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INTRODUCTION: The aim of the ECOMRAID trial (Epileptic seizure related Complication RAte in residential population of persons with epilepsy and Intellectual Disability) was to study seizure-related complications (status epilepticus, respiratory complications, or other severe complications) in people with epilepsy and intellectual disability living in a residential setting. The results of the present study are a prerequisite for performing a prospective study into the effectiveness of nocturnal surveillance patients with high risk for Sudden unexpected death in epilepsy (SUDEP). MATERIAL AND METHODS: A retrospective study was conducted in three general residential care institutions and one residential specialized epilepsy clinic. In this 5-year cohort, we collected the following data: age (at inclusion and in case of death), sex, type of residential care, different types of complications, rescue/emergency medication administration, transfers to another department (internal midcare / monitoring unit or general hospital) and a self-designed SUDEP risk score. Our primary research questions were to assess the number of patients who experienced seizure-related complications and their individual complication rates. The secondary research questions were to document the relationship of these complications with the SUDEP risk score, with the type of residential living, and with the frequency of interventions by caregivers. RESULTS: We included 370 patients (1790 patient-years) and in 135 of them, we found 717 seizure-related complications. The following complication rates were found: all complications: at 36%, status epilepticus: at 13%, respiratory complications: at 5%, and other complications at 26%. In residential care institutions, we found fewer patients with complications compared to the specialized epilepsy clinic (all complications 24% vs 42%, OR 0.44, pâ¯<â¯0.01; status epilepticus 5% vs 17%, OR 0.27, pâ¯<â¯0.01; other: complications 19% vs 30%, OR 0.56, pâ¯<â¯0.05). In residential care institutions, we found more "other complications" than in the specialized epilepsy clinic (89% vs 71%, OR 3.13, pâ¯<â¯0.0001). The annual frequency of all complications together was higher in residential care institutions (range 0 to 21 vs 0 to 10, pâ¯<â¯0.05). Rescue medication was given to 75% of the patients, but more often in the specialized epilepsy clinic (median 2.6 vs 0.5 times/patient/year, pâ¯<â¯0.001). In the specialized epilepsy clinic, more patients were transferred to a midcare / monitoring unit or general hospital (56% vs 9%, OR 13.44, pâ¯<â¯0.0001) with higher yearly frequencies (median 0.2 vs 0.0, pâ¯<â¯0.001). There were no reported cases of SUDEP. The median SUDEP risk score was higher in the specialized epilepsy clinic (5 vs 4, pâ¯<â¯0.05) and was weakly correlated with the status epilepticus (ρâ¯=â¯0.20, pâ¯<â¯0.001) and (total) complication rate (ρâ¯=â¯0.18, pâ¯<â¯0.001). CONCLUSION: We found seizure-related complications in more than one-third of the patients with epilepsy and intellectual disability living in a residential setting over a period of 5â¯years. The data also quantify seizure-related complications in patients with epilepsy and intellectual disability.
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Epilepsia , Deficiência Intelectual , Estado Epiléptico , Morte Súbita Inesperada na Epilepsia , Humanos , Morte Súbita/epidemiologia , Epilepsia/complicações , Epilepsia/epidemiologia , Deficiência Intelectual/complicações , Deficiência Intelectual/epidemiologia , Estudos Prospectivos , Estudos Retrospectivos , Fatores de Risco , Convulsões/complicações , Convulsões/epidemiologia , Estado Epiléptico/complicaçõesRESUMO
BACKGROUND: Long-term use of antiseizure drugs is associated with a low bone mineral density (BMD) and an increased fracture risk. The literature regarding institutionalised children on chronic antiseizure drugs is limited. Therefore, the aim of this cross-sectional study is to evaluate the prevalence of low BMD and the history of fractures in institutionalised children with epilepsy and intellectual disability (ID). METHODS: A dual-energy X-ray absorptiometry of lumbar spine (L1-L4) and hip was performed in 24 children, residing in a long-stay care facility in the Netherlands. Additionally, serum concentrations of albumin, calcium and 25-hydroxyvitamin D were determined. Data on fractures were retrospectively extracted from the medical files. RESULTS: Ages of the children (14 male and 10 female) ranged from 5 to 17 years with a mean age of 13.0 (±3.2). The criteria of the International Society for Clinical Densitometry (ISCD) were used for classification of bone mineral disorders. Eight (33.3%) children had a normal BMD (Z-score > - 2.0). Of the 16 children with a low BMD (Z-score ≤ - 2.0), three were diagnosed as osteoporotic, based on their fracture history. Ten children (41.7%) were reported to have at least one fracture in their medical history. Serum concentrations of albumin-corrected calcium (2.28-2.50 mmol/L) and (supplemented) vitamin D (16-137 nmol/L) were within the normal range. CONCLUSIONS: This study demonstrated that 67% of institutionalised children with epilepsy and ID had low BMD and 42% had a history of at least one fracture, despite supplementation of calcium and vitamin D in accordance with the Dutch guidelines.
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Epilepsia , Deficiência Intelectual , Osteoporose , Adolescente , Densidade Óssea , Criança , Criança Institucionalizada , Pré-Escolar , Estudos Transversais , Epilepsia/tratamento farmacológico , Epilepsia/epidemiologia , Feminino , Humanos , Deficiência Intelectual/epidemiologia , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: In the Diagnostic and Statistical Manual of Mental Disorders-Fifth edition (DSM-5), the diagnostic criteria of intellectual disability (ID) include three domains of adaptive deficits: the conceptual, social and practical. Substantial intra-individual differences between domains can be considered an ID domain discrepancy. METHOD: We explored the associations between ID domains, discrepancies and epilepsy in 189 adults (mean age = 47.9; SD = 15.6). Each DSM-5 ID domain was assessed separately, using subscales of the Vineland II for the social and practical domains, and psychological instruments, including intelligence tests, for the conceptual domain. A set of standardised criteria is proposed to identify an ID domain discrepancy. RESULTS: An ID domain discrepancy seemed to be present in about one-third of subjects and was particularly present in subjects with moderate ID (53.4%). Impairment in the social domain was most often the reason for the discrepancy. The presence of a discrepancy was significantly related to a focal (localised) epilepsy type (OR = 2.3, P = .028) and a mixed seizure type (OR = 1.4, P = .009). Epilepsy characteristics that are indicative of a more severe and refractory epilepsy, including various seizure types, a high seizure frequency, a combined epilepsy type (both focal and generalised epilepsy) and an early age at onset, were significantly related to more severe impairments in conceptual, social and practical adaptive behaviour (all P values <.01). CONCLUSIONS: With a substantial proportion of the subjects who had both ID and epilepsy with an ID discrepancy, professionals should be aware of this and take all domains of ID into account when studying or working with this vulnerable population.
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Adaptação Psicológica/fisiologia , Epilepsia/fisiopatologia , Deficiência Intelectual/diagnóstico , Deficiência Intelectual/fisiopatologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Manual Diagnóstico e Estatístico de Transtornos Mentais , Epilepsia/epidemiologia , Feminino , Humanos , Deficiência Intelectual/classificação , Deficiência Intelectual/epidemiologia , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
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.
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Transtorno Autístico/diagnóstico , Transtorno Autístico/epidemiologia , Epilepsias Mioclônicas/diagnóstico , Epilepsias Mioclônicas/epidemiologia , Adolescente , Adulto , Transtorno do Deficit de Atenção com Hiperatividade/diagnóstico , Transtorno do Espectro Autista , Transtornos Globais do Desenvolvimento Infantil/complicações , Manual Diagnóstico e Estatístico de Transtornos Mentais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Automutilação , Adulto JovemRESUMO
BACKGROUND: The focus of medicine is shifting from treatment to preventive care. The expression of biomarkers of dementia and Alzheimer's disease (AD) appear decades before the onset of observable symptoms, and evidence has emerged supporting pharmacological and non-pharmacological interventions to treat modifiable risk factors of dementia. However, there is limited research on the epidemiology, clinical phenotypes, and underlying pathobiology of cognitive diseases in Asian populations. OBJECTIVES: The objectives of the Biomarkers and Cognition Study, Singapore(BIOCIS) are to characterize the underlying pathobiology of Cognitive Impairment through a longitudinal study incorporating fluid biomarker profiles, neuroimaging, neuropsychological and clinical outcomes in a multi-ethnic Southeast Asian population. DESIGN, SETTING, PARTICIPANTS: BIOCIS is a 5-year longitudinal study where participants are assessed annually. 2500 participants aged 30 to 95 will be recruited from the community in Singapore. To investigate how pathology presents with or without minimal clinical symptoms and vice versa, CI and unimpaired individuals will be recruited. Participants will undergo assessments to characterise biomarkers of dementia through neuroimaging, fluid biomarkers, cognitive assessments, behavioural and lifestyle profiles, retinal scans and microbiome indicators. RESULTS: Since commencement of recruitment in February 2022, 1148 participants have been enrolled, comprising 1012 Chinese, 62 Indian, and 35 Malay individuals. Mean age and education is 61.32 years and 14.34 years respectively with 39.8% males. 47.9 % of the cohort are employed and 32.06% have a family history of dementia. The prevalence of cerebral small vessel disease is 90.2% with a mean modified Fazekas white matter hyperintensity score of 4.1. CONCLUSION: The BIOCIS cohort will help identify novel biomarkers, pathological trajectories, epidemiology of dementia, and reversible risk factors in a Southeast Asian population. Completion of BIOCIS longitudinal data could provide insights into risk-stratification of Asians populations, and potentially inform public healthcare and precision medicine for better patient outcomes in the prevention of Alzheimer's disease and dementia.
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Biomarcadores , Disfunção Cognitiva , Humanos , Singapura/epidemiologia , Estudos Longitudinais , Masculino , Idoso , Pessoa de Meia-Idade , Feminino , Disfunção Cognitiva/diagnóstico , Disfunção Cognitiva/epidemiologia , Adulto , Idoso de 80 Anos ou mais , Testes Neuropsicológicos , Cognição/fisiologia , Neuroimagem , Demência/epidemiologia , Demência/diagnóstico , Projetos de PesquisaRESUMO
PURPOSE: Long-term antiepileptic drug use is associated with low bone mineral density (BMD), fractures and abnormalities in bone metabolism. We aimed at determining the prevalence of bone mineral disorders in patients with refractory epilepsy treated with antiepileptic drugs. METHODS: A cross-sectional survey was conducted in adult patients (n = 205) from a residential unit of a tertiary epilepsy centre. Screening for bone mineral disorders was performed with dual-energy X-ray absorptiometry (DXA) scan of spine and hip (including bone mineral density and vertebral fracture assessment) and laboratory measurements. Patient information regarding demography, epilepsy characteristics and medication use was recorded. Based on DXA T-scores, prevalence of bone mineral disorders (osteopenia and osteoporosis) was calculated. Correlations between DXA T-scores and epilepsy parameters were explored. RESULTS: Of the 205 patients, there were 10 dropouts. 80% (n = 156/195) of the patients had low BMD: 48.2% had osteopenia and 31.8% had osteoporosis. Of those having low BMD, 51.9% (n = 81/195) was between 18 and 50 years. The T-score of the femoral neck correlated significantly with total duration of epilepsy, cumulative drug load and history of fractures. Linear regression analysis showed that of the epilepsy-related parameters, only cumulative drug load significantly predicted low femoral neck T-score (P = 0.001). CONCLUSION: In this high-risk population, we obtained a very high prevalence of 80% of low BMD. Both men and women were affected as well as patients <50 years of age. This study illustrates the magnitude of the problem of bone mineral disorders in chronic epilepsy.
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Anticonvulsivantes/efeitos adversos , Doenças Ósseas Metabólicas/induzido quimicamente , Doenças Ósseas Metabólicas/epidemiologia , Pacientes Internados , Osteoporose/induzido quimicamente , Osteoporose/epidemiologia , Absorciometria de Fóton , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Densidade Óssea/efeitos dos fármacos , Doença Crônica/tratamento farmacológico , Estudos Transversais , Epilepsia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Análise de Regressão , Adulto JovemRESUMO
BACKGROUND: An association between antiepileptic drugs (AEDs), low bone mineral density (BMD), fractures, and abnormalities in bone metabolism has been suggested for a longer period, although conclusive evidence has not been reported. We aimed at studying patient characteristics in a high-risk population. METHODS: All adult patients from a residential unit of a tertiary epilepsy center who were diagnosed with osteoporosis and consequently treated with a bisphosphonate at that moment were included. Correlations between reported fractures and patient characteristics were explored. RESULTS: Of the total population of 261 adult patients, 54 patients were included resulting in a high prevalence rate of 21% osteoporosis in this population. The number of fractures correlated significantly with ambulatory status (r = -0.269, P = 0.05), drug load (r = 0.286, P = 0.04), and current number of AEDs (r = 0.283, P = 0.04). Correlations could not be provided for individual drugs in our population as only a minority was on monotherapy and even less patients had always been on monotherapy of the same antiepileptic drug. Linear regression analysis showed that cumulative drug load (defined by a surrogate parameter: the total duration of epilepsy multiplied by the number of AEDs) was the dominant factor explaining the occurrence of fractures. CONCLUSION: In this high-risk population, we obtained a positive and strong correlation between the occurrence of fractures in a diagnosed population with osteoporosis and the cumulative drug load of AEDs. This effect seems general, independent of the type of AEDs that were used.
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Anticonvulsivantes/efeitos adversos , Densidade Óssea/efeitos dos fármacos , Epilepsia/tratamento farmacológico , Osteoporose/induzido quimicamente , Fraturas por Osteoporose/induzido quimicamente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticonvulsivantes/administração & dosagem , Anticonvulsivantes/uso terapêutico , Epilepsia/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteoporose/complicações , Fraturas por Osteoporose/complicaçõesRESUMO
PURPOSE: The aim of this longitudinal study was to assess trabecular bone scores (TBS) in institutionalized adults with refractory epilepsy and intellectual disability and to study the association of TBS and incident fractures during seven years of follow-up. METHODS: In 2009 and 2016, all institutionalized adult patients of a long-stay care facility in the Netherlands (n=261) were invited to undergo a dual-energy X-ray absorptiometry (DXA) including vertebral fracture assessment (VFA) and assessment of TBS. Vertebrae T4-L4 were analyzed using quantitative morphometry. New and worsening vertebral fractures (VFs) were considered as incident VFs. Data regarding clinical fractures were extracted from the medical files. Patients were treated with anti-osteoporosis medication according to the Dutch guideline. RESULTS: Baseline and follow-up DXA, VFA and TBS could be obtained in 136 patients (83 male) aged between 18 and 79 years old (44.7±15.5). At baseline, 36 patients (26.5%) were diagnosed with osteoporosis, 68 (50.0%) with osteopenia and 32 patients (23.5%) had a normal bone mineral density (BMD). As for TBS, 26 patients (19.1%) had a partially degraded microarchitecture and 26 patients (19.1%) a degraded microarchitecture. During seven years of follow-up, 80 patients (59%) sustained at least one fracture, of which 28 patients (35%) had one or more major osteoporotic fractures. Thirty-four patients (25.0%) had at least one new or worsening morphometric VF. Compared to baseline, TBS significantly decreased over seven years of follow-up in non-treated patients (-0.039±0.064, p<.001). In patients who were treated with bisphosphonates for more than one year during follow-up, TBS did not change significantly (p=.093). In multivariate analyses, no significant associations were found between TBS at baseline and incident fractures during follow-up. CONCLUSION: In this study, we found a high incidence of fractures and TBS decreased significantly over seven years of follow-up in non-treated institutionalized adult patients with refractory epilepsy and intellectual disability, but TBS was not associated with incident fractures.
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Epilepsia Resistente a Medicamentos , Deficiência Intelectual , Fraturas da Coluna Vertebral , Adulto , Humanos , Masculino , Adolescente , Adulto Jovem , Pessoa de Meia-Idade , Idoso , Osso Esponjoso/diagnóstico por imagem , Seguimentos , Epilepsia Resistente a Medicamentos/complicações , Deficiência Intelectual/epidemiologia , Deficiência Intelectual/complicações , Estudos Longitudinais , Vértebras Lombares , Fraturas da Coluna Vertebral/complicações , Fraturas da Coluna Vertebral/epidemiologia , Densidade ÓsseaRESUMO
PURPOSE: To determine the incidence of clinical fractures over seven years of follow-up, in adults with epilepsy and intellectual disability, residing in a long-stay care facility. METHODS: In 2009, all institutionalized adult patients (n = 261) were invited to undergo a Dual-energy X-ray Absorptiometry (DXA) measurement and a Vertebral Fracture Assessment (VFA). Participants were followed over seven years or until date of discharge (in case of moving from the care facility) or date of death. The patients' medical files were screened for radiology reports and staff notes, to identify clinical fractures. Fracture incidence rates (IR) were determined and compared for subgroups, by calculating incidence rate ratios. Hazard ratios were calculated to identify factors associated with fracture risk, using Cox Proportional Hazards analyses. RESULTS: A total of 205 patients (124 male, 60.5%) aged between 18 and 88 years (median 48, IQR 34-60) were enrolled. At baseline, 92 patients (44.9%) were diagnosed with osteopenia and 65 (31.7%) with osteoporosis. Between 2009 and 2016, 30 patients (14.6%) deceased and 3 patients (1.5%) left the care facility. During follow-up, 156 clinical fractures were reported in 82 patients (40.0%). Thirty-eight patients (18.5%) had at least one major osteoporotic fracture. Overall, the IR was 11.6 fractures per 100 person-years. Fracture risk was significantly lower in patients who were wheelchair dependent than in patients who were able to walk (p<.001). CONCLUSION: This study demonstrated that 40% of institutionalized adults with epilepsy and intellectual disability had at least one clinical fracture during seven years of follow-up, despite adequate anti-osteoporosis treatment.
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Epilepsia , Deficiência Intelectual , Fraturas por Osteoporose , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Densidade Óssea , Epilepsia/epidemiologia , Seguimentos , Humanos , Incidência , Deficiência Intelectual/complicações , Deficiência Intelectual/epidemiologia , Masculino , Pessoa de Meia-Idade , Fraturas por Osteoporose/epidemiologia , Adulto JovemRESUMO
PURPOSE: Long-term exposure to anti-epileptic drugs has been shown to decrease bone mineral density (BMD). The aim of this 7-year follow-up study was to explore changes in bone status, using quantitative ultrasound (QUS) and Dual-energy X-ray Absorptiometry (DXA) in adults with refractory epilepsy and intellectual disability (ID) residing at a long-term care facility. Both measurements can be challenging to conduct in this population. METHODS: In 2009 and 2016, a total of 126 patients (18-79 years) underwent QUS of the heel and DXA of lumbar spine (LS) and hip (femoral neck (FN) and total hip (TH)). Subgroup analysis was performed for patients with (group A, n = 53) and without (group B, n = 73) bisphosphonate use during follow-up. RESULTS: Overall, weak to moderate correlations between changes in DXA and QUS parameters were found. For group A, correlations varied from r = .31 to .59, whereas correlations did not exceed r = .40 in group B. Patients in group A showed a larger increase or a smaller decrease in BMD for all DXA regions during follow-up (p < .001 for ΔLS and ΔFN BMD, p = .001 for ΔTH BMD). For change in QUS parameters, no significant difference between groups was found. CONCLUSION: In this study we demonstrated the limited use of QUS in the monitoring of bone status in our study population. Although correlations between changes in QUS parameters and axial DXA are positive and mostly significant, QUS only explains little of the variability in DXA values and is inadequate for measuring treatment response in this population.
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Absorciometria de Fóton/normas , Anticonvulsivantes/efeitos adversos , Densidade Óssea , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Deficiência Intelectual , Ultrassonografia/normas , Adolescente , Adulto , Idoso , Comorbidade , Difosfonatos/uso terapêutico , Epilepsia Resistente a Medicamentos/tratamento farmacológico , Epilepsia Resistente a Medicamentos/epidemiologia , Feminino , Seguimentos , Humanos , Deficiência Intelectual/epidemiologia , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
INTRODUCTION: Several risk factors for sudden unexplained death in epilepsy patients (SUDEP) have been proposed, but subsequent work has yielded conflicting data. The relative importance of various risk factors for SUDEP was never explored. The aim of this study is to review systematically risk factors for SUDEP and also to determine their relevance for SUDEP by calculating relative risk factor ratios. METHODS AND MATERIALS: Authors performed a literature-search on "SUDEP" in Medline, the Cochrane Library and EMBASE. Studies with unknown number of SUDEP cases or with less than five SUDEP cases and reviews were excluded from further analysis. The value of each paper was assessed, based on the quality of the study and the reliability of the diagnosis of SUDEP. This value ranged from 1 (low quality) to 10 (high quality). Papers with a value below 7 were eliminated for further analysis. For each analysed factor, a risk factor ratio was determined, with a higher ratio for a stronger risk factor. RESULTS: A number of strong risk factors for SUDEP: young age, early onset of seizures, the presence of generalized tonic clonic seizures, male sex and being in bed. Weak risk factors for SUDEP: prone position, one or more subtherapeutic bloodlevels, being in the bedroom, a strucural brain lesion and sleeping. CONCLUSIONS: In this study, authors have designed a quality scale to select papers. The relative importance of risk factors for SUDEP is demonstrated.
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Morte Súbita/etiologia , Epilepsia Tônico-Clônica/epidemiologia , Epilepsia/complicações , Fatores Etários , Idade de Início , Feminino , Humanos , Masculino , Fatores de Risco , Fatores Sexuais , SonoRESUMO
OBJECTIVE: To evaluate risk factors for sudden and unexpected death in epilepsy (SUDEP) in a high-risk population, i.e. patients treated in a Dutch tertiary referral center for epilepsy. METHODS: All patients who died between January 1999 and April 2004 while under treatment of the epilepsy center were identified. Based on clinical data, deaths were classified as definite, probable, possible or non-SUDEP. Potential risk factors were compared in SUDEP cases and non-SUDEP cases. RESULTS: SUDEP incidence was 1.24 per 1000 patient years. SUDEP patients died at a younger age than patients from the control group of non-SUDEP deaths with epilepsy and had an earlier onset of epilepsy. However, the frequently mentioned factors in previous studies, i.e. male sex, generalized tonic-clonic seizures, high seizure frequency, specific AEDs, polytherapy with several AEDs, mental retardation, psychiatric illness and psychotropic comedication, were not found to be correlated with SUDEP. CONCLUSIONS: Even in this high-risk population of patients with refractory epilepsy, treated in a tertiary referral center, SUDEP is not a frequently occurring phenomenon. Specific risk factors could not be identified within an already high-risk population.
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Morte Súbita/epidemiologia , Epilepsia/mortalidade , Adolescente , Adulto , Fatores Etários , Idoso , Anticonvulsivantes/uso terapêutico , Criança , Pré-Escolar , Epilepsia/classificação , Epilepsia/tratamento farmacológico , Humanos , Pessoa de Meia-Idade , Países Baixos , Estudos Retrospectivos , Fatores de RiscoRESUMO
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.
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Epilepsia/complicações , Convulsões/complicações , Traumatismos da Medula Espinal/etiologia , Acidentes por Quedas , Adulto , Anticonvulsivantes/efeitos adversos , Densidade Óssea/efeitos dos fármacos , Vértebras Cervicais , Epilepsia/tratamento farmacológico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Traumatismos da Medula Espinal/patologia , Traumatismos da Medula Espinal/reabilitaçãoRESUMO
PURPOSE: In newly diagnosed patients with Dravet syndrome sodium channel blockers are usually avoided. However, in many adult patients the diagnosis was made long after the initiation of therapy. The purpose of our study was to acquire information concerning the potential risks and benefits of (ox)carba(ma)zepine withdrawal in adult patients with genetically confirmed Dravet syndrome. METHOD: We identified 16 adults with Dravet syndrome, living in a tertiary care facility for people with epilepsy and an intellectual disability. We reviewed clinical history, genetic findings, the type and duration of sodium channels blockers that were used, seizure types and frequency, and the effect of a change in these medications. RESULTS: The study population consisted of 9 men and 7 women. Median age was 35 years (range 20-61 years). An attempt to withdraw carbamazepine (CBZ) was made in 9 patients. In 3 of these patients an increase in tonic-clonic seizures was observed. An attempt to withdraw oxcarbazepine (OXC) was made in 3 patients, leading to a complete stop in 2 patients. 3 of the 4 deaths in the withdrawal-group were related to epilepsy. CONCLUSION: In adult patients with Dravet syndrome withdrawal of CBZ or OXC is not without risks. We suggest that (ox)carba(ma)zepine withdrawal should be considered in these patients but only if there is a good reason to do so and only if they are closely monitored.
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Anticonvulsivantes/uso terapêutico , Carbamazepina/análogos & derivados , Carbamazepina/uso terapêutico , Epilepsias Mioclônicas/tratamento farmacológico , Síndrome de Abstinência a Substâncias , Bloqueadores do Canal de Sódio Disparado por Voltagem/uso terapêutico , Adulto , Anticonvulsivantes/efeitos adversos , Carbamazepina/efeitos adversos , Epilepsias Mioclônicas/genética , Epilepsias Mioclônicas/mortalidade , Feminino , Humanos , Pacientes Internados , Masculino , Pessoa de Meia-Idade , Canal de Sódio Disparado por Voltagem NAV1.1/genética , Oxcarbazepina , Estudos Retrospectivos , Convulsões/tratamento farmacológico , Convulsões/genética , Convulsões/mortalidade , Centros de Atenção Terciária , Bloqueadores do Canal de Sódio Disparado por Voltagem/efeitos adversos , Adulto JovemRESUMO
An increasing number of studies suggest a direct effect of antiepileptic drug (AED) therapy on bone health: Patients on chronic AED therapy may have an increased risk of fractures, reduced bone mineral density, osteopenia, and osteoporosis. In an attempt to distinguish general and specific risk factors, this review examines the available empirical research. The pathophysiology is discussed and guidelines for early detection and treatment options are proposed.
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Anticonvulsivantes/efeitos adversos , Doenças Ósseas , Densidade Óssea , Doenças Ósseas/induzido quimicamente , Doenças Ósseas/diagnóstico , Doenças Ósseas/prevenção & controle , Bases de Dados Factuais/estatística & dados numéricos , Epilepsia/tratamento farmacológico , Humanos , Fatores de RiscoRESUMO
The aim of this study was to retrospectively assess the recanalization rate, factors associated with and time taken for recanalization to occur in a matched ruptured and unruptured aneurysm population that were treated with endovascular coiling.Ruptured and unruptured aneurysms treated between 2002 and 2007 were matched for aneurysm location, diameter and neck size. Recanalization rate, time to recanalize, re-treatment rate and clinical outcome were analysed. Ninety-eight matched ruptured and unruptured aneurysms (49 aneurysms in each group) were studied. 46.8% of aneurysms in the ruptured group achieved complete obliteration on the initial post treatment angiogram versus 34.7% in the unruptured group. The ruptured group had a higher rate of recanalization (40.4% versus 20.4%). 25.5% of aneurysms had significant recanalization in the ruptured group versus 6.1% in the unruptured group (p=0.009). The retreatment rate was higher in the ruptured group (21.3% versus 6%). Ruptured aneurysms took a shorter time to recanalize with a mean time of 5.3±3.8 months versus 12.4±7.7months (p=0.003). Multivariate logistic regression analysis found neck size (p=0.0098), wide neck morphology (p=0.0174), aneurysm diameter (p< 0.0001) and ruptured aneurysms (p=0.0372) were significant predictors of recanalization. The majority of patients in both groups had a good outcome with GOS=5 (85.7% and 83.7%) but two deaths occurred in the ruptured group.Ruptured and unruptured aneurysms showed significant differences in rate, degree and timing of recanalization, thus requiring different protocols for imaging follow-up post endovascular treatment. Earlier and more frequent imaging follow-up is recommended for ruptured aneurysms.
Assuntos
Aneurisma Roto/epidemiologia , Aneurisma Roto/terapia , Embolização Terapêutica/estatística & dados numéricos , Aneurisma Intracraniano/epidemiologia , Aneurisma Intracraniano/terapia , Adulto , Estudos de Coortes , Feminino , Seguimentos , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Retratamento/estatística & dados numéricos , Estudos Retrospectivos , Resultado do TratamentoRESUMO
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.
Assuntos
Anticonvulsivantes/administração & dosagem , Epilepsia/tratamento farmacológico , Deficiência Intelectual/tratamento farmacológico , Transtornos do Humor/tratamento farmacológico , Psicotrópicos/administração & dosagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antidepressivos/administração & dosagem , Antipsicóticos/administração & dosagem , Carbamazepina/administração & dosagem , Estudos de Coortes , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Epilepsia/complicações , Feminino , Humanos , Deficiência Intelectual/complicações , Lamotrigina , Masculino , Pessoa de Meia-Idade , Transtornos do Humor/complicações , Estudos Retrospectivos , Triazinas/administração & dosagem , Ácido Valproico/administração & dosagem , Adulto JovemAssuntos
Demência/terapia , Nutrição Enteral , Defesa do Paciente , Idoso , Tomada de Decisões , Ética Médica , Família/psicologia , Feminino , Gastrostomia , HumanosRESUMO
BACKGROUND AND PURPOSE: Clot extent, location, and collateral integrity are important determinants of outcome in acute stroke. We hypothesized that a novel clot burden score (CBS) and collateral score (CS) are important determinants of clinical and radiologic outcomes and serve as useful additional stroke outcome predictors. MATERIALS AND METHODS: One hundred twenty-one patients with anterior circulation infarct presenting within 3 hours of stroke onset were reviewed. The Spearman correlation was performed to assess the correlation between CBS and CS and clinical and radiologic outcome measures. Patients were dichotomized by using a 90-day modified Rankin scale (mRS) score. Uni- and multivariate logistic regression models were used to assess variables predicting favorable clinical and radiologic outcomes. Receiver operating characteristic and intraclass correlation coefficient (ICC) analyses were performed. Diagnostic performance of a CBS threshold of >6 was assessed. RESULTS: There were 85 patients (mean age, 70 +/- 14.5 years). Patients with higher CBS and CS demonstrated smaller pretreatment perfusion defects and final infarct volume and better clinical outcome (all, P < .01). CBS (P = .009) and recanalization (P = .015) independently predicted favorable outcome. A CBS >6 predicted good clinical outcome with an area under the curve of 0.75 (95% confidence interval [CI], 0.65-0.84; P = .0001), sensitivity of 73.0 (95% CI, 55.9-86.2), and specificity of 64.6 (95% CI, 49.5-77.8). The recanalization rate with intravenous recombinant tissue plasminogen activator was higher in patients with CBS >6 (P = .04; odds ratio, 3.2; 95% CI, 1.1-9.4). The ICC was 0.97 (95% CI, 0.95-0.98) and 0.87 (95% CI, 0.80-0.91) for CBS and CS, respectively. CONCLUSIONS: CBS and CS are useful additional markers predicting clinical and radiologic outcomes.
Assuntos
Angiografia Cerebral/métodos , Circulação Colateral , Infarto da Artéria Cerebral Média/diagnóstico por imagem , Trombose Intracraniana/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Idoso , Idoso de 80 Anos ou mais , Revascularização Cerebral , Circulação Cerebrovascular , Feminino , Humanos , Infarto da Artéria Cerebral Média/terapia , Trombose Intracraniana/terapia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Valor Preditivo dos Testes , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
This study assessed the impact of epilepsy on motor function in children. We aimed to analyze the effect in uncomplicated epilepsies (cryptogenic partial and idiopathic generalized epilepsy). A group of 87 children with epilepsy (47 males, 40 females; mean age 8y, standard deviation [SD] ly 9mo, range 4y 11mo to 12y 11mo), but without learning disability* or other neurological comorbid disorders, was compared with a control group of 107 children (76 males, 31 females; mean age 8y 4mo, SD 2y 2mo, range 4y 7mo to 12y 2mo). The differences in main motor skills and psychomotor speed were analyzed using the Movement ABC and computerized measures for simple reaction times and finger tapping. No significant difference in motor function was found. The overall psychomotor development of children with epilepsy is comparable to controls. However, a significant slowing of psychomotor speed in the group with epilepsy was reported. No relation with antiepileptic drug treatment was demonstrated and no difference in psychomotor speed between different types of epilepsy was found. The existence of a condition leading to excessive neuronal discharge leads to a general cortical inhibition. The slowing of psychomotor speed in children with uncomplicated epilepsy may be the behavioural presentation of this inhibition.