ABSTRACT
INTRODUCTION: Autism and behavioral characteristics in adults with Dravet syndrome (DS) have rarely been systematically studied. METHOD: Three scales were used to assess the outcomes of DS in adulthood in terms of autism and behavior. All the adult patients with DS, nine male and four female, aged between 18 and 60 years, living at the Epilepsy Center Kempenhaeghe in The Netherlands were included in the study. In addition, the past medical history of each patient was systematically screened for diagnoses like autism, Pervasive Development Disorder-Not Otherwise Specified (PDD-NOS), autism spectrum disorder (ASD), hyperactivity, Attention Deficit Hyperactivity Disorder (ADHD), and self-mutilation. Information concerning past and current use of psychoactive drugs was also evaluated. RESULTS: Eight patients (61.5%) were classified as having autism spectrum disorder (ASD) according to the AVZ-R or according to the medical record. Self-mutilation was seen in four patients (30.8%), hyperactivity in none. Three patients (23.1%) currently used psychoactive drugs. CONCLUSION: Autism spectrum disorders persist in adult patients with DS, while certain characteristics associated with behavioral problems, such as hyperactivity or use of psychoactive medication, seem to be less prominent than in childhood.
Subject(s)
Autistic Disorder/diagnosis , Autistic Disorder/epidemiology , Epilepsies, Myoclonic/diagnosis , Epilepsies, Myoclonic/epidemiology , Adolescent , Adult , Attention Deficit Disorder with Hyperactivity/diagnosis , Autism Spectrum Disorder , Child Development Disorders, Pervasive/complications , Diagnostic and Statistical Manual of Mental Disorders , Female , Humans , Male , Middle Aged , Netherlands/epidemiology , Self Mutilation , Young AdultABSTRACT
A large number of patients with epilepsy and intellectual disability take medication, amongst which antiepileptic and psychotropic drugs, often simultaneously. Certain antiepileptic drugs have mood-stabilizing properties, e.g. carbamazepine, valproic acid and lamotrigine. The aim of this study was to investigate whether the use of these mood-stabilizers is associated with a different use of psychotropic drugs in a population of institutionalized epilepsy patients with intellectual disability. We performed a retrospective, cohort study of adults with intellectual disability and epilepsy at the long-stay department of an epilepsy centre in The Netherlands. 246 residents were included. In patients using lamotrigine we found a statistically significant lower use of antidepressants. We also found significant less prescriptions of anxiolytics in patients using AEDs with mood-stabilizing properties (carbamazepine, valproic acid and lamotrigine). When considering the effect of gender, we found that male patients took significantly more antipsychotics. Most important, we found an inverse relation between the drug load of carbamazepine and/or valproic acid and/or lamotrigine and the use of psychotropic drugs. In a population of institutionalized epilepsy patients with intellectual disability, higher drug loads of mood-stabilizing antiepileptic drugs correspond with less use of psychotropic drugs.
Subject(s)
Anticonvulsants/administration & dosage , Epilepsy/drug therapy , Intellectual Disability/drug therapy , Mood Disorders/drug therapy , Psychotropic Drugs/administration & dosage , Adolescent , Adult , Aged , Aged, 80 and over , Antidepressive Agents/administration & dosage , Antipsychotic Agents/administration & dosage , Carbamazepine/administration & dosage , Cohort Studies , Dose-Response Relationship, Drug , Drug Therapy, Combination , Epilepsy/complications , Female , Humans , Intellectual Disability/complications , Lamotrigine , Male , Middle Aged , Mood Disorders/complications , Retrospective Studies , Triazines/administration & dosage , Valproic Acid/administration & dosage , Young AdultABSTRACT
Hypothalamic hamartomas (HH) are developmental malformations that are associated with gelastic seizures, other types of seizures, cognitive decline, and symptoms related to hypothalamic dysfunction. Although aggressive behavior is frequently described, data on the neuropsychiatric profile are limited. In this article, five patients with HH are described who displayed a wide variety of psychiatric symptoms that, dependent on the time frame, met the criteria for several categorical diagnoses. Major neuropsychiatric symptoms comprised aggression that is only partial context dependent, compulsive behavior, psychotic symptoms not responding to treatment, and organic mood instability. HH should therefore be considered a neuropsychiatric syndrome with a highly variable expression that can be best captured by a thorough description of behaviors, symptoms, sequelae of epilepsy, and hypothalamic dysfunction.
Subject(s)
Behavioral Symptoms/etiology , Hamartoma/complications , Hamartoma/psychology , Hypothalamic Neoplasms/complications , Hypothalamic Neoplasms/psychology , Adult , Aggression , Compulsive Behavior , Female , Humans , Male , Middle Aged , Mood DisordersABSTRACT
The incidence of cervical spinal cord injuries (c-SCI) in patients with refractory epilepsy is 30-40 times higher than in the normal population. The injuries occur after seizure-related falls. Risk factors and pitfalls in diagnosis are discussed. Awareness of the risk within this population of developing c-SCI should receive more widely recognition, especially in centres that treat this population.
