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PubMed2006

What about us? Siblings of children with epilepsy.

RATIONALE: It is known that epilepsy has a severe impact in the quality of life of the patients; however, it affects the lives of all family members. The psychosocial repercussions of epilepsy are often of greater significance than the seizures themselves. METHODS: This was a prospective study, conducted from January 2005 to December 2005 at the pediatric epilepsy clinic of our University Hospital. Parents were interviewed by one of the authors according to a structured questionnaire about the impact of epilepsy in the life of the siblings of children with epilepsy. RESULTS: One hundred and twenty-seven children, siblings of 78 patients with epilepsy were evaluated. From the 127 siblings of children with epilepsy, 60 were girls and 67 were boys. Ages ranged from 5 to 18 years old (mean=11.7 years). After the diagnosis of epilepsy, the siblings had only negative feelings toward the disease, mostly sadness and fear. CONCLUSION: Our data showed that the impact of epilepsy in the lives of siblings of children with epilepsy is much more severe than previously suspected.

Adolescent↗

PubMed2005

Survey of public awareness, attitudes, and understanding toward epilepsy in Nhan Chinh, Hanoi, Vietnam, in 2003.

PURPOSE: Many studies have shown that cultural and social awareness of, attitudes toward, and knowledge about epilepsy can have an impact on the acceptance of treatment. The aim of this study is to characterize public awareness of attitudes toward and understanding of epilepsy in a Vietnamese community and to compare results with those obtained in other cultures. METHODS: A survey concerning the knowledge, attitudes, and practices of Vietnamese people with respect to epilepsy was carried out in the Nhan Chinh precinct of Hanoi. One thousand people were randomly selected for face-to-face interviews regarding epilepsy. RESULTS: Of the subjects surveyed, 54.6% had heard of epilepsy; 45.5% knew someone with epilepsy; 49.2% had witnessed an epileptic seizure; 56% would not allow their son or daughter to marry someone with epilepsy; 42.1% did not believe that epileptic patients could hold down a normal job; 77.8% believed that epilepsy is an organic disorder of the brain; 23.8% thought that epilepsy is a form of dementia; and 91% thought that epileptic patients require medical care. CONCLUSION: As compared with surveys conducted in other countries, our survey showed that the awareness of epilepsy in this Vietnamese community is limited, and gives rise to alternative attitudes and practices.

Adolescent↗

PubMed2006

Intractable epilepsy: A survey of patients and caregivers.

The social and health consequences associated with epilepsy are often magnified among patients with refractory epilepsy. Despite recent advances in the treatment of seizure disorders, many people with epilepsy continue to suffer from uncontrolled seizures and adverse side effects from medical therapy. This survey is the first to focus solely on the experiences, attitudes, and quality of life of a refractory epilepsy population, both those with the condition and their caregivers. To participate in this survey, respondents had to currently be experiencing seizures or troubling treatment side effects and had to have tried at least two different epilepsy medications. These survey data represent three groups of participants (n = 903): those with epilepsy who self-reported on their condition (Group 1, n = 503), the caregivers of those with refractory epilepsy (Group 2, n = 200), and those with epilepsy who had their condition reported on by a caregiver (Group 3, n = 200). This survey revealed that the negative consequences associated with epilepsy tend to be greater among those experiencing treatment side effects and a greater number of seizures. Physicians must take into account medication side effects and quality-of-life issues when treating patients with epilepsy.

Adolescent↗

PubMed2006

The oral glucose tolerance test is frequently abnormal in patients with uncontrolled epilepsy.

PURPOSE: The clinical efficacy of the ketogenic diet as therapy for patients with difficult-to-treat epilepsy prompted us to investigate the glucose metabolism of these patients under an oral overload of glucose, that is, in the oral glucose tolerance test (OGTT). METHODS: Thirty patients (12 males, 18 females; age range: 17-59, mean: 35.1) with difficult-to-treat epilepsy, 23 patients with controlled epilepsy (11 males, 12 females; age range: 14-66, mean: 36.9), and 39 control subjects (18 males, 21 females; age range: 16-58, mean: 33.3) were evaluated with the OGTT. For patients with epilepsy, we also measured C-peptide and glycosylated hemoglobin in the fasting state. Glucose levels lower than 70 mg/dL at any point of the curve were considered to be abnormal. RESULTS: All subjects in the control group and the group with controlled epilepsy had a normal OGTT. In contrast, all 30 patients with difficult-to-treat epilepsy had at least one point on the OGTT curve below the normal range (P<0.001), most often 180 and 240 minutes after the oral glucose load (P<0.001). C-peptide levels were significantly lower in the group with difficult-to-treat epilepsy as compared with the group with controlled epilepsy. Fasting glycohemoglobin and insulin levels did not differ between the two patient groups. CONCLUSIONS: We suggest that undiagnosed metabolic disturbances in patients with difficult-to-treat epilepsy may somehow contribute to their refractoriness to conventional pharmacological therapy. We propose the hypothesis that calorie-restricted diets aimed at correcting OGTT curves may prove beneficial in treating patients with difficult-to-treat epilepsy. Our hypothesis generates a clear endpoint for the diet, and its demonstration would provide new standards for diet-based antiepileptic regimens. Accordingly, our results may help in understanding the positive consequences of ketogenic or calorie-restricted diets in persons with seizures.

Adolescent↗

PubMed2006

Validation of a Portuguese version of a generic health quality-of-life questionnaire for children with neurological and neuropsychiatric disorders, including epilepsy.

PURPOSE: To validate a Portuguese version of a generic HRQL instrument already used for children with epilepsy. To analyze differences of scores among children with epilepsy and co-morbidities in contrast to other neurological and neuropsychiatric diagnosis, especially Attention Deficit and Hyperactivity Disorder (ADHD). METHODS: A total of 194 children (64 with epilepsy, 94 with ADHD and 38 with other neurological or neuropsychiatric diagnosis) were consecutively seen in a neuropaediatric ambulatory unit. Parents or caregivers responded to a Portuguese version of ICIS (Impact of Childhood Illness Scale). The questionnaire was analyzed for internal consistency and face validity. Clinical and socio-demographic variables were also analyzed. RESULTS: Internal consistency, analyzed by Cronbach's alpha showed good results for total and combined scores and all sections of ICIS, except for the section "impact of illness and its treatment". The diagnostic categories differed in several ICIS sections, with the worst scores for children with epilepsy and co-morbidities. Children who only had epilepsy had a worse score on the section about treatment impact compared with children with ADHD, but better scores in relation to disease impact in development and parents/family. In the epilepsy group, HRQL scores were worst for symptomatic epilepsy, whilst seizure control and socioeconomic factors affected each section of the instrument in different ways. CONCLUSIONS: ICIS Portuguese version has good psychometric properties. It is a useful tool for analyzing HRQL in children with epilepsy and other neurodevelopmental and psychiatric disorders. Epilepsy and psychiatric co-morbidity seems to have an additive impact on a child's HRQL. This result is clinically important since epilepsy management must include not only seizure control but also mental health support.

Adolescent↗

PubMed2024

Exploring correlations between immune cell phenotypes and the risk of epilepsy: A bidirectional Mendelian randomization study.

BACKGROUND: Neuroinflammation plays an important pathophysiological role in epilepsy; however, the precise connection between immune cells and epilepsy remains unclear. This study used Mendelian randomization (MR) to analyze the causal relationship between 731 immune cell traits and epilepsy. METHODS: Based on data from a genome-wide association study (GWAS), a bidirectional two-sample MR analysis was conducted to investigate the potential influence of immune cell phenotypes on epilepsy. Five MR methods were used to analyze the results, with the inverse variance weighted (IVW) method as the primary method, and the results were corrected using the false discovery rate (FDR) method. Sensitivity analyses were performed to test for heterogeneity and horizontal pleiotropy. RESULTS: After correction for FDR, four immune traits remained significantly associated with epilepsy risk: CD25 expression on memory (OR = 1.04, 95 % CI = 1.02 ∼ 1.06,P = 2.55 × 10-4), IgD+CD38dim (OR = 1.05, 95 % CI = 1.02 ∼ 1.08, P = 4.73 × 10-4), CD24+CD27+ (OR = 1.04, 95 % CI = 1.02 ∼ 1.06, P = 4.82 × 10-4), and IgD-CD38dim (OR = 1.04, 95 % CI = 1.02 ∼ 1.06, P = 1.04 × 10-3) B cells. The risk of generalized epilepsy was significantly associated with two immune cell traits, whereas that of focal epilepsy was significantly associated with seven immune cell traits. Furthermore, immune cell phenotypes are not affected by genetically predicted epilepsy. CONCLUSION: This MR study affirms the causal connection between circulating immune cells and epilepsy, offering guidance for further understanding of the immune mechanisms that underlie epilepsy and the discovery of novel targets for therapy.

Humans↗

PubMed2024

Dissecting the association between blood pressure traits, hypertension, antihypertensive medications and epilepsy: A Mendelian randomization study.

BACKGROUND: Observational studies suggest that hypertension and epilepsy have a high co-occurrence, and antihypertensive medications may have impacts on the prevention and treatment of epilepsy. However, the directionality of causation between them is elusive. METHOD: By leveraging genome-wide association studies (GWAS) summary data of each trait, we firstly performed bidirectional univariate Mendelian randomization (UVMR) to assess the strength and direction of the associations between pairs of traits, then multivariate MR (MVMR) was conducted to adjust for potential confounders in causalities. Cochran's Q statistics, leave-one-out analysis, MR-Egger regression and MR-Pleiotropy Residual Sum and Outlier methods (MR-PRESSO) were employed to evaluate the robustness of the results. Drug target MR was proceeded to assess the association between five classes of first-line antihypertensive medications and epilepsy. Specifically, single nucleotide polymorphisms (SNPs) extracted from GWAS data on systolic blood pressure (SBP)/diastolic blood pressure (DBP), along with expression quantitative trait loci (eQTL) were utilized as proxies for antihypertensive medications, respectively. RESULTS: Forward UVMR results provided evidence that genetically predicted blood pressure traits and hypertension have causal effects on epilepsy, while reverse UVMR indicated no causal impacts of epilepsy on blood pressure traits or hypertension. The sensitivity analysis results were robust. The causalities between DBP, hypertension and epilepsy remained remarkable after adjustment by MVMR. Inverse-variance-weighted MR (IVW-MR) yielded evidence of positive association only between Beta-Blockers target genes based on DBP GWAS screening and epilepsy. Summary-data-based MR (SMR) identified a positive correlation between Beta-Blockers target gene ADRA1D and epilepsy risk. CONCLUSIONS: Hypertension has a causal effect on epilepsy and managing DBP in patients with hypertension through Beta-Blockers may help prevent epilepsy.

Humans↗

PubMed2002

Effects of chronic epilepsy on intellectual functions.

Intractable epilepsy is related to various transient and chronic brain electric and neurochemical disturbances. There is increasing evidence that long-lasting chronic epilepsy may induce secondary neuronal loss and metabolic dysfunctions. Still a matter of controversy is, however, whether cognitive abilities of patients deteriorate with increasing duration of intractable epilepsy. We present results from two independent cross-sectional studies dealing with measures of global cognitive performance in two different ways. The first study investigated in 78 patients with refractory temporal lobe epilepsy (TLE) the influence of duration of epilepsy on the difference between an estimated measure of former or pre-morbid intelligence and the current performance in an intelligence test. The second study aimed at duration of epilepsy-dependent effects on current IQ measures of 209 patients with refractory TLE. Both studies showed that the duration of epilepsy contributes to the explanation of interindividual variability in IQ measures of adult TLE patients to a higher degree than age and age of epilepsy onset. Similar to several studies on hippocampal neuronal density, hippocampal volume, and glucose metabolism, the presented cross-sectional data demonstrate that a duration of chronic epilepsy exceeding two decades is associated with worse cognitive abilities. Consequently, refractory TLE seems to induce a very slow but ongoing cognitive deterioration. It is assumed that epilepsy-related noxious events and agents exhaust the compensatory capacity of brain functions. A high cognitive reserve capacity, however, might delay the onset of deterioration.

Adult↗

PubMed2001

Epilepsy associated with infantile hemiparesis: predictors of long-term evolution.

To study the evolution of epilepsy associated with infantile hemiparesis (IH) in relation to age and identification of factors predictive of pharmacoresistance. Thirty-four children with epilepsy and associated IH were followed for a period of 13 years and 3 months (range 5-19 years). All the patients underwent clinical evaluation and EEG, CT and/or MRI. Disease course was evaluated from the time of diagnosis of epilepsy to end of follow-up by differentiating the cases with severe pharmacoresistance from those with favourable outcome. Several possible prognostic factors were identified predicting evolution toward intractable epilepsy. Univariate statistical analysis by calculating odds ratio (OR) with 95% confidence interval (CI) and multivariate analysis by logistic regression were performed. Eleven cases presented severe epilepsy evolving toward pharmacoresistance; duration of epilepsy was always longer than 8 years. Twenty-three cases (seven with severe epilepsy and 16 with mild epilepsy) evolved toward remission; in these patients epilepsy duration was shorter (2-7 years) and a complete remission was obtained within 12 years of age. Significant prognostic factors associated with pharmacoresistance included: non-vascular causes, cortical lesions, mixed and frequent seizures during the first two years of epilepsy. Our results show that surgical treatment could be considered in cases with unfavourable prognostis factors.

Adolescent↗

PubMed1997

Dispensing epilepsy medication: a method of determining the frequency of symptomatic individuals with seizures.

We estimated the prevalence and incidence of epilepsy in The Netherlands using drug-dispensing information from the PHARMO database, containing medication histories of nearly 300,000 individuals. An algorithm based on antiepileptic drug prescription records was used to identify patients with epilepsy requiring medication for seizure control. The algorithm was validated by comparing positive algorithm identifications to medical diagnoses from general practitioners and hospital records. In 1990-1991, the algorithm revealed 1158 patients with "certain" epilepsy, and 451 patients with "probable" epilepsy. Epilepsy was present in 93% of patients on polytherapy, and 58% on monotherapy. Clonazepam monotherapy was non-specific for epilepsy. The use of carbamazepine monotherapy for epilepsy was age-dependent. After correcting the algorithm for these drugs, and standardizing to the Dutch population, the point prevalence of epilepsy was 4.8/1000 (95% CI: 4.5-5.0). The incidence rate was 0.72/1000 person-years (95% CI: 0.65-0.79). Using drug-dispensing data for epilepsy medication, it is possible to make valid estimations of the number of epilepsy patients requiring drug therapy.

Adolescent↗

PubMed1999

Depression and schizophrenia in epilepsy: social and biological risk factors.

BACKGROUND: In a retrospective study we investigated the role of social and biological risk factors for the development of major depression and schizophreniform psychoses in epilepsy. We tested the hypotheses that social risk factors are associated with depression and biological risk factors are associated with schizophreniform psychoses. METHOD: We studied 25 patients with epilepsy and paranoid-hallucinatory psychosis, 25 patients with epilepsy and major depression, and 50 non-psychiatric epilepsy patients (controls) with respect to biological and psychosocial variables. RESULTS: Schizophrenic patients had an earlier age of onset of epilepsy and a more severe epilepsy as characterised by history of status epilepticus, multiple seizure types and severity of seizures compared to non-psychiatric controls. Simple seizure symptoms were often vegetative and EEGs showed various abnormalities including temporal lobe discharges but no lateralisation to either side. With respect to antiepileptic drugs (AED) there were only few significant differences between groups: Polytherapy as well as treatment with phenytoin (DPH) was more frequent in psychotic patients as compared to non-psychiatric patients. Patients with psychoses were also characterized by a disturbed familial background, lack of interpersonal relationships, social dependency and professional failure. Depressive patients were significantly older than non-psychiatric controls and they suffered more frequently from focal epilepsies arising from the temporal lobe. They did not differ from controls with respect to severity of epilepsy. Treatment with valproate (VPA) was inversely linked with depression, suggesting that VPA may have prophylactic antidepressive properties in epilepsy patients. There were no psychosocial variables significantly linked with depression. CONCLUSIONS: In this study, patients with different forms of psychiatric complications in epilepsy could clearly be distinguished from controls. However, we could not confirm the simple hypothesis that there are biological predictors for schizophreniform psychoses and psychosocial predictors for major depression. Neurological and sociological variables seem linked with both, suggesting a multifactorial etiology.

Adult↗

PubMed2000

A questionnaire survey about doctor-patient communication, compliance and locus of control among south Indian people with epilepsy.

To date, very few studies have investigated patients' views on the information they receive from the doctor concerning epilepsy and its management. Little information is available about the influence of doctor-patient communication and locus of control on the compliance of persons with epilepsy. We investigated, through a questionnaire-interview design, among patients attending the epilepsy clinic of a tertiary referral center in South India, their views about the provision of information by the doctor, and their compliance and locus of control. We also determined the interrelation between doctor-patient communication, compliance and locus of control. Our subjects comprised 200 adult persons with epilepsy, 113 males and 87 females, mean age 30.5 (range 18-67) years. Over one-third of the subjects received from the doctor insufficient information about epilepsy and its treatment. There was a significant positive correlation between effective doctor-patient communication and compliance. A majority of our patients had an external locus of control, which negatively influenced the compliance. Even in a comprehensive epilepsy clinic of a model tertiary referral center in a developing country, a significant proportion of patients do not receive optimal information about epilepsy from the doctor. Knowledge about their disease will encourage people with epilepsy to make informed choices, and achieve better compliance and personal control of their problems. Educating primary and secondary care physicians about the importance of doctor-patient communication in the management of epilepsy and educating the public about the positive aspects of life in epilepsy cannot be overemphasized.

Adolescent↗

PubMed2003

Risk factors for sudden unexpected death in epilepsy: a controlled prospective study based on coroners cases.

We performed a controlled prospective study of pathologically verified sudden unexpected death in epilepsy (SUDEP) in a coronial setting, to identify risk factors. We prospectively studied coronial deaths of people with epilepsy in Vic., Australia, during a 21-month period. Fifty SUDEP and 50 subjects with epilepsy who died of other causes (controls) were collected sequentially. Clinical data was obtained shortly after death from questionnaires completed by treating doctors, discussion with family members and coronial files, including police reports of death, autopsy and toxicology reports. Factors assessed were age, sex, duration of epilepsy, type of seizure(s), seizure frequency, symptomatic epilepsy, including post-traumatic epilepsy, presence of structural brain lesion, idiopathic epilepsy, mental retardation, psychiatric illness, including dementia, recent stressful life event, particular antiepileptic drugs (AEDs) and AED polytherapy, compliance with AED treatment, psychotropic drug prescription, alcohol and other substance abuse, place of death and evidence of terminal seizure. The SUDEP group was characterised by younger age and higher proportion found dead in bed and with evidence of terminal seizure compared to controls. The profile of patients at risk for SUDEP are young people with epilepsy. They are most likely to die in sleep and our data support the view that SUDEP is a seizure-related event. This, taken in conjunction with the finding that there was no increased risk associated with a particular AED in monotherapy or multiple AEDs suggests that attempts to better treat patients' epilepsy with AEDs might decrease the risk of SUDEP. Although the literature suggests that SUDEP is more frequent in patients with severe epilepsy, we did not find a correlation with seizure frequency suggesting that other clinical indices may be more important.

Adolescent↗

PubMed1998

Patient views on primary care services for epilepsy and areas where additional professional knowledge would be welcome.

In the past decade there has been increasing interest in the part that general practice can play in the care of people with epilepsy. Primary care services for epilepsy vary from practice to practice. Some studies have suggested that people with epilepsy prefer secondary care services and are not keen for their epilepsy to be managed in general practice, but much of the data were collected in secondary care. This study collected data from various sources about present provision of services, patient satisfaction with services, views about service development, areas where GP knowledge may be improved and whether the site of data collection influenced the results. A questionnaire was piloted, then distributed and collected through branches of the British Epilepsy Association, general practice and secondary care clinics. Data collected were both quantitative and qualitative. One hundred and seventy-eight questionnaires were collected from three sources. The responders were a severe seizure group. Structured care in general practice was uncommon with 54% being seen only when needed. Dose and type of antiepileptic medication was rarely altered in general practice. Information about their condition was given to 44% of the responders by their GP. Sixty-one percent would prefer their epilepsy care to be 'shared' between primary and secondary services. The majority of patients were satisfied with GP services, felt they could easily discuss their epilepsy, but 58% felt they 'rarely' or 'never' received enough information about their condition in general practice. Satisfaction with GP care varied, dependent on where the data were collected. Patients would value more information and more time to discuss the effects of their epilepsy. In conclusion general practice care for epilepsy is still reactive. Patients value more information and more time to discuss implications. The data collection point affects the results; any conclusions about the organisation of epilepsy care should draw data from community patient samples.

Adolescent↗

PubMed2002

High incidence of language disorder in children with focal epilepsies.

To investigate the relationship between the presence of language disorder, type of epilepsy, and epileptic seizures in childhood, language levels, types of language impairment, and aetiologies were examined in 109 children, aged between 5 and 17 years, attending a national children's epilepsy assessment unit over a 4-year period. There were 70 males and 39 females. Median age was 11 years 4 months (range 5 to 18 years 9 months). In addition to neurological assessment, simultaneous video and EEG monitoring and prolonged ambulatory EEG, each child underwent a comprehensive series of multidisciplinary tests, including intelligence, language, and communication assessments. Classification of seizures and epilepsy syndromes was agreed in conference by a physician specializing in childhood epilepsies, a paediatric neurologist, and a neuropsychiatrist. Other test procedures were administered by a speech and language pathologist with assistance from a neuropsychologist when relevant. Level of language disability in these children was associated with a range of aetiological factors. Evidence was found of a significant number of associations between focal epilepsies, certain seizure types, and language disorder. Of the 46 (42.2%) children with language disorders in the research sample, 30 had localization-related epilepsies and a further three had epilepsies which were undetermined as to whether focal or generalized. Children with focal epilepsies were 30% more likely to have language disorder than other language disability subtypes. The research demonstrated a clear though often subtle association between focal (localization-related) epilepsy and language disorder, indicating an increased risk in this patient group. Children with simple or complex partial seizures were more likely to have language disorder than other language disability subtypes; they also tended to have the lowest number of seizure types per case. This is of importance to clinicians managing children with epilepsy and has implications for their educational and social welfare.

Adolescent↗

PubMed1999

Health-related quality of life in childhood epilepsy: the results of children's participation in identifying the components.

Separate groups of children with epilepsy, recruited from a regional pediatric epilepsy database, and their parents were established to discuss their life with epilepsy. Twenty-nine children (aged between 6 years and 10 years 4 months) and 42 of their parents were placed into nine and 17 groups respectively. The participants provided information about their own perceptions of life with epilepsy. Discussions were taped and textual analysis followed to extract, understand, explain, and categorize the health-related quality of life (HRQL) components. The process enabled us to identify the burdens and concerns of children with epilepsy, and to identify five emerging dimensions: (1) the experience of epilepsy, (2) life fulfillment and time use, (3) social issues, (4) impact of epilepsy, and (5) attribution. Identifying and understanding the components of HRQL is crucial for developing an HRQL scale in childhood epilepsy. In addition, this list of elements can help health professionals improve their services by considering and addressing aspects of the epilepsy experience beyond the traditional issues for children with epilepsy and their families.

Attitude to Health↗

PubMed2001

Comparison of epilepsy and asthma perception among preschool teachers in Taiwan.

PURPOSE: The purpose of this study was to investigate preschool teachers' perceptions to children with epilepsy and asthma in middle areas of Taiwan. METHODS: Two-hundred sixteen preschool teachers were investigated with a questionnaire regarding their knowledge, attitude, and practice. There were 22 questions in the questionnaire (half of the questions were for epilepsy, and others for asthma). Differences between teachers' perceptions of children with epilepsy and asthma were analyzed. RESULTS: More teachers thought epilepsy was a hereditary disease and asthma was an acquired disease. Over 30% of participants thought epileptic seizures were associated with insanity but asthma attacks were not. Acceptance of children with epilepsy was significantly lower than that of children with asthma. Children with epilepsy were less encouraged by teachers to play with others. Teachers have more consideration for aggression of children with epilepsy than of those with asthma. More teachers were worried about the objection from the other parents if there were a child with epilepsy in the class. CONCLUSIONS: Perceptions of epilepsy and asthma among preschool teachers were different significantly. The finding may explain why the children with epilepsy showed lower school performance among the children with chronic illness in past studies.

Adolescent↗

PubMed2003

The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma.

PURPOSE: Hamartomas of the hypothalamus (HH) cause an uncommon and unusual epilepsy syndrome. The condition is recognized to affect children, but the presentation in adults is not well understood. We present 19 children and adult patients with HH, including three patients whose epilepsy began in adult life. The patterns of clinical presentation, evolution of the epilepsy from childhood to adult life, and electroclinical diagnostic features are presented. METHODS: Nineteen patients, both children and adults with HH and epilepsy, were evaluated clinically, with EEG, video-EEG, and magnetic resonance imaging (MRI) scanning. Seven patients underwent surgical resection of the hamartoma. Stereotactic thermocoagulation of the hamartoma was performed in two patients. RESULTS: Gelastic seizures occurred at onset of epilepsy in 15 of 16 early-onset cases. Subsequently, multiple seizures types occurred, which then evolved to mainly partial epilepsy with tonic or complex partial seizures (five of eight adults), or became entrenched symptomatic generalized epilepsy with atypical absences, drop attacks, and secondarily generalized seizures, and cognitive impairment (three of eight adults). In the adult-onset patients, gelastic seizures were not prominent, the epilepsy was milder, and they functioned normally. Stereotactic thermocoagulation of the hamartoma resulted in improvement in seizure control in two patients. CONCLUSIONS: Gelastic seizures are not a prominent feature of epilepsy in adult patients with HH. The epilepsy associated with HH, although severe at onset, can evolve into a milder syndrome in later life. For less severely affected patients, minimally invasive alternatives to the traditionally difficult open surgical treatment should be considered.

Adolescent↗