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Long-term follow-up of childhood epilepsy associated with tuberous sclerosis.

PURPOSE: To study the clinical and electroencephalographic (EEG) characteristics of patients whose epilepsy is associated with tuberous sclerosis, with special reference to their clinical course. METHODS: We investigated the electroclinical and radiologic features of 38 patients with epilepsy associated with tuberous sclerosis. RESULTS: Eleven patients showed only generalized epilepsy, and 10 showed only localization-related epilepsy throughout their clinical course. Among the other 17 cases, the nature of the epilepsy changed between generalized and localization-related epilepsies during the clinical course. A shift from generalized to localization-related epilepsies was more common than the reverse. Seventeen had West syndrome (WS), three had Lennox-Gastaut syndrome (LGS), and eight had epilepsies that evolved from WS to LGS. Tonic spasms, mostly in series, were seen in all 28 patients with generalized epilepsy. Eleven of the 28 patients had partial seizures and tonic spasms in the same period. Six of them showed "simultaneous seizures," consisting of tonic spasms in series and a partial seizure. Partial seizures were the main seizure type in 27 patients with localization-related epilepsy, but three of them also showed tonic spasms that included "simultaneous seizures." Ictal EEGs revealed multiple active foci in the same period that could shift during the clinical course. Neither the location nor number of tubers was related to the clinical course. As for seizure outcome, 12 (32%) of 38 patients were free from seizures at follow-up. CONCLUSIONS: In epilepsies associated with tuberous sclerosis, there may be an interrelation between generalized and localization-related epilepsies, as well as one between generalized and partial seizures.

Child↗

[Children with drug-resistant partial epilepsy: criteria for the identification of surgical candidates].

The majority of candidates for epilepsy surgery, in both children and adults, belong to the syndromic category of partial epilepsies. However, particularly in children, the clinical expression of epilepsy may sometimes be misleading, as paroxysmal events may present as generalized seizures although having a focal onset. The spectrum of surgical possibilities for early-onset epilepsy has tended to widen rapidly and to include not only other focal epilepsies but also more difficult types. This is because developmental lesions that are the major cause of infantile epilepsy are often poorly localized, involve extensive brain areas and require extensive operations (e.g. hemispherotomy). Surgery is also used for progressive conditions such as Rasmussen's encephalitis or Sturge-Weber syndrome. Some forms of generalized symptomatic epilepsy may also benefit from palliative surgical procedures (callosotomy in cases with repetitive drop attacks often as a manifestation of a Lennox-Gastaut syndrome). The timing of surgery partly depends on the probable effectiveness of the operation available for each particular patient. Candidates for epilepsy surgery must be identified early in the process of the disease, thus increasing the chances for a satisfactory cognitive and behavioral outcome. The decision should be taken by experienced multidisciplinary groups, that will also ensure post-operative follow-up, both in terms of medical management and psycho-social integration. Prospective studies are still needed, to evaluate the long-term cognitive evolution of children operated on early for their epilepsy and cured. Increasing evidence indicates that drug therapy is likely to fail to achieve control of the seizures when two-three of the major drugs, properly chosen by competent clinicians, have not obtained satisfactory results, i.e. full control of seizures and absence of side effects. Testing of all possible drugs is not advisable, because this process would be excessively long when surgery is a reasonable possibility. The requirements for resective surgery in children with partial drug-resistant epilepsy vary with the type of resection considered. Three basic requirements apply to almost all cases: (a) the epileptogenic area must be localized to a territory whose removal is contemplated; (b) that no other independent epileptogenic area exists in those areas that are not included in the planned resection, and (c) that any possible deficit resulting from resection must be acceptable. The current experience confirms that the complete resection of the epileptogenic area is the major condition for a satisfactory surgical result. The possibilities for resective surgery depend upon the localization of the ictal onset zone, its relationship with adjacent functional brain areas and the availability of convergent data that point to a single localization. Epilepsy surgery mandates a multidisciplinary approach that requires special skills and sophisticated instruments and materials that cannot be improvised. A global evaluation of risks and expected benefits is always required. All decisions require a close collaboration between the epilepsy team, the patient and the family. With the exception of high quality MRI and video-EEG recording of seizures, the need to perform any other complementary presurgical investigation should be evaluated on an individual basis.

Anticonvulsants↗

Sleep and benign partial epilepsies of childhood: EEG and evoked potentials study.

The electroclinical picture and nosological limits of benign partial epilepsy of childhood with rolandic spikes (BERS) have been better defined by nocturnal sleep records. In all stages of sleep, there is a significant increase in frequency and amplitude of rolandic spikes (RS) without change of their morphology. Another interesting observation is the appearance of independent spike foci in sleep, or brief subclinical spike wave discharges which are limited to the state of drowsiness. More recently, other types of partial epilepsy of childhood with benign evolution have been identified: (a) partial epilepsy with induced spike representing somatosensory evoked potentials; (b) benign psychomotor epilepsy; (c) partial epilepsy with occipital spike waves. In all these forms, the sleep records are essentially similar to those in BERS and have been very helpful in the nosological identification of these forms of epilepsy. For this reason, the sleep records of these special forms are truly informative for the clinician from the diagnostic and prognostic viewpoint. On the other hand, some investigators, have pointed out that, in the initial stage of these benign forms of partial epilepsy, there may be more or less significant intellectual impairment and behavioral disorder, sometimes accompanied by frequent brief absences. From the EEG viewpoint, this condition is characterized by brief discharges of slow spike wave complexes amounting to a pattern of "electrical status epilepticus". This special electroclinical condition mimics the Lennox-Gastaut syndrome but is generally self-limited. Thus, a correct differential diagnosis is very important from the diagnostic viewpoint. There is good evidence that sleep records permit an earlier identification of these conditions and strongly contribute to a correct differential diagnosis. In the benign partial epilepsy the SEPs, during awake and sleep, morphology and latency are normal, while the N60 amplitude is increased. A group of children with benign partial epilepsy shows EEG spikes evoked by tapping, and giant N60 component. This giant component persists during sleep and is not specific for any type of benign partial epilepsy. In conclusion, the results of sleep recordings are conducive to a correct diagnosis and better definition of the nosological delineation of partial epilepsies in childhood; they also provide a better comprehension of their evolution, and thus of their prognosis. The Evoked Potentials seem be a useful tool in the study of benign partial epilepsy.

Adolescent↗

Modern management of epilepsy: Adolescents.

Epilepsy most commonly starts in the first two decades of life. Adolescence is a time of great change both in the epilepsy itself and in a number of other areas. Growth into adulthood, issues of preparation for university or employment, driving, drinking, preparation for marriage/conception and a general increase of responsibility add to the complexity of this time of life. Epilepsy affects all these areas to a significant degree. The incidence of several epilepsy syndromes peaks in adolescence. These include juvenile myoclonic epilepsy, juvenile absence epilepsy, epilepsy with grand mal on awakening, benign partial seizures of adolescence and reading epilepsy. Photosensitivity also appears to peak around puberty and needs to be managed well to avoid both unreasonable risks and unnecessary restrictions. Early diagnosis and correct management of the epilepsy and the specific epilepsy syndrome are the main factors in minimizing the difficulties. Epilepsy may change in the early adolescent years, with seizures starting and stopping or altering in form, all of which add to the uncertainty. Denial of the epilepsy may lead to risk-taking which may include be provided on the high risk of the unsupervised bath, the effect of irregular sleep, alcohol, driving, sport, employment, genetic implications, advantages/adverse effects of specific antiepileptic drugs and the role of surgery. The doctor should listen, counsel and inform. Adolescents generally do not appreciate being given advice. They should be empowered by the doctor to make informed decisions and encouraged to take control in a situation which they may view as implying devastating loss of control, unless it is managed wisely.

Adolescent↗

The search for epilepsies ideal for clinical and molecular genetic studies.

The first step in localizing the chromosomal site of specific epilepsies is to define their pattern of inheritance. This determination is now being carried out for benign juvenile myoclonic epilepsy; fifty multigenerational families are being studied in three separate epilepsy programs in Los Angeles, Winston-Salem, NC, and Berlin. Concurrent with these studies, investigators are combining the principles of classic linkage analysis, using 30 protein markers, with the use of restriction-fragment-length polymorphisms to determine the chromosomal location of juvenile myoclonic epilepsy. Two problems appear formidable, however. First, since the chromosomal location of specific epilepsies is unknown, the entire human genome must be screened. Second, once the location of a specific epilepsy gene is narrowed down to a region of 10(6) base pairs, the problem of identifying the actual molecular defect is difficult, especially if we have no assay or method to show that a given gene is culpable for producing epilepsy. An approach more likely to succeed is to use as markers the DNA fragments of proteins that are suspected to cause the disease in experimental models of genetic epilepsies; for example, the gamma-aminobutyric acid receptor genes, which are suspected to cause myoclonic epilepsy in experimental animals, can be tested in benign juvenile myoclonic epilepsy. At the same time, other marker proteins could be used to locate the chromosomal site of other specific epilepsies. Once the chromosomal site is determined, recombinant DNA technology will permit the measurement of the precise arrangement of the genes for these restriction-fragment-length polymorphisms and protein markers at a given locus of a chromosome.

Chromosome Mapping↗

Positron emission tomography in epilepsy: correlative study.

Positron emission tomography (PET) was performed with the 18F-fluoro-deoxy-glucose method on 29 patients with epilepsy (generalized epilepsy, 4; partial epilepsy, 24; undetermined type, 1). The subjects were restricted to patients with epilepsy without focal abnormality on X-CT. All the patients with generalized epilepsy showed a normal pattern on PET. Fourteen out of the 24 patients with partial epilepsy and the 1 with epilepsy of undetermined type showed focal hypometabolism on PET. The hypometabolic zone was localized in areas including the temporal cortex in 11 patients, frontal in 2 and thalamus in 1. The location of hypometabolic zone and that of interictal paroxysmal activity on EEG were well correlated in most patients. The patients with poorly-controlled seizure showed a higher incidence of PET abnormality (12 out of 13) than those with well-controlled seizures (2 out of 11). The incidence of abnormality on PET and MRI and the location of both abnormality were not necessarily coincident. These results indicated that the PET examination in epilepsy provides valuable information about the location of epileptic focus, and that the findings on PET in patients with partial epilepsy may be one of the good indicators about the intractability of partial epilepsy, and that PET and MRI provide complementary information in the diagnosis of epilepsy.

Blood Glucose↗

Epilepsies after Pocket Monster seizures.

BACKGROUND: We conducted a 5-year follow-up study in patients with a seizure induced by animated cartoon "Pocket Monster." METHODS: A recurrence of seizures was observed in 25 of 91 patients with a Pocket Monster seizure. The patients were divided into two groups: Epilepsy group of 12 patients with a history of epilepsy and Nonepilepsy group of 13 patients without a history of epilepsy. RESULTS: Age below 12 years and generalized seizures were more often in Nonepilepsy group than in Epilepsy group. Seizure recurrence was earlier in Epilepsy group than in Nonepilepsy group. Photoparoxysmal response was relatively infrequent in Epilepsy group. The majority of patients were classified into idiopathic generalized epilepsies in Nonepilepsy group, whereas most patients were categorized into localization-related epilepsies in Epilepsy group. CONCLUSIONS: Epilepsies after Pocket Monster seizures were different according to the presence or absence of a history of epilepsy. These results will be useful in order to determine the treatment of a patient with a visually induced seizure.

Cartoons as Topic↗

The epidemiology of the epilepsies in children.

The epilepsies are a heterogeneous collection of neurological conditions and syndromes characterized by recurrent, unprovoked, paroxysmal seizure activity. There are several types of epileptic seizures and syndromes that are unique to children, including infantile spasms, Lennox-Gastaut syndrome and absence seizures. Febrile seizures and neonatal seizures, while not epilepsy, are relatively common types of seizures in infants and children and are likely markers of risk of later epilepsy. Thus, it is important to consider the epidemiological features of the epilepsies as they occur specifically in infants and children. The purpose of this review is to summarize what is currently known about the epidemiology of the childhood epilepsies and to identify promising areas for further population-based studies. The epilepsies are an important cause of neurological morbidity in children. The average annual rate of new cases (incidence) of epilepsy is approximately 5-7 cases per 10,000 children from birth to age 15 years, and in any given year, about 5 of every 1,000 children will have epilepsy. There is evidence that the incidence of the epilepsies in some populations of children may be decreasing over time, and this possibility merits further investigation. Factors that are known to increase risk of the epilepsies in children include congenital malformations of the central nervous system (CNS), moderate or severe head trauma, CNS infections, certain inherited metabolic conditions, and genetic factors. However, these account for only 25% to 45% of cases, and thus, the etiology of most cases of the epilepsies remains obscure. The paucity of well-controlled etiological studies is due largely to formidable methodological problems in conducting epidemiological studies of the epilepsies. The prognosis for seizure control is generally good, although children with remote symptomatic seizures and those with additional neurological disabilities do less well.

Adolescent↗

Medical risks in epilepsy: a review with focus on physical injuries, mortality, traffic accidents and their prevention.

The present review aims at highlighting selective aspects of the medical risks in epilepsy and their prevention. Emphasis is put on accidents and physical injuries, including risk factors and effectiveness of prevention; mortality, its causes, risk factors and prevention of seizure-related deaths, as well as traffic accidents, their risk factors and the effectiveness of prevention. Accidents and injuries are slightly more frequent among people with epilepsy than in the general population. This increased risk is probably most prevalent in patients with symptomatic epilepsy and frequent seizures, most often in combination with associated handicaps. The majority of accidents are trivial and occur at home. The most frequent injuries among patients with epilepsy are contusions, wounds, fractures, abrasions and brain concussions. The standardised mortality ratio (SMR; the ratio of observed number of deaths in a population with epilepsy to that expected, based on age and sex-specific mortality rates in a reference population) in population-based studies of epilepsy is 2-3 compared to the general population. This increased mortality is largely related to the etiology of the epilepsy and is probably not influenced by the treatment of the epilepsy. On the other hand, most fatalities in patients with chronic, therapy resistant epilepsy seem to be seizure-related and often sudden unexpected deaths (SUDEP). The frequency of such seizure-related deaths is most likely to be reduced by intensified treatment aiming at early seizure control, although appropriate studies for definitive evidence are still lacking. Apparently, there is an increased rate of traffic accidents in drivers with epilepsy, even if population-based prospective data are lacking. Many of these accidents are seizure-related. Probably, the extent to which physicians report their patients with uncontrolled epilepsy to the authorities is too low, but this has not yet been explored. Moreover, the preventive measures in legislation may be ignored by many people with epilepsy.

Accidents, Traffic↗

Cardiac autonomic dysfunctions in chronic refractory epilepsy.

BACKGROUND: Sudden unexplained death is an important cause of mortality in patients with epilepsy and cause for this is not fully understood. One of the explanations is autonomic dysfunction (AD). Studies of AD in chronic refractory epilepsy are very few in the literature. AIM: To evaluate cardiovascular autonomic functions in chronic refractory epilepsy patients. METHODS AND MATERIALS: Seventy-three patients (31.5+/-9.8 years, M:F::45:28) with chronic intractable epilepsy attending the "refractory epilepsy clinic" at a tertiary referral center (National Institute of Mental Health and Neuro Sciences (NIMHANS), Bangalore, India) were enrolled. Age and gender matched healthy subjects were recruited as controls. Heart rate (HR) and blood pressure (BP) at rest and HR response after deep breathing, Valsalva maneuver, postural change and BP response to postural change and isometric work were recorded. AD was graded as early if one of HR or BP, definite if two or more HR and severe if two or more HR with BP based tests were detected to be abnormal. RESULTS: The mean age at onset and duration of epilepsy was 12.4+/-8.5 years and 19.02+/-9.07 years, respectively. Twenty-three (31.5%) patients of refractory epilepsy had early involvement while 25 patients (primary generalized: 8, partial: 17) had Definite AD, and 16 (primary generalized: 4, partial: 12) had severe autonomic dysfunction. ANCOVA results showed expiration-inspiration, standing maximum:minimum ratio, standing 2 min systolic and isometric diastolic BP of the dysfunction group significantly differ compared to the control group. Patients with longer duration of epilepsy (23.2 years) had more severe dysfunction (p < 0.05) than patients with relatively shorter duration (17.5 years) of epilepsy. Antiepileptic drugs (AED) used did not show any significant role on autonomic functions in this study. CONCLUSION: This is the first study from India to evaluate autonomic functions in refractory epilepsy patients. Autonomic dysfunction was noted in 56.3% of patients. Anticonvulsants used were not associated with AD. Longitudinal controlled studies with 'newly diagnosed' epilepsy patients will enhance further understanding about the role of autonomic system in epilepsy.

Adult↗

Risk factors for childhood epilepsy: a case-control study.

BACKGROUND: Risk factors for epilepsy are conditions associated with an increased frequency of epilepsy, and those for childhood epilepsy are different from those for epilepsy occurring later in life. In the present matched case-control study, we attempted to identify some possible risk factors for childhood epilepsy. METHODS: All children with epilepsy who attended the clinic of Motahary in Shiraz, Iran during a 6-month period were included in the study. Neurologically normal children, matched for age and sex, visiting the same clinic were considered as controls. The data on patients and controls were obtained from answers to a questionnaire obtained through personal interviews. Details on the patient, family history, parental smoking, breast versus formula feeding, and parental age at the time of childbirth were included. Medical records were then reviewed. RESULTS: In total, 142 patients and 138 controls participated. Positive family history of epilepsy increased the risk of developing epilepsy by 3.34-fold. A higher risk of epilepsy was observed in patients living in rural areas (OR = 2.44). CONCLUSIONS: Positive family history of epilepsy and residence in rural areas were the two major risk factors associated with epilepsy in this study; neonatal jaundice, parental age, and passive smoke exposure added no risk. Breast-feeding was not shown to have a protective effect against development of epilepsy.

Case-Control Studies↗

Kids' perception about epilepsy.

PURPOSE: Epilepsy remains a stigmatized condition. Lack of information has been pointed to as a cause of the perpetuation of stigma. Our goal was to survey children's perception of epilepsy. METHODS: We used a questionnaire to determine if the children knew what epilepsy is and, if they did not know, what did they think epilepsy is. Twenty-nine children (15 girls; mean age 10 years, range 9-11 years) from a fourth-grade class of an elementary school in Campinas, Sao Paulo, Brazil, completed the questionnaires individually at the same time in the classroom. This took about 20 minutes. RESULTS: Only four children said they knew what epilepsy is: a disease of swallowing the tongue (3) and a disease that can kill (1). The perceptions of children who said they did not know what epilepsy is were: a disease that can kill, a disease of swallowing the tongue, a contagious disease, a serious illness, a head injury. Three children knew someone with epilepsy, and only two of them had said they knew what epilepsy is. CONCLUSION: The perceptions elicited from the children had a negative connotation; only one child mentioned a relationship between epilepsy and the brain. The spontaneous thoughts of children in this age group, without the contamination of political correctness, may reflect society's collective unconsciousness of the prejudice toward epilepsy and people with epilepsy and needs to be further investigated. Continuous, repetitive educational efforts are necessary in elementary school to change these negative perceptions of epilepsy in our society.

Child↗

Epilepsy.

Seizures are common and are treated in all branches of medicine. Approximately 10% of the population will have one or more seizures during their lifetime. Seizures are symptoms that occur in acute illness, ie, provoked seizures, or in epilepsy, ie, unprovoked seizures. Epilepsy is any disorder in which spontaneous recurrence of unprovoked seizures is the main symptom. It is a common chronic neurologic disorder and affects 1% to 3% of the population. Classification of seizure type is important because it enables identification of the region of the brain where the seizure originated and guides initial diagnostic testing. Classification of epilepsy syndrome, rather than only type of seizure, is more important. Epilepsy syndromes are defined by many factors, including type of seizures, age at onset of seizures, family history of seizures, and findings at physical examination, electroencephalography (EEG), and neurologic imaging studies. Identifying the epilepsy syndrome provides insight into natural history, prognosis, diagnostic testing, and therapy of the disorder and facilitates communication between health care professionals. Understanding seizure type provides useful information even when the epilepsy syndrome cannot be classified. Many sudden events are easily confused with seizures, in particular, pseudoseizures, syncope, migraine, cerebrovascular disease, movement disorders, and sleep disorders. In most cases a detailed history and physical examination concentrated on the details of the event, and results of routine EEG and magnetic resonance imaging can aid in determination of which events are seizures. Video EEG monitoring is occasionally necessary to capture events to enable definitive determination of whether they are seizures and to further characterize them. Provoked seizures are treated with relief of the provoking factor. Antiepileptic drugs (AEDs) are not indicated. However, AEDs may be required to treat unprovoked seizures of new onset in patients at high risk for seizure recurrence or when a second seizure can have devastating psychosocial effects. High risk for recurrence is present when there is a history of brain insult, an EEG demonstrates epileptiform abnormalities, and magnetic resonance images demonstrate a structural lesion. AED therapy is the standard treatment for epilepsy, ie, two or more seizures. Selection of the appropriate AED depends on type of seizure and epilepsy present, and individual drug characteristics, including pharmacokinetics, side effects, dosing interval, and cost. All available AEDs except ethosuximide are effective as adjunctive therapy, and most are effective as initial monotherapy for partial seizures. Generalized seizures preferentially respond to valproate, lamotrigine, and topiramate, among other drugs. If trials of more than two AEDs do not control seizures, additional AEDs are unlikely to be effective, and the patient should be referred to an epilepsy center, where other treatment options, in particular, epilepsy surgery, can be offered. Epilepsy surgery renders 60% to 70% of patients with temporal lobe epilepsy free of disabling seizures.

Adult↗

When antiepileptic drugs aggravate epilepsy.

Paradoxically, an antiepileptic drug (AED) may aggravate epilepsy. The number of AEDs is steadily increasing, and the occurrence of paradoxical aggravation will probably become a frequent problem. The overall status of the patient treated for epilepsy can be altered due to maladjustment to the diagnosis of epilepsy, to unwanted side-effects, to overdosage and to the occurrence of tolerance. However, the main mechanism of aggravation is the occurrence of an inverse pharmacodynamic effect. The specific effect of the AED is such that it controls epilepsy in most cases and increases seizures in other cases. Idiopathic generalised epilepsies (IGE) are particularly prone to pharmacodynamic aggravation: typical absences are constantly increased by carbamazepine (CBZ), vigabatrin, tiagabine, gabapentin, while phenytoin (PHT) is less aggravating. Juvenile myoclonic epilepsy is often aggravated by CBZ, less constantly by PHT and other AEDs. Generalised tonic-clonic seizures found in IGEs may respond to AEDs that aggravate the other seizure types. In symptomatic generalised epilepsies, patients have often several seizure types that respond differently to AEDs: myoclonias are generally aggravated by the same drugs that aggravated IGEs; tonic seizures in the Lennox-Gastaut syndrome respond to CBZ, which may however aggravate atypical absences. In severe myoclonic epilepsy of infancy, there is a nearly constant aggravating effect of lamotrigine. In some patients with benign rolandic epilepsy, a clear aggravation may be produced by CBZ, with occurrence of negative myoclonias, atypical absences, drop attacks, and at the maximum evolution into a state of electrical status epilepticus during sleep. It is much more difficult to pinpoint specific pharmacological sensitivity in other focal epilepsies, but aggravation clearly occurs. When treating epilepsy, the clinician should act according to seizure type, or, better, to epilepsy type. Patients are usually aware of aggravation before their doctors: we should listen carefully whenever they express a 'dislike' for an AED.

Anticonvulsants↗

The social and economic impact of epilepsy in Zambia: a cross-sectional study.

BACKGROUND: Among the 40 million people with epilepsy worldwide, 80% reside in low-income regions where human and technological resources for care are extremely limited. Qualitative and experiential reports indicate that people with epilepsy in Africa are also disadvantaged socially and economically, but few quantitative systematic data are available. We sought to assess the social and economic effect of living with epilepsy in sub-Saharan Africa. METHODS: We did a cross-sectional study of people with epilepsy concurrently matched for age, sex, and site of care to individuals with a non-stigmatised chronic medical condition. Verbally administered questionnaires provided comparison data for demographic characteristics, education, employment status, housing and environment quality, food security, healthcare use, personal safety, and perceived stigma. FINDINGS: People with epilepsy had higher mean perceived stigma scores (1.8 vs 0.4; p<0.0001), poorer employment status (p=0.0001), and less education (7.1 vs 9.4 years; p<0.0001) than did the comparison group. People with epilepsy also had less education than their nearest-age same sex sibling (7.1 vs 9.1 years; p<0.0001), whereas the comparison group did not (9.4 vs 9.6 years; p=0.42). Housing and environmental quality were poorer for people with epilepsy, who had little access to water, were unlikely to have electricity in their home (19%vs 51%; p<0.0001), and who had greater food insecurity than did the control group. During pregnancy, women with epilepsy were more likely to deliver at home rather than in a hospital or clinic (40%vs 15%; p=0.0007). Personal safety for people with epilepsy was also more problematic; rape rates were 20% among women with epilepsy vs 3% in the control group (p=0.004). INTERPRETATION: People with epilepsy in Zambia have substantially poorer social and economic status than do their peers with non-stigmatised chronic medical conditions. Suboptimum housing quality differentially exposes these individuals to the risk of burns and drowning during a seizure. Vulnerability to physical violence is extreme, especially for women with epilepsy.

Adult↗

Recent developments in the quest for myoclonic epilepsy genes.

Understanding the latest advances in the molecular genetics of the epilepsies is important, as it provides a basis for comprehending the new practice of epileptology. Epilepsies have traditionally been classified and subtyped on the basis of clinical and neurophysiologic concepts. However, the complexity and variability of phenotypes and overlapping clinical features limit the resolution of phenotype-based classification and confound epilepsy nosology. Identification of tightly linked epilepsy DNA markers and discovery of epilepsy-causing mutations provide a basis for refining the classification of epilepsies. Recent discoveries regarding the genetics surrounding certain epilepsy types (including Lafora's progressive myoclonic epilepsy, the severe myoclonic epilepsy of infancy of Dravet, and idiopathic generalized epilepsies) may be the beginning of a better understanding of how rare Mendelian epilepsy genes and their genetic architecture can explain some complexities of the common epilepsies.

Epilepsies, Myoclonic↗

Comorbidity headache and epilepsy in childhood.

RATIONALE: Epilepsy and headache are both frequent in childhood. Because seizures are frequently a frightening event, other medical conditions--including headache--are often neglected not only by the patient, but also by the physician. The objective of this study was to verify the comorbidity between headache and epilepsy in childhood. METHODS: This was a prospective study conducted at the pediatric epilepsy clinic of our university hospital. Fifty children with epilepsy and ability to describe their symptoms, between 5 and 18 years old, were interviewed according to a semi-structured questionnaire. The headache was classified according to the International Headache Society. The frequency of headache was compared with the findings of a control group composed by children without epilepsy, siblings of children with epilepsy. RESULTS: Fifty children were evaluated, 29 boys, mean age 11 years. Twenty-three (46 %) patients presented with headache, as opposed to only 1 (2.5 %) in the control group ( p < 0.01). Ten (43.5 %) had migraine, 4 (17.4 %) had tension type headache and in 9 (39.1 %) the type of headache could not be established. In 9/23 (39 %) a temporal relationship between headache and epilepsy was present, 6 postictal and 3 preictal. There was no difference in gender, age, type of seizure and family history of headache in the groups of patients with or without headache. However, most patients with headache were older than 10 years (54.5 %) and had idiopathic epilepsy (65.2 %; p < 0.01). The headache usually started in the same year or after the diagnosis of epilepsy (95 %; p < 0.01). CONCLUSION: Headache and epilepsy are a common comorbidity in childhood, and occur mostly in children older than 10 years with idiopathic epilepsy. The headache usually starts in the same year or after the diagnosis of epilepsy.

Adolescent↗

Children with new-onset epilepsy: neuropsychological status and brain structure.

Abnormalities in cognition, academic performance and brain volumetrics have been reported in children with chronic epilepsy. The nature and degree to which these problems may be present at epilepsy onset or may instead become more evident over time remains to be determined. This study characterizes neuropsychological status, brain structure and their interrelationship in children with recent-onset epilepsy compared with healthy controls. Children (age: 8-18 years) with recent-onset idiopathic epilepsy (n = 53) and healthy controls (n = 50) underwent comprehensive neuropsychological assessment and quantitative volumetric measurement of segmented (grey and white matter) volumes of total cerebrum and lobar regions. Compared with controls, children with recent-onset epilepsy exhibit a pattern of mild diffuse cognitive impairment, regardless of epilepsy syndrome, as well as academic underachievement that in a subset of children antedates the first recognized seizure. There are no overall differences in MR morphometric analyses of total cerebral or lobar tissue volumes. Controls show a strong association between cognitive development and increasing cerebral tissue volume (especially white matter volume), an association that is absent in children with epilepsy. Children with a history of academic achievement problems exhibit the most abnormal cognitive function and have significant volumetric reductions in left occipital and parietal lobe grey matter. Patients with idiopathic epilepsy exhibit cognitive dysfunction and academic underachievement at the onset of the disorder, irrespective of epilepsy syndrome, and indications of antecedent neurocognitive impairment are present in a subset of children. Volumetric abnormalities are not yet apparent in the epilepsy group as a whole, but there are indications of an altered structure-function relationship in epilepsy, and the subset of children with prior history of academic problems have abnormal volume of posterior left hemisphere grey matter. These early abnormalities need to be integrated into lifespan models of the neuropsychology of epilepsy.

Adolescent↗