Epileptological aspects of juvenile neuronal ceroid lipofuscinosis (CLN3 disease) through the lifespan

Purpose Juvenile neuronal ceroid lipofuscinosis (CLN3 disease) is the most common neurodegenerative disorder in childhood with survival until young adult age. Visual loss is followed by epilepsy, cognitive, neuropsychiatric, and motor symptoms. We have studied the evolution of electroencephalographic (EEG) and seizure characteristics. Methods Twenty-four patients were recruited via the Norwegian CLN3 disease parent association. Parents were interviewed. Medical records and EEG reports/recordings were collected. Electroencephalographic elements were classified according to Standardized computer-based organized reporting of EEG (SCORE). The evolution of EEG features along with seizure types was assessed by testing the difference in proportions with standardized normal deviate comparing findings below and above 15 years of age. Results Mean age at study or death (n = 12) was 21.2 (10–39) years. Twenty-two patients had experienced seizures; the first was usually bilateral tonic–clonic (TC). Later, focal motor seizures frequently occurred, often with increasing multifocal and polymorphic features. Paroxysmal nonepileptic motor and autonomous symptoms were also suspected in several patients. Distinct myoclonic seizures were uncommon. In four patients, we identified episodes of bradycardia/sinus arrest. Electroencephalography showed progressive slowing of the background activity (p = 0.029). Focal epileptiform discharges were rare and mainly seen at age < 10. Combined multifocal and bilateral epileptiform discharges increased in adolescence (p = 0.002). Conclusion Seizure and EEG characteristics change with time in CLN3 disease. Tonic–clonic seizures are common at onset, and multifocal motor seizures increase with age. In contrast, focal epileptiform abnormalities are more common in childhood, compared to later multifocal and bilateral discharges. This seizure disorder belongs to the combined generalized and focal epilepsies. Paucity of myoclonic seizures does not warrant classification as a classic progressive myoclonic epilepsy. When attacks with only behavior arrest occur, cardiac conduction abnormalities should be considered

Associations between parental alcohol problems in childhood and adversities during childhood and later adulthood: a cross-sectional study of 28047 adults from the general population

Abstract Background Adverse childhood experiences (ACE) are related to adverse physical and mental health outcomes. However, few larger studies based on a general population sample with age groups ranging from young adults to elderly have investigated whether parental alcohol problems increase the risk of offspring subjective reports of ACE both during childhood and current adult adversities. The purpose of this study was to examine the associations between parental alcohol problems and adversities during childhood and later in adulthood. Methods The 28,047 respondents were adults (> 18 years old) from the general population who participated in the Norwegian Counties Public Health Survey. The study had a cross-sectional design and included respondents’ evaluations of childhood experiences and current adult adversities. The short version of the Children of Alcoholics Screening Test (CAST-6, cut-off ≥3) measured parental alcohol problems. Multivariable logistic regression was adjusted for gender, age, and education. Results Growing up with parental alcohol problems strongly increased the risk of experiencing a dysfunctional family environment during childhood (odds ratio [OR] 6.84; 95% confidence interval [CI] 6.36–7.36), perceiving childhood as difficult (OR 5.01; 95% CI 4.58–5.49), and reporting a lack of support from a trusted adult (OR 3.07; 95% CI 2.86–3.29). Parental alcohol problems were associated with a modestly increased risk of harmful alcohol use (OR 1.38; 95% CI 1.29–1.48), but the association with struggling with bad memories was strong (OR 4.56; 95% CI 4.17–4.98). Conclusions Parental alcohol problems increased the risk of offspring experiencing adversities during both childhood and adulthood. Providing supportive services to these children and their families and addressing this issue as part of treatment is important to prevent alcohol related harm

Anxiety, depression and behavioral problems among adolescents with recurrent headache: the Young-HUNT study

Background It is well documented that both anxiety and depression are associated with headache, but there is limited knowledge regarding the relation between recurrent primary headaches and symptoms of anxiety and depression as well as behavioral problems among adolescents. Assessment of co-morbid disorders is important in order to improve the management of adolescents with recurrent headaches. Thus the main purpose of the present study was to assess the relationship of recurrent headache with anxiety and depressive symptoms and behavioral problems in a large population based cross-sectional survey among adolescents in Norway. Methods A cross-sectional, population-based study was conducted in Norway from 1995 to 1997 (Young-HUNT1). In Young-HUNT1, 4872 adolescents aged 12 to 17 years were interviewed about their headache complaints and completed a comprehensive questionnaire that included assessment of symptoms of anxiety and depression and behavioral problems, i.e. conduct and attention difficulties. Results In adjusted multivariate analyses among adolescents aged 12–14 years, recurrent headache was associated with symptoms of anxiety and depression (OR: 2.05, 95% CI: 1.61-2.61, p < 0.001), but not with behavioral problems. A significant association with anxiety and depressive symptoms was evident for all headache categories; i.e. migraine, tension-type headache and non-classifiable headache. Among adolescents aged 15–17 years there was a significant association between recurrent headache and symptoms of anxiety and depression (OR: 1.64, 95% CI: 1.39-1.93, p < 0,001) and attention difficulties (OR: 1.25, 95% CI: 1.09-1.44, p =0.001). For migraine there was a significant association with both anxiety and depressive symptoms and attention difficulties, while tension-type headache was significantly associated only with symptoms of anxiety and depression. Non-classifiable headache was associated with attention difficulties and conduct difficulties, but not with anxiety and depressive symptoms. Headache frequency was significantly associated with increasing symptoms scores for anxiety and depressive symptoms as well as attention difficulties, evident for both age groups. Conclusions The results from the present study indicate that both anxiety and depressive symptoms and behavioral problems are associated with recurrent headache, and should accordingly be considered a part of the clinical assessment of children and adolescents with headache. Identification of these associated factors and addressing them in interventions may improve headache management