Epileptic spasms without hypsarrhythmia in infancy and childhood: tonic spasms as a seizure type.

Epileptic spasms were defined by the International League Against Epilepsy Task Force on Classification and Terminology in 2001 as a specific seizure type. Epileptic spasms without hypsarrhythmia have been described in some series of patients, occurring either in infancy or childhood. More prolonged epileptic spasms without hypsarrhythmia were previously defined as a different seizure type, and referred to as "tonic spasm seizures". Here, we present a 5-year-old boy who started having epileptic spasms without hypsarrhythmia at 8 months of age, effectively treated with oxcarbazepine. With the withdrawal of medication, epileptic spasms returned. Video-EEG monitoring revealed high-voltage slow waves superimposed by low-voltage fast activity, followed by an electrodecremental phase and a burst of asymmetric fast activity, time-locked to clinical tonic spasm seizures. Brain MRI showed left temporal atrophy with temporal pole grey/white matter junction blurring and ictal PET-CT showed left basal frontal hypermetabolism. Seizures were refractory to several AEDs and vigabatrin was introduced with seizure cessation. Despite efforts to classify epileptic spasms, these are still considered as part of the group of unknown seizure types. In some cases, a focal origin has been suggested, leading to the term "periodic spasms" and "focal spasms". In this case, epileptic spasms without hypsarrhythmia, associated with tonic spasms, may be a variant of focal spasms and might be considered as an epileptic syndrome. [Published with video sequence].

Personality traits in juvenile myoclonic epilepsy: evidence of cortical abnormalities from a surface morphometry study.

Cluster B personality disorders (PD), characterized as emotional instability, immaturity, lack of discipline, and rapid mood changes, have been observed among patients with juvenile myoclonic epilepsy (JME) and have been associated with a worse seizure outcome. Proper understanding of the neurobiology of PD associated with JME could contribute to understanding the basis for earlier and more effective interventions. In the present study, volumetric and geometric features of cortical structure were assessed through an automated cortical surface reconstruction method aiming to verify possible structural cortical alterations among patients with JME. Twenty-two patients with JME with cluster B PD, 44 patients with JME without psychiatric disorders, and 23 healthy controls were submitted to a psychiatric evaluation through SCID I and SCID II and to an MRI scan. Significant cortical alterations in mesiofrontal and frontobasal regions, as well as in other limbic and paralimbic regions, were observed mainly in patients with JME with PD. The present study adds evidence to the hypothesis of frontal and limbic involvement in the pathophysiology of cluster B PD in JME, regions linked to mood and affective regulation, as well as to impulsivity and social behavior. Moreover, a multidimensional pattern of frontal, limbic, and paralimbic changes was observed through a method of structural analysis which offers different and simultaneous geometric features, allowing the elaboration of important pathophysiologic insights about cluster B PD in JME.

Auras in temporal lobe epilepsy with hippocampal sclerosis: relation to seizure focus laterality and post surgical outcome.

We examined the relationship between presence and frequency of different types of auras and side of lesion and post surgical outcomes in 205 patients with medically intractable mesial temporal lobe epilepsy (MTLE) with unilateral hippocampal sclerosis (HS). With respect to the number of auras, multiple auras were not associated with side of lesion (p=0.551). The side of HS was not associated with the type of auras reported. One hundred fifty-seven patients were operated. The occurrence of multiple auras was not associated with post-surgical outcome (p=0.740). The presence of extratemporal auras was significantly higher in patients with poor outcome. In conclusion, this study suggests that the presence of extratemporal auras in patients with MTLE-HS possibly reflects extratemporal epileptogenicity in these patients, who otherwise showed features suggestive of TLE. Therefore, TLE-HS patients undergoing pre-surgical evaluation and presenting clinical symptoms suggestive of extratemporal involvement should be more extensively evaluated to avoid incomplete resection of the epileptogenic zone.

Common infectious and parasitic diseases as a cause of seizures: geographic distribution and contribution to the burden of epilepsy

This educational review article aims to provide information on the central nervous system (CNS) infectious and parasitic diseases that frequently cause seizures and acquired epilepsy in the developing world. We explain the difficulties in defining acute symptomatic seizures, which are common in patients with meningitis, viral encephalitis, malaria, and neurocysticercosis, most of which are associated with increased mortality and morbidity, including subsequent epilepsy. Geographic location determines the common causes of infectious and parasitic diseases in a particular region. Management issues encompass prompt treatment of acute symptomatic seizures and the underlying CNS infection, correction of associated predisposing factors, and decisions regarding the appropriate choice and duration of antiseizure therapy. Although healthcare provider education, to recognize and diagnose seizures and epilepsy related to these diseases, is a feasible objective to save lives, prevention of CNS infections and infestations is the only definitive way forward to reduce the burden of epilepsy in developing countries.

Psychoses of epilepsy: a study comparing the clinical features of patients with focal versus generalized epilepsies.

In the literature, psychosis of epilepsy (POE) has been described as one of the most frequent psychiatric comorbidities of epilepsy, occurring particularly in association with temporal lobe epilepsy. However, the presence of such psychiatric disorders among patients with idiopathic generalized epilepsies has also been mentioned. In this study, we evaluated the clinical features of psychotic disorders in a series of patients with temporal lobe epilepsy related to mesial temporal sclerosis (TLE-MTS) and juvenile myoclonic epilepsy with the aim of describing and comparing the clinical patterns of the psychotic symptoms in such frequent and important epilepsy syndromes. POE occurred most frequently in patients with TLE-MTS (P=0.01), but no differences were observed between the groups with respect to the subtypes and core symptoms of psychoses. The clinical implications of POE in both epilepsy syndromes are discussed.

Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms.

A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences].

Are personality traits of juvenile myoclonic epilepsy related to frontal lobe dysfunctions? A proton MRS study.

PURPOSE: Personality traits characterized by emotional instability and immaturity, unsteadiness, lack of discipline, hedonism, frequent and rapid mood changes, and indifference toward one's disease have been associated with patients who have juvenile myoclonic epilepsy (JME). Literature data demonstrate worse seizure control and more psychosocial dysfunctions among patients with JME who have those traits. In this controlled study we performed a correlation analysis of psychiatric scores with magnetic resonance spectroscopy (MRS) values across JME patients, aiming to verify the existence of a possible relation between frontal lobe dysfunction and the prevalence of personality disorders (PDs) in JME. METHODS: Sixteen JME patients with cluster B PDs, 41 JME patients without any psychiatric disorder, and 30 healthy controls were submitted to a psychiatric evaluation and to a quantitative multivoxel MRS of thalamus; insula; cingulate gyrus; striatum; and frontal, parietal, and occipital lobes. Groups were homogeneous according to age, gender, and manual dominance. Psychiatric evaluation was performed through the Scheduled Clinical Interview for DSM-IV, Axis I and II (SCID I and II, respectively). RESULTS: A significant reduction of N-acetyl-aspartate over creatinine (NAA/Cr) ratio was observed mainly in the left frontal lobe in the JME and PD group. In addition, a significant increase in the glutamate-glutamine over creatinine GLX/Cr ratio was also observed in this referred region in the same group. DISCUSSION: These data support the hypothesis that PDs in JME could represent neuronal dysfunction and possibly a more severe form of this epileptic syndrome.

Personality traits related to juvenile myoclonic epilepsy: MRI reveals prefrontal abnormalities through a voxel-based morphometry study.

Studies involving juvenile myoclonic epilepsy (JME) patients have demonstrated an elevated prevalence of cluster B personality disorders (PD) characterized as emotional instability, immaturity, unsteadiness, lack of discipline, and rapid mood changes. We aimed to verify a possible correlation between structural brain abnormalities in magnetic resonance image (MRI) and the PD in JME using voxel-based morphometry (VBM). Sixteen JME patients with cluster B PD, 38 JME patients without psychiatric disorders, and 30 healthy controls were submitted to a psychiatric evaluation through SCID I and II and to a MRI scan. Significant reduction in thalami and increase in mesiofrontal and frontobasal regions' volumes were observed mainly in JME patients with PD. Structural alterations of the orbitofrontal cortex (OFC), involved in regulation of mood reactivity, impulsivity, and social behavior, were also observed. This study supports the hypothesis of frontobasal involvement in the pathophysiology of cluster B PD related to JME.

Correlation between temporal pole MRI abnormalities and surface ictal EEG patterns in patients with unilateral mesial temporal lobe epilepsy.

OBJECTIVE: The objective of this retrospective study is to analyze ictal patterns observed during continuous Video-EEG monitoring in patients with temporal lobe epilepsy (TLE) due to unilateral hippocampal sclerosis (HS), and to correlate these EEG patterns to temporal pole abnormalities observed on magnetic resonance imaging exams. METHODS: We analyzed 147 seizures from 35 patients with TLE and unilateral HS. Ictal patterns were classified and correlated to signal abnormalities and volumetric measures of the temporal poles. Volume differences over 10% were considered abnormal. RESULTS: The most frequent type of ictal pattern was rhythmic theta activity (RTA), encountered in 65.5% of the seizures. Rhythmic beta activity (RBA) was observed in 11% of the seizures, localized attenuation in 8%, interruption of epileptiform discharges in 6%, repetitive discharges in 5.5%, and rhythmic delta activity (RDA) in 4%. Sixty-six percent of the patients presented signal abnormalities in the temporal pole that were always ipsilateral to the HS. Sixty percent presented significant asymmetry of the temporal poles consisting of reduced volume that was also always ipsilateral to HS. Although patients with RTA as the predominant ictal pattern tended to present asymmetry of temporal poles (p=0.305), the ictal EEG pattern did not correlate with temporal pole asymmetry or signal abnormalities. CONCLUSIONS: RTA is the most frequent initial ictal pattern in patients with TLE due to unilateral HS. Temporal pole signal changes and volumetric reduction were commonly found in this group of patients, both abnormalities appearing always ipsilateral to the HS. However, neither temporal pole volume reduction nor signal abnormalities correlated with the predominant ictal pattern, suggesting that the temporal poles are not crucially involved in the process of epileptogenesis.

Early destructive lesions in the developing brain: clinical and electrographic correlates.

OBJECTIVE: Early brain insults can cause cavitary lesions including porencephaly (POR) and multicystic encephalopathy (MCE). The objective of this study was to investigate clinical and electrographic correlates associated to these types of destructive brain lesions. METHOD: Patients with POR and MCE were selected and submitted to clinical and Video-EEG monitoring. The following variables were analyzed: demographic data, type of lesion, presence of gliosis, perinatal complications, epilepsy, brain atrophy, and presence and frequency of epileptiform discharges. RESULTS: Twenty patients were included, 65% males, 35% females, ages ranging from 1 to 40 years, 14 with MCE and 6 with POR. Eighteen patients had hemiparesis, 19 had epilepsy (current or in the past), seven of them had refractory seizures, and 16 had epileptiform discharges. All patients with MCE had gliosis while only 2 with POR had it. CONCLUSIONS: No correlation was observed between type of lesion and clinical and electrographical outcome. However, a positive correlation was observed between frequency of discharges and presence of brain atrophy, and between MCE and gliosis.

Self-perception of factors that precipitate or inhibit seizures in juvenile myoclonic epilepsy.

PURPOSE: To assess self-perception of factors that precipitate or inhibit seizures in patients with juvenile myoclonic epilepsy (JME). PATIENTS AND METHODS: Thirty-six males and 39 females with JME and mean age of 25.8+/-8.7 years were analysed. All patients completed a standardized questionnaire to assess for the presence or absence of precipitant or inhibitory factors for their seizures in a face-to-face interview. These data were statistically analysed through logistic and linear regression models and Phi coefficient. RESULTS: Ninety-two percent of the patients identified at least one precipitating factor (PF). In order of frequency the following PFs were recorded: stress (83%), sleep deprivation (77%), specific thoughts/mental concentration (23%), performance of hand activities and complex finger movements (20%), flashing lights and playing games (15%), speaking out in public (11%) and alcohol intake (11%), reading (7%), calculating and writing (5%), playing musical instruments (4%), drawing (3%), and specific types of music (1%). Menstrual cycle was the third most important PF in the women (33%). Although PFs were easily recognized, 77% of the patients stated that they were unable to avoid the occurrence of the seizures. CONCLUSIONS: Structured questionnaire is useful in stimulating patients to self-report seizure precipitants. Patients with higher education and uncontrolled seizures identified them more easily. The presence of a significant number of uncommon PFs, such as mental and motor hand tasks, considered uncommon for other epileptic syndromes, suggests that the role of these factors may be under-recognized in JME.

Language- and praxis-induced jerks in patients with juvenile myoclonic epilepsy.

Reflex traits have been described in patients with idiopathic generalized epilepsy. We report on four patients with juvenile myoclonic epilepsy in whom the coexistence of praxis- and language-induced jerks was documented in video-polygraphic EEG recordings.[Published with video sequences].

Topiramate for the treatment of juvenile myoclonic epilepsy.

OBJECTIVE: The aim of this study was to evaluate the efficacy and tolerability of topiramate (TPM) in juvenile myoclonic epilepsy (JME). METHOD: We assessed seizure control and adverse effects of TPM in 22 patients (18 females) aged 13 to 53 years. Target TPM dosage was up to 200 mg/day. The patients were subdivided into 3 groups: those treated with seizure control plus side effects (n=4); treated with non-controlled seizures (n=15) and with JME newly diagnosed (n=3). RESULTS: Sixteen patients completed the first year of the follow-up. Generalized tonic-clonic seizures were completely controlled in 10 (62.5%); more than 50% of reduction in 4 (25.0%) and less than 50% in 2 (12.5%). Myoclonia were controlled in 11 (68.8%) and persisted in 5 (31.2%) patients. Absence seizures were present in 5 (22.7%) of whom 2 (9.0%) showed more than 50% of seizure reduction while 3 (13.6%) presented worsening. Discontinuations were due to inadequate seizure control and adverse events (N=4), low compliance and loss of follow-up (N=2) and subject choice (N=1). CONCLUSION: TPM showed to be an effective and well-tolerated drug in the treatment of JME. Although frequently observed, TPM side effects were tolerable and the drug could be maintained in the majority of patients.

Temporopolar blurring in temporal lobe epilepsy with hippocampal sclerosis and long-term prognosis after epilepsy surgery.

PURPOSE: We conducted a retrospective study in order to investigate the clinical significance of temporopolar grey/white matter abnormalities (GWMA) in patients with temporal lobe epilepsy (TLE) and unilateral hippocampal sclerosis (HS) with a long post-surgical follow-up. METHODS: The study comprised 122 consecutive patients with medically refractory TLE and unilateral HS who underwent epilepsy surgery and had a minimum postoperative follow-up of 5 years. Patients were divided into two groups, based on findings of pre-surgical MRI: group 1 with GWMA and 2 with normal signal and grey/white matter definition in temporal pole. Demographic and clinical data were reviewed and compared between groups. RESULTS: GWMA were found in 52.5% of patients, always ipsilateral to HS. Compared with group 2, group 1 patients had earlier epilepsy onset (mean, 9.3 vs 14.4 years, P=0.001), a higher occurrence of first seizure ≤2 years of age (25.8% vs 10.5%, P=0.036; OR=2.96 [95% CI=1.07-8.19]), and greater prevalence of left HS (76.6% vs 43.1%, P<0.001; OR=4.31 [95% CI=1.98-9.38]). No differences were found in gender, presence or type of initial precipitating injury, history of secondary generalized seizures, duration of epilepsy, seizure frequency before surgery, neuropsychological evaluation and presence or lateralization of pre-surgical interictal epileptiform discharges. Postoperative follow-up varied from 5 to 11.5 years (mean 7.4) and was similar in both groups (P=0.155). The proportion of patients classified as seizure-free (Engel class I) at last follow-up in groups 1 and 2 were 73.4% and 69%, respectively (P=0.689). Similarly, the percentages of seizure-free patients with no antiepileptic drugs at last evaluation were not different between groups (P=0.817). In logistic regression analysis, left HS (P=0.001; OR=4.166 [95% CI=1.86-9.34]) and age at epilepsy onset ≤2 years (P=0.047; OR=3.885 [95% CI=1.86-17.50]) were independently associated with risk of having GWMA. CONCLUSION: GWMA are frequent findings in patients with TLE and HS, and may help lateralize the epileptogenic zone. Our data support the hypothesis that GWMA are caused by seizure-related insults during the critical period of cerebral myelination. GWMA did not influence the postoperative seizure outcome of patients with TLE and HS, even after an extended duration of post-surgical follow-up.

Refractory nonconvulsive status epilepticus in coma: analysis of the evolution of ictal patterns.

OBJECTIVE: Nonconvulsive status epilepticus (NCSE) is currently considered as one of the most frequent types of status epilepticus (SE). The objective of the present study was to identify the natural history of the electrographical evolution of refractory NCSE and to establish the relationship between ictal patterns and prognosis. METHODS: We analyzed, retrospectively, 14 patients with loss of consciousness and NCSE. The ictal patterns were classified as discrete seizures (DS), merging seizures (MS), continuous ictal discharges (CID), continuous ictal discharges with flat periods (CID-F), and periodic lateralized epileptiform discharges (PLEDs). RESULTS: The ictal patterns were DS (n=7; 50.0%), PLEDs (n=3; 1.4%), CID (n=2; 14.3%), MS (n=1; 7.1%), and CID-F (n=1; 7.1%). CONCLUSIONS: NCSE electrographic findings are heterogeneous and do not follow a stereotyped sequence. PLEDs were related to a higher probability of neurological morbidity and mortality.

Low educational level effects on the performance of healthy adults on a Neuropsychological Protocol suggested by the Commission on Neuropsychology of the Liga Brasileira de Epilepsia.

OBJECTIVE: To assess cognitive measures and impact of education on cognitive performance (CP) of low educational levels healthy adults (LELHA) on a Neuropsychological Protocol (NP) suggested by the Liga Brasileira de Epilepsia. METHOD: 138 subjects from an Education Program for Adults divided into two, age and gender matched groups of 69 subjects, answered the NP: Group 1 (mean of 6.9 ± 2.95 months of formal education) and Group 2, 47.8 ± 10 months. Data were compared as z-scores. RESULTS: The mean IQ was 77.1 ± 5.50 and 79.4 ± 3.30 in Groups 1 and 2, respectively (p=0.001). Both performed below the normal curve and Group 1 worse than 2. CP correlated with schooling, especially executive functions (54.1% vs 36.2%) and language (52.9% vs 25.7%). CONCLUSION: LELHA showed significant cognitive impairment in verbal and visuospatial areas. If these results had been obtained in epilepsy patients they would be interpreted as global cognitive impairment.

Prognosis of juvenile myoclonic epilepsy is related to endophenotypes.

PURPOSE: To investigate if phenotypic variations have prognostic implications in juvenile myoclonic epilepsy (JME). METHODS: Sixty-five consecutive JME patients had video-EEG recording and were followed for at least three years. Reflex traits were defined as seizures and/or EEG discharges induction by eye-closure, photic stimulation, language, praxis or calculation. Patients had psychiatric evaluation and answered to STAI (State-Trait Anxiety Inventory). Seizure control was classified according to Prasad et al. (2003).(24) Patients were divided into Group 1: good seizure control and Group 2: moderate or poor seizure control. Video-EEG/EEG evaluation was repeated in 21 patients. RESULTS: Forty of 65 (61.5%) patients reached good seizure control, 25 (38.5%) of whom became seizure free. Group 2 patients had longer epilepsy duration (13.9±9.0 vs. 8.7±8.2; p=0.019); higher prevalence of the combination of all three seizure types (72.0% vs. 30.0%; p=0.003); discharges in baseline EEG (56.0% vs. 22.5%; p=0.008); seizure recording (68% vs. 20%; p<0.001) and sensitivity to praxis (63.6% vs. 29.6%; p=0.023). Compared to seizure-free patients, those with persistent seizures presented younger age at epilepsy onset (12.6±3.33 years vs. 15.4±5.47 years; p=0.015); higher prevalence of personality disorders (25% vs. 4%; p=0.029); higher scores in STAI-T (45.9±11.31 vs. 36.6±11.43; p=0.011) and higher incidence of sensitivity to praxis (58.6% vs. 25.0%; p=0.04) and to language (53.8 vs. 16.7%; p=0.026) tasks. Repetition of EEG/video-EEG revealed a parallel evolution of reflex traits disappearance and seizure control. DISCUSSION: Clinical features and reflex traits have prognosis implications in JME.

Prevalence of epilepsy in children from a Brazilian area of high deprivation.

This study assessed the prevalence rate of epilepsy and its causes in children and adolescents in one area of high deprivation in São Paulo, São Paulo, in Southeast Brazil. Between July 2005 and June 2006, 4947 families from a population of 22,013 inhabitants (including 10,405 children and adolescents between the ages of 0 and 16 years) living in the shantytown of Paraisópolis, were interviewed. In the first phase, a validated questionnaire was administered, to identify the occurrence of seizures. In the second phase, clinical history, neurologic examination, electroencephalography, and structural neuroimaging were performed. The diagnosis of epilepsy, including etiology, seizure types, and epileptic syndrome classification, was according to criteria of the International League Against Epilepsy. The screening phase identified 353 presumptive cases. In the second phase, 101 of these cases (33.8%) received the diagnosis of epilepsy. Crude prevalence of epilepsy was 9.7/1000 and prevalence of active epilepsy was 8.7/1000. Partial seizures were the most frequent seizure type (62/101). Symptomatic focal epilepsy was the most common form, and hypoxic-ischemic encephalopathy the most common etiology, reflecting the socioeconomic conditions of this specific population. Adequate public policies regarding perinatal assistance could help reduce the prevalence of epilepsy.

The integrity of corpus callosum and cluster B personality disorders: a quantitative MRI study in juvenile myoclonic epilepsy.

Evidence suggests increased prevalence of cluster B personality disorders (PD) among patients with juvenile myoclonic epilepsy (JME), which has been associated with worse seizure control and more psychosocial dysfunctions. A preliminary voxel-based morphometry study demonstrated corpus callosum (CC) volume reduction in patients with JME and cluster B PD, particularly in the posterior midbody and isthmus. In this study we aimed to follow up these results with region of interest analysis. Sixteen JME patients with cluster B PD, 38 JME patients without any psychiatric disorder, and 30 demographically matched healthy controls submitted to a psychiatric evaluation and a magnetic resonance imaging scan. The total and regional callosal areas were obtained from the midsagittal slice using a semi-automated program. Psychiatric evaluation was performed through SCID-I and -II. Significant reductions in the posterior region of the CC were observed in the JME with PD group relative to the other groups. These data support previous findings of callosal reductions in cluster B PD, as well as a possible involvement of CC in patients with JME and such personality characteristics.