Ectopic cerebral tissue in the middle ear. A case report

Most idiopathic generalized epilepsies have an onset in childhood or adolescence, with a moderate second incidence peak in the presenium predominantly in women. This study addressed the question of a later onset. The available literature and the records of four personal data sets (two prospective incidence surveys of epileptic seizures, one prevalence study of epilepsy, and one clinical series of individuals with epilepsy) were screened for patients who had experienced a first generalized convulsive seizure with bilateral spike-wave complexes on EEG after 60 years of age. Reports of first idiopathic generalized tonic-clonic seizures occurring after age 60 were extremely rare and none was found in our four cohorts regardless of the methodology involved. Only five case reports were found, all involving a woman. Two had a family history of seizure disorders and two had had at least one seizure earlier in life. Idiopathic generalized epilepsy of late onset, if this condition actually exists, is likely to be the consequence of a genetic predisposition triggered by acquired epileptogenic factors.     

Development of bioactive functions in Hydrangeae Dulcis Folium. VII. Immunomodulatory activities of thunberginol A and related compounds on lymphocyte proliferation

PURPOSE: Vagus nerve stimulation (VNS) has been reported to produce >90% reduction in the number of seizures in children with intractable epilepsy. These encouraging results need confirmation. METHODS: Sixteen children, 10 boys and 6 girls aged 4-19 years, were treated with VNS (Cyberonics, Webster, TX, U.S.A.) for 12-24 months. Seizure frequency, seizure severity, changes in quality of life (QOL: visual analogue scale), and side effects were recorded. Eight children had partial and 8 had generalized seizures; 4 of the latter had Lennox-Gastaut syndrome (LGS). RESULTS: During the tenth to twelfth month of VNS, 6 of 16 children experienced > or =50% reduction in seizure frequency. One girl became seizure-free. Seizure severity showed an average decrease in the score from 15 to 11. After 10 months of treatment, QOL was estimated to have improved > or =50% in 6 of 16 children. Reduction in seizure frequency, decreased seizure severity, and reported improvement in QOL did not entirely coincide. Six children experienced hoarseness, 1 had neck pain, 2 had hypersalivation, 2 experienced tiredness, 2 had aspiration episodes during liquid intake, and 6 had electrical transmission problems; in 4 the problem has been surgically corrected. Five stimulators were turned off due to lack of efficacy. CONCLUSIONS: Six of 16 children with refractory epilepsy treated with VNS improved, with a reduction not only in seizure frequency but also in seizure severity and in QOL.     

Merosin-negative congenital muscular dystrophy, occipital epilepsy with periodic spasms and focal cortical dysplasia. Report of three Italian cases in two families

We report clinical, EEG and neuroimaging findings of three patients in two Italian families with merosin-negative congenital muscular dystrophy (CMD), drug-resistant occipital epilepsy, diffuse persistent cerebral white matter changes and focal cortical dysplasia. Clinical and epilepsy histories, EEG and neuroimaging findings were very similar in all patients. Seizures started in childhood and mainly consisted of periodic spasms, a particular type of partial seizure characterized by clusters of epileptic spasms. The motor expression of the spasms was very mild so that they had been frequently missed or misinterpreted as non-convulsive generalized absence seizures. Interictal EEG showed occipital spike-waves and bilateral synchronous slow spike-wave discharges. Ictal EEG showed prolonged periodic sequences of slow waves with associated fast rhythm complexes, characteristic of periodic spasms. Two patients had normal intelligence, one patient presented moderate mental retardation. Focal cortical dysplasia in the posterior areas of the brain, in addition to marked diffuse white matter alterations, was detected in the magnetic resonance images of all patients. Findings in these patients indicate that in merosin-negative CMD brain involvement can include cortical dysplasia, in addition to white matter changes. In such cases the brain damage can lead to a childhood-onset localization-related symptomatic occipital epilepsy. Epileptic seizures and cortical dysplasia can be, however, difficult to detect in CMD. The clinical semiology of epileptic seizures may in fact be modified because of muscular weakness. This implies that epilepsy may be misdiagnosed or even missed and EEG-polymyographic recordings may be necessary to identify it. Similarly, cortical dysplasia may be very localized and visible by neuroimaging only if it is carefully investigated on the basis of epileptological and EEG-polymyographic findings.     

Prevalence, classification, and severity of epilepsy and epileptic syndromes in children

PURPOSE: To determine the point prevalence of active childhood epilepsy in a defined area and evaluate the usefulness of ILAE classification of seizures, and epilepsies/syndromes with special interest in severe epilepsies. METHODS: By using the latest ILAE International Classification of Epileptic Seizures (ICES, 1981) and Epilepsies and Epileptic Syndromes (ICE, 1989), we determined the age- and sex-specific prevalence rates of epilepsy, type of seizures, epilepsies, and recognizable epileptic syndromes, as well as the proportion of severe cases in each seizure/epilepsy/syndrome category in all children 0-15 years of age from a geographically defined area in Finland. All medical records, neurophysiological recordings and available clinical data were reviewed retrospectively. RESULTS: Point prevalence of active epilepsy on December 12, 1992 was 3.94 per 1,000. According to ICES/ICE, we were able to classify 96% of seizures and 90% of epilepsies and syndromes. Generalized seizure and epilepsy/syndrome types were more prevalent in children 0-6 years of age and partial/localization-related in children 6-15 years of age. Epilepsy was intractable in 17% of all cases and correlated significantly with symptomatic etiology and early onset of epilepsy, as well as with additional neuroimpairments. CONCLUSIONS: A considerable number of cases fell into the nonspecific categories of ICE, which limits the value of present epilepsy/syndrome classification in terms of prognosis, prediction, and indication for special investigations in individual cases. A number of intractable cases was relatively low, indicating good prognosis in many childhood epilepsies, especially when additional neuroimpairments are absent.     

Secretory production of erythropoietin and the extracellular domain of the erythropoietin receptor by Bacillus brevis: affinity purification and characterization

OBJECTIVE: To study the safety and efficacy of methylphenidate in children with the dual diagnosis of epilepsy and attention deficit hyperactivity disorder (ADHD). STUDY DESIGN: Thirty children, aged 6.4 to 16.4 years, with epilepsy and ADHD were studied during a 4-month period. During the initial 2 months of the study, the children were treated with antiepileptic drugs (AEDs) only, and for the remaining 2 months, methylphenidate was added at a morning dose of 0.3 mg/kg. They underwent neurologic assessment, brain computed tomography, IQ testing, and assessment with the Childhood Behavior Checklist at baseline before methylphenidate therapy. Electroencephalography, AED determinations, and the continuous-performance task (CPT) test were done at baseline and after 2 months of methylphenidate therapy. A double-blind, crossover design was used to compare the effects of methylphenidate versus placebo on an electroencephalogram, AED levels, and the CPT. On the 2 days of testing, the child received AEDs and a capsule containing either placebo or methylphenidate. RESULTS: None of the 25 children of this sample who were seizure free had attacks while taking methylphenidate. Of the 5 children with seizures, 3 had an increase in attacks, whereas the other 2 showed no change or a reduction. There were no significant changes in AED levels or electroencephalographic findings. Methylphenidate benefited 70% of children according to parental report; methylphenidate also enhanced performance on the CPT. Side effects of methylphenidate were mild and transient. CONCLUSION: Methylphenidate is effective in treating children with epilepsy and ADHD and safe in children who are seizure free. Caution is warranted for those still having seizures while receiving AED therapy.     

Thyroid function in lead smelter workers: absence of subacute or cumulative effects with moderate lead burdens

The electroencephalographic abnormalities seen in Landau-Kleffner syndrome (LKS) (language deterioration) are non-specific, and consist of a variety of epileptiform discharge patterns including continuous slow spike-wave discharges during sleep, focal sharp waves with spikes, and centrotemporal (rolandic) spikes. Similarly, the EEG abnormalities seen in autistic epileptiform regression (language and social/behavioral deterioration) are non-specific and overlap with those seen in LKS. By contrast, distinct epilepsy syndromes in otherwise normal children occur in the EEG-defined benign focal epilepsies of childhood. Occipital spikes or spike-wave present either in the older child with visual symptoms and headache or in the younger child with autonomic symptoms followed by brief or prolonged partial motor seizures. Seven young children (five from a consecutive series of 42) presenting clinically with autism or autistic regression and possible or definite seizures, whose EEGs revealed occipital spikes or spike-wave characteristic of the benign epilepsies, are reported. Although occipital spikes are commonly seen in young children as an age-dependent EEG-defined benign focal epilepsy, their high frequency in this population with cognitive difficulties suggests a possible causal relation. The effects of the epileptiform discharge on cognitive functioning presumably reflect extension into temporal and parietal lobes, rather than occipital disturbances per se.     

Predictors of epilepsy in children who have experienced febrile seizures

We examined the frequency of development of afebrile seizures in 1706 children who had experienced at least one febrile seizure and were followed to the age of seven years. Epilepsy developed by seven years of age in 20 per 1000 (2 per cent), and another 10 per 1000 had at least one afebrile seizure that did not meet our definition of epilepsy. In children whose neurologic or developmental status was suspect or abnormal before any seizure and whose first seizure was complex (longer than 15 minutes, multiple or focal) epilepsy developed at a rate 18 times higher than in children with no febrile seizures (92 vs. 5 per 1000; P less than 0.001). In the largest group with febrile seizures, those previously normal with noncomplex first febrile seizures, epilepsy developed in 11 per 1000; this rate, although moderate, was greater than that for children with no febrile seizures (P = 0.027). Prior neurologic and developmental status and characteristics of the first febrile seizure are important predictors of epilepsy after febrile seizures.     

Cell biological analysis with respect to cause of fibrous opacification of the anterior capsule after cataract extraction

To assess whether juvenile-onset epilepsy or motor disability is complicated by an increased number of mental health disorders or experience of psychosomatic symptoms in young adulthood, we studied 81 subjects with epilepsy and 52 with motor disabilities at the age of 19 to 25 years and compared them with 211 controls. The main diagnostic tool, the Present State Examination, was administered to those attending the interviews in person who were of normal intelligence; there were 62, 38, and 123 subjects in the three categories, respectively. Compared with the controls, the subjects with epilepsy showed an equal prevalence of psychiatric disorders whereas those with motor disabilities had a significantly higher prevalence, particularly of depressive disorders. The reported prevalence of psychosomatic symptoms confirmed this main result. Psychological illness affected everyday life of two out of five subjects with motor disabilities, but only half of those in the other two groups. It is concluded that motor disability since childhood, but not epilepsy, could be a factor that increases susceptibility to psychiatric morbidity, especially depression, and causes a large number of psychosomatic symptoms. The results challenge staff of clinics working with such adolescents to find individual approaches in preventing the negative influence of psychological disorders on social life.     

Continuous spike-wave activity during sleep. Electroencephalographic and clinical features

Seventeen children were retrospectively evaluated. They exhibited continuous spike-wave activity during slow wave sleep (CSWS). Five of these had only speech problems and seizures (Landau-Kleffner syndrome) (group 1). The other cases had developmental milestones acquisition delay and/or mental retardation (group 2). Epileptic seizures were present in 11 of these, tetraparesis was observed in 5, hemiparesis in 2, microcephaly in 2 and behavior disturbances in 4 cases. The electroencephalogram showed in all cases diffuse CSWS. Group 1 showed diffuse activity, at times accentuated in the centrotemporal region (4/5). Group 2 had widespread discharges, including multifocal activity (5/12), sometimes with anterior predominance (7/12). We concluded that CSWS is a non specific electrographic pattern observed in some types of epilepsy in childhood that have different clinical presentation. It has however some topographic differentiation, depending upon the lesional sites.     

Preoperative predictors of anterior temporal language areas

OBJECT: Although it is known that 5 to 10% of patients have language areas anterior to the rolandic cortex, many surgeons still perform standard anterior temporal lobectomies for epilepsy of mesial onset and report minimal long-term dysphasia. The authors examined the importance of language mapping before anterior temporal lobectomy. METHODS: The authors mapped naming, reading, and speech arrest in a series of 67 patients via stimulation of long-term implanted subdural grids before resective epilepsy surgery and correlated the presence of language areas in the anterior temporal lobe with preoperative demographic and neuropsychometric data. Naming (p < 0.03) and reading (p < 0.05) errors were more common than speech arrest in patients undergoing surgery in the anterior temporal lobe. In the approximate region of a standard anterior temporal lobectomy, including 2.5 cm of the superior temporal gyrus and 4.5 cm of both the middle and inferior temporal gyrus, the authors identified language areas in 14.5% of patients tested. Between 1.5 and 3.5 cm from the temporal tip, patients who had seizure onset before 6 years of age had more naming (p < 0.02) and reading (p < 0.01) areas than those in whom seizure onset occurred after age 6 years. Patients with a verbal intelligence quotient (IQ) lower than 90 had more naming (p < 0.05) and reading (p < 0.02) areas than those with an IQ higher than 90. Finally, patients who were either left handed or right hemisphere memory dominant had more naming (p < 0.05) and reading (p < 0.02) areas than right-handed patients with bilateral or left hemisphere memory lateralization. Postoperative neuropsychometric testing showed a trend toward a greater decline in naming ability in patients who were least likely to have anterior language areas, that is, those with higher verbal IQ and later seizure onset. CONCLUSIONS: Preoperative identification of markers of left hemisphere damage, such as early seizure onset, poor verbal IQ, left handedness, and right hemisphere memory dominance should alert neurosurgeons to the possibility of encountering essential language areas in the anterior temporal lobe (1.5-3.5 cm from the temporal tip). Naming and reading tasks are required to identify these areas. Whether removal of these areas necessarily induces long-term impairment in verbal abilities is unknown; however, in patients with a low verbal IQ and early seizure onset, these areas appear to be less critical for language processing.     

Psychopathology in pediatric complex partial and primary generalized epilepsy

Structured psychiatric interviews were administered to 60 children with complex partial seizure disorder (CPS), 40 children with primary generalized epilepsy with absences (PGE), and 48 control children, aged 5 to 16 years. Significantly more patients with epilepsy had psychiatric diagnoses compared with the control children. There were no statistically significant differences, however, in the number of patients with CPS and PGE with psychiatric diagnoses. Other than a schizophrenia-like psychosis found only in the patients with CPS, the two groups of patients had similar psychiatric diagnoses. The presence of psychopathology was related to significantly lower IQ scores and socioeconomic status, but not to seizure-related factors. These findings suggest that the psychopathology of children with CPS and PGE reflects different subtle neuropsychological deficits.     

Juvenile myoclonic epilepsy

CONCLUSION: We conclude that despite inevitable variability the clinical picture of JME is characteristic. It is easy to diagnose JME if one thinks of it while the history should be thoroughly analyzed. An EEG recording during sleep confirms the diagnosis. An early diagnosis of JME permits adequate prognosis of the subsequent course of epilepsy, and adequate therapy brings remission in most of the patients. If treatment starts following the large number of severe GTC seizures, the response to therapy is incomplete. The persistency of the illness throughout the life, the need for continuous medication and therapeutic unresponsiveness in cases with late diagnosis, do not justify the increasing misconception that JME is of benign nature. Diagnosis of JME is rare because of insufficient familiarily of physicians with the illness. BACKGROUND: Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epileptic syndrome characterized with the combination of myoclonic, generalized tonic-clonic (GTC) and absence seizures that are readily provoked by sleep deprivation. PATIENTS: Forty-three patients, aged from 14 to 51 years, participated in a 5-year follow-up study. Diagnosis was made according to the criteria (Table 1) for diagnosis of JME set by Panayiotopoulos et al. (1994). Nineteen patients made their first contact with a neurologist at the Institute of Neurology and were diagnosed as JME, while the remaining 24 were referred to from other medical institutions with a diagnosis of therapy resistant to focal epilepsy. All patients underwent a somatic and neurological examination, "mini mental test," EEG in waking and CT scan of the brain. Some patients had EEG performed during sleep and some had MRI of the head. RESULTS: JME began between 9 and 26 (average 17) years. All patients had myoclonic seizures, 98% had GTC and 23% absence seizures. The first myoclonic seizure occurred between 9 and 24 years while the frst GTC seizure occurred between 10 and 32 years. Myoclonic seizures (83% of patients) and GTC seizures (70% of patients) occurred most often immediately after awaking. The most frequent provocative factors were insufficient sleep, alcohol abuse and tiredness. Epilepsy in the family was present in 39%, focal neurological deficiency in 9% and pathological findings on of CT and MRI in 7% of patients. Waking EEG was pathological in 77% of patients; it included generalized spike-wave discharges in 73%, multiple spike-wave complexes in 33% and focal discharges in 12% of patients, respectively. In all 26 patients tested, sleep EEG was pathological most often with multiple spike-wave complexes in 85% and 3-4 Hz spike-wave complexes in 57% of patients. The correct diagnosis of JME following a comprehensive examination was made in 24 (56%) patients after a delay of 1 to 35 years. In 24 patients with delayed diagnosis of JME the replacement of earlier medication with valproic acid (VPA) induced remission in 18 patients (75%) while 1 patient (4%) experienced a reduction in the number of seizures. Five patients (21%) did not respond to VPA medication: 2 due to a weak compliance, another 2 due to inefficient medication and 1 because of the preexistent malabsorption syndrome. In 19 patients (44%) with initial diagnosis of JME, VPA was introduced immediately upon diagnosis. Of them, 15 (79%) had excellent response to VPA, 1 refused therapy and for 3 patients there is no information. In 2 patients VPA was substituted due to side effects (hepatotoxicity and alopetia) with lamotrigine (low doses), which brought about decrease in frequency and mitigation in myoclonic seizures.     

Complexity of focal spikes suggests relative epileptogenicity

The EEGs of 39 children with focal or multifocal spikes were subjected to singular value decomposition (SVD) as provided by a commercial software program. We noted that in children with spikes but no clinical seizures the variance accounted for by the first component averaged 91.9%, whereas in children with seizures it was 68.0% (p < .001). The first component accounted for 85.4% in children with single spike foci, for 71.5% in those with multifocal spikes, and for 61.4% (p < 0.002) in those with both focal spikes and generalized spike-wave complexes. Spikes in the frontal and frontopolar areas were the most complex, suggesting that at least in children they tend to be the partial expression of a generalized seizure tendency rather than a result of strictly local pathology.     

Epileptic syndromes which show continuous spike and wake complexes during slow wave sleep

INTRODUCTION AND OBJECTIVE: The association of cognitive and/or behavioral disturbances, epileptic seizures and continuous discharges spike-waves during slow sleep in the EEG, has been described in three epileptic syndromes: continuous spike-wave during slow sleep syndrome (CSWS), Landau-Kleffner syndrome (LKS) or acquired epileptic aphasia, and atypical benign partial epilepsy of childhood (ABPE). MATERIAL AND METHODS: We report 13 patients; two of these have been diagnosed of CSWS. four of LKS and seven of ABPE, according to the definition criteria. RESULTS: The main clinical features in each patient are presented here. In all patients, continuous spike-wave discharges were observed for more than > 80% of the total sleep period at some time during the course of the illness. There are many similarities at the age of onset, the occurrence of several types of seizures, the evolution and the electroencephalographic findings in awake state and sleep. CONCLUSIONS: It suggests that the CSWS, LKS and ABPE have probably the same pathophysiology and that they are the severe, moderate and benign forms of a single epileptic syndrome, age-related, with continuous spike waves and various neuropsychological and behavioral disturbances. The neuropsychological manifestations depend probably on the localization of the original epileptic focus.     

Sex difference in force generation capacity during repeated maximal knee extensions

PURPOSE: To define the risk of seizure recurrence (RSR) that families and physicians would accept before discontinuing antiepileptic drugs (AEDs) for children with controlled epilepsy. METHODS: A questionnaire was completed by families of 76 children with epilepsy > or = 3 months seizure-free and by their attending epilepsy specialist (n = 4). RESULTS: Forty-two percent of families were unwilling to discontinue AEDs with an RSR of 25%. In contrast, 20% were willing to accept a > 75% RSR. Several factors differentiated the risk acceptable to families: previous seizure frequency (risk adverse with intermediate frequency), multiple seizure types (risk taking), grade or grades repeated in school (risk adverse), and the family's strategy of playing lotteries. Although families and physicians were prepared to accept similar median RSR (35 and 40%, respectively), individual answers did not correlate (r2 = -0.07). Physicians were unable to predict the families response (r2 = 0.09). CONCLUSIONS: Our current practice is to discontinue AEDs after 2 years of seizure-free results in seizure recurrence of 30-40%. This risk may seem excessive to more than half of families, whereas other families will risk stopping AEDs at higher risks of recurrence. Physicians are poor judges of the degree of risk that is acceptable to a particular family, which may account in part for the anxiety manifested by families at AED discontinuation.     

Haloperidol induces changes in the electrocorticogram of rats with genetic petit mal epilepsy

The WAG/Rij rats, a genetic animal model of human absence epilepsy, were injected with D2 autoreceptor antagonist haloperidol. Increase in the amount of spike-wave discharges and the prevalence of short spike-wave discharges was observed. The pattern of spike-wave discharge sequence changed in a characteristic way. The findings may testify to participation of dopaminergic system both in the processes of generation and cessation of the spike-wave complex absence epilepsy.     

Changes in seizure frequency and test-retest scores on the Wechsler Adult Intelligence Scale

Test-retest performance on the Wechsler Adult Intelligence Scale (WAIS) of two groups of adult epilepsy patients are presented and compared. In one group, Seizures Improved (SI) group, seizure frequency had decreased during the test-retest interval, and in the other group, Seizures Unimproved (SU) group, the number of seizures had either increased or stayed the same over the test-retest interval. The SI group showed a significant test-retest improvement on WAIS Verbal IQ, Performance IQ, and Full Scale IQ, as well as on eight of 11 WAIS subtests. In comparison, the SU group showed significant increases only on the Performance IQ and Object Assembly subtest. Furthermore, differences between the two groups were observed in the pattern of test-retest changes seen on the Performance measures relative to the Verbal measures. The results suggest that change in seizure frequency is one of the factors associated with test-retest changes in the intellectual functioning of epilepsy patients.     

Childhood epilepsy and asthma: comparison of quality of life

We report results from the first data collection on an ongoing longitudinal study aimed at describing the natural history of adaptation to childhood epilepsy and asthma in children and their families. We studied 136 children with epilepsy and 134 children with asthma aged 8-12 years. Data were collected from the children, their mothers, and their school teachers through interviews, school records, and questionnaires. The two samples were compared on four domains of quality of life: physical, psychological, social, and school. Data were analyzed by a 2 x 2 between-subjects multivariate analysis of covariance with type of illness (epilepsy or asthma) as the independent variable and length of time since onset of illness as a covariate. A significant main effect was noted for illness [multivariate F (15, 236) = 11.36, p < 0.001]. Our major finding was that children with epilepsy had a relatively more compromised quality of life in the psychological, social, and school domains. In contrast, children with asthma had a more compromised quality of life in the physical domain. Our findings suggest that attention simply to seizure control in the clinical setting will not address the full range of quality-of-life problems of children with epilepsy.     

Dynamic cerebral autoregulation during sevoflurane anesthesia: a comparison with isoflurane

OBJECTIVE: To study the incidence and pattern of epilepsy in patients with periventricular leukomalacia (PVLM) in two specialty clinic settings. BACKGROUND: Motor and cognitive deficit as well as epilepsy are common in patients with PVLM. With modern imaging techniques, PVLM is now easily recognized. METHODS: Epileptic seizures and syndromes as well as motor and cognitive deficits were correlated with MRI findings. Two patient populations were studied: Group A-children with cerebral palsy and PVLM presenting to a center for children with motor disability (n = 19); and Group B-epileptic patients with PVLM presenting to a tertiary epilepsy center (n = 12). A single patient with PVLM and epilepsy who underwent extensive investigations, including intracranial EEG telemetry, is reported. RESULTS: In Group A, 47% of patients had epilepsy (9/19). PVLM was found in 1.27% of patients investigated for epilepsy at a tertiary epilepsy center (12/942). The majority of patients in both groups had multiple seizure types, with complex partial seizures being most common. Of patients with seizures (Groups A and B), 85.7% had intractable epilepsy (18/21). Intracranial EEG in the illustrative case demonstrated a multifocal epileptic process with occipitotemporal predominance. CONCLUSIONS: PVLM was an uncommon underlying cause in patients presenting with epilepsy (Group A); however, patients presenting with motor disability and PVLM (Group B) had a high incidence of seizures. PVLM in epileptic patients is associated with multiple seizure types and medically refractory disease.     

ILAE-defined epilepsy syndromes in children: correlation with quantitative MRI

PURPOSE: The role of quantitative magnetic resonance imaging (MRI) in evaluation of childhood epilepsy remains poorly defined, with minimal published data. Previous work from our center questioned the specificity of hippocampal asymmetry (HA) in an outpatient group whose epilepsy was defined by using clinical and interictal data only. By using childhood volunteer controls and defining epilepsy syndromes using video-EEG monitoring, we readdressed the utility of HA in differentiating mesial temporal lobe epilepsy (MTLE) from other partial and generalized epileptic syndromes in children. METHODS: Seventy children were enrolled; entry criteria were age younger than 18 years with predominant seizure type recorded on video-EEG telemetry with volumetric MRI in all cases. Thirty healthy child volunteers had volumetric MRI. Epilepsy syndrome classification was according to ILAE. RESULTS: Control data revealed symmetric hippocampi, mean smaller/larger ratio of 0.96 (0.95-0.97, 95% CI) with no gender or right/left predominance. Overall 23% of patients had significant HA. Mean hippocampal ratio for MTLE was 0.78 (95% CI, 0.70-0.86), significantly lower than controls and from all other epilepsy syndromes. HA was highly specific (85%) to the syndrome of MTLE. Other potential epileptogenic lesions were found in 27 (39%) patients, lowest yield in frontal and mesial temporal syndromes. Dual pathology was present in 10% of patients. There was no significant association between HA and risk factors. CONCLUSIONS: In this study, we found that HA in children with a well-defined epilepsy syndrome is highly sensitive and specific for MTLE. Whether this will correlate with surgical outcome, as in adults, is the subject of ongoing study.