefore been applied in a group of young patients. MATERIAL AND METHODS: Eight children and young adults from East Denmark with spasticity and 12 with dystonia aged 3-18 years (median 10.9 years) were tested, operated and treated with continuous intrathecal baclofen for a period of 2-64 months (median 22.2 months). Registration of efficacy, fillings, adjustments of baclofen and other therapies were performed in an out patient setting since 1995. RESULTS: Spasticity in lower extremities was reduced from Ashworth score 3.5-4.5 (median 4.2) to Ashworth score 2.5-4.0 (median 2.9; p < 0.001) during infusion with baclofen 5-33 micrograms/kg/24 hours (median 19 micrograms/kg/24 hours). The infusion catheter tip was placed at levels Th1-Th12 (median Th7.5). Peroral baclofen was reduced from an average of 5.0 to 0.44 mg/kg/24 hours, tizanidine from 0.4 to 0.1 mg/kg/24 hours, and dantrolene from 4.0 to 0.4 mg/kg/24 hours. After initial adjustments successively increased dosages of average 0.46 microgram/kg/month were needed to maintain the same level of efficacy. In questionnaires parents or guardians rated less spasticity in lower extremities in 15 out of 19 patients, and less pain in 13 out of 19 patients. CONCLUSION: Continuous intrathecal baclofen was effective in treating severe spasticity and dystonia of cerebral origin with major effect on muscles of the lower extremities, pelvis, and back and in particular opisthotonus was relieved. Efficacy on upper extremities was far less pronounced.

[Epilepsy surgery of Danish children 1996-2002]. / Epilepsikirurgisk behandling af danske børn 1996-2002.

INTRODUCTION: Epilepsy surgery is done in order to obtain seizure freedom or to reduce the number of epileptic seizures in selected patients not responding to medical treatment. Few Danish children have had this treatment, most probably because of some restrictions imposed by The Danish National Health Board. We present the results of the first 22 Danish children who have had epilepsy surgery. MATERIAL AND METHODS: All children up to the age of 15 operated from January 1st 1996 to May 1st 2002 are included. According to the guidelines of The Danish National Health Board, 17 children have been operated abroad because the epileptic focus was extratemporal or intracranial EEG-monitoring was necessary. Follow-up varied from five years and six months to one month. RESULTS: At the time of the operation, all children had a long-lasting medical intractable epilepsy. 18 children had daily seizures and 15 were mentally retarded. Multi-lobar resection was performed in ten, hemispherectomy in three and lobar resection in eight children. Total seizure-free outcome was seen in 12 children, whereas three only had simple partial seizures (totally 68 per cent). The remaining children had at least a 50 per cent seizure reduction. On an average, the cognitive function was unchanged one year after the surgery. The majority of the parents stated that the behaviour of the child had improved on one or more specific issues. One mother stated that to a certain degree she regretted the operation. In four children, an unexpected complication was registered (slight hemiparesis and a hemianopsia). DISCUSSION: The results documented that epilepsy surgery is effective for selected children in spite of long-lasting severe epilepsy. In order to alleviate the consequences of severe epilepsy it is, however, important to refer the patients early. There seems to be a need for more and early referrals for childhood epilepsy surgery in Denmark.

[Survival of children born with cerebral palsy. Children born 1971-1986]. / Overlevelse for børn født med cerebral parese. Fødselsårgangene 1971-1986.

INTRODUCTION: Since 1967, children with cerebral palsy in Eastern Denmark have been registered in the Danish Cerebral Palsy Registry. We wanted to investigate the survival of Danish patients with cerebral palsy and examine different factors that influence on survival. We also compared the survival rate with that of the background population. MATERIAL AND METHODS: All patients in the Cerebral Palsy Register, born between 1971 and 1986, were included in our study cohort. We examined the influence of the following factors on the survival of persons with cerebral palsy: sex, epilepsy, type of cerebral palsy, mental retardation, functional disability, birth year, and birth weight. Each factor was tested with Cox regression. RESULTS: Seventy-seven of the 1093 persons included (7%), died before September 1997. Our study showed that survival was associated with the type of cerebral palsy, epilepsy, mental retardation, and the degree of functional disability. Surprisingly, birth year and birth weight had no influence on survival. Persons with cerebral palsy had a lower survival rate than the background population. However, there was no significant difference between cerebral palsy persons with walking abilities, and the background population. DISCUSSION: Compared to earlier studies, the survival rate seems to have improved. The Danish cerebral palsy patients had a survival as good as, and maybe even better, than that of cerebral palsy patients from other countries. Persons with low functional disabilities had a survival rate comparable to that of the background population, a finding that may be relevant in regard to life insurance.