bluuebunny/crossref_metadata_2025 huggingface dataset:
Do Males 30–50 Years of Age with Chronic Epilepsy and on Long‐Term Anticonvulsant Medication Have Lower‐Than‐Expected Risk of Developing Coronary Heart Disease?
Authors: Nakken Karl Otto , Kornstad Stig
Published: August 2005

Summary: Purpose: It has been claimed that long‐term use of antiepileptic drugs (AEDs) brings about changes in the lipid profile which might resist development of cardiovascular disease. The aim of this study was to test the following hypothesis: Do males with chronic epilepsy and on long‐term treatment with AEDs have a lower than expected risk of developing coronary heart disease?

Methods: The coronary risk profile in 40 male patients with chronic epilepsy, aged 30–50 years, was explored, using an American individual coronary risk factor test program (the Cooper test). We made a survey of the patients' risk factors: total cholesterol, high density lipoprotein (HDL) cholesterol, triglycerides, glucose, blood pressure, body fat percentage, physical fitness, hereditary factors, smoking habits, and degree of stress. Each factor was given a score, and the sum of scores constituted the total coronary risk profile. Eighty men in the same age group, casually selected from an industrial medical service, served as controls.

Results: There was no statistically significant difference in the total coronary risk profile between the epilepsy patients and the controls. Nor were there any significant differences among the individual risk factors except for physical fitness level, which was significantly lower among the epilepsy group than among the controls. In the epilepsy group, however, there was a trend toward higher levels of total cholesterol, higher levels of HDL‐cholesterol, lower levels of total cholesterol/HDL‐cholesterol ratio, increased body fat percentage, greater heredity and somewhat greater coronary risk score as compared with the healthy controls, but the differences did not reach statistical significance. The protective effect against coronary heart disease exercised by the enzyme‐inducing AEDs seems to be counteracted by other factors, like reduced physical fitness and increased body fat percentage.

Conclusions: Our hypothesis was not confirmed, because we found that the 40 patients with chronic epilepsy tested had about the same risk of developing coronary heart disease as did the control group.
Brief Communication Complex Partial Status Epilepticus in Late‐Onset MELAS
Authors: Leff Alex P. , McNabb Andrew W. , Hanna Michael G. , Clarke Charles R. A. , Lamer Andrew J.
Published: August 2005

Summary: A patient with recurrent episodes of complex partial status epilepticus and a distinctive pattern of periodic lateralized epileptic form discharges (PLEDs) is presented. The patient was subsequently shown to have a mitochondria1 disorder of the MELAS type, a hitherto unreported association. The case illustrates that CPSE should be added to the list of possible causes of acute neurological deterioration in MELAS patients.
History of “Epileptic Vertigo”: Its Medical, Social, and Forensic Problems
Authors: Bladin Peter F.
Published: August 2005

Summary:We traced the history of the association of vertigo with the condition of epilepsy through the ages. In ancient times, vertigo was closely linked with epilepsy; indeed, it was believed to be the harbinger of chronic seizures. With the advent of modern scientific study of epilepsy initiated by the French in the 18th and 19th centuries, vertigo, not yet associated with disease of the inner ear or vestibular connections, assumed a specific role in the clinical gradation of seizure entities. It was believed to be the mildest form of epilepsy. Later, with the establishment of the conceptual linkage of “larval” or “masked” epilepsy with outbursts of violence, “epileptic vertigo” was considered the trigger for potentially lethal behavior and thus assumed a much‐feared reputation. Evidence for this abounds in the medical, legal, and even the popular literature at the end of the 19th century. The role of vertigo and its epileptic associations occupied the attention of most of the pioneer workers in epileptology of that era, and it was finally agreed that as a symptom the inner ear rather than epilepsy underlay its causation. Even today, epilepsy and vertigo are conceptually associated, sometimes erroneously.
Retrospective Analysis of the Effects of Psychotherapeutic Interventions on Outcomes of Psychogenic Nonepileptic Seizures
Authors: Aboukasm Amer , Mahr Gregory , Gahry Barbara Rader , Thomas Andrea , Barkley Gregory L.
Published: August 2005

Summary: Purpose: Uncertainty exists regarding the effectiveness of psychotherapy in patients with psychogenic nonepileptic seizures (PNES). We retrospectively reviewed the outcomes of therapeutic interventions in a cohort of patients with PNES.

Methods: We identified patients with PNES by outpatient video‐EEG (VEEG) between 1992 and 1996. We retrospectively analyzed the effects on outcomes of various types of interventions for PNES administered at random, using a structured interview and review of medical records. Patients with subjective events and severe psychosis were excluded. Patients who required inpatient monitoring because the outpatient VEEG was inconclusive were also excluded.

Results: One hundred patients were diagnosed with PNES between 1992 and 1996: 61 patients completed the study. The average age was 43.3 years, and 90% were female. Nine (15%) had had PNES <1 year, and 7 (11%) had epilepsy. Eleven of 16 patients (68%) who received five or more counseling sessions, including showing the videotaped recording of the nonepileptic spell (13 of 16) by the comprehensive epilepsy program (CEP) psychotherapist had cessation (7 of 16) or reduction (4 of 16) of their episodes. Eleven of 15 patients (73%) who received only the feedback and care of their CEP neurologists had similar favorable outcome. Twelve of 25 patients (48%) who received counseling by a non‐CEP psychotherapist or psychiatrist had cessation (10 of 25) or reduction (2 of 25) of episodes. Five patients who received no feedback or intervention had no improvement (4 of 5) or worsening (1 of 5) of the PNES.

Conclusions: Psychotherapy or feedback provided by CEP professionals with experience in epilepsy and PNES improves outcome and may be superior to other or no interventions.
Presurgery Expectations, Postsurgery Satisfaction, and Psychosocial Adjustment After Epilepsy Surgery
Authors: Wheelock Ingrid , Peterson Christopher , Buchtel Henry A.
Published: August 2005

Summary: Purpose: The medical benefits of epilepsy surgery are well documented, but the psychosocial consequences of surgery have received less attention. This is especially true of the roles of expectations and satisfaction in postsurgery functioning. The present study was designed to examine the relationships between expectation, satisfaction with surgery, and psychosocial functioning in patients and their significant others before and after epilepsy surgery.

Methods: The neuropsychology findings of 79 patients undergoing epilepsy surgery were examined from assessments made before, 2 months after, and 1 year after anterior temporal lobectomy (ATL) was performed. The Minnesota Multiphasic Personality Inventory (MMPI) and the Washington Psychosocial Seizure Inventory (WPSI) were used to assess psychosocial functioning. A subset of 32 patients and their significant others were followed prospectively with an additional semistructured interview to assess expectations for and satisfaction with surgery outcome.

Results: Before surgery, patients showed considerable problems with psychosocial adjustment. After surgery, patients in the seizure‐free group showed improvements on the psychosocial measures at both follow‐ups, whereas patients with continued seizures showed improvement at the 2‐month follow ‐up and then a decline to baseline or worse at the time of the 1‐year follow‐up. Subjects who were studied prospectively had high presurgery expectations for seizure elimination. After surgery, subjects whose expectations were met reported high satisfaction whereas subjects whose expectations were not met reported low satisfaction. Satisfaction with surgery was associated with better psychosocial functioning.

Conclusions: Such data help elucidate the ways in which successful epilepsy surgery results in improved psychosocial functioning. The results identify ways to increase the likelihood that both patients who are seizure‐free and those with reduced seizure frequency may benefit from surgery.
The Efficacy of Lamotrigine in Children and Adolescents with Refractory Generalized Epilepsy: A Randomized, Double‐Blind, Crossover Study
Authors: Eriksson A‐S. , Nergårdh A. , Hoppu K.
Published: August 2005

Summary: Purpose: We report a double‐blind, placebo‐controlled crossover study of lamotrigine (LTG) as add‐on treatment in therapy‐resistant, generalized epilepsy in children and adolescents (n = 30).

Methods: Twenty patients had Lennox‐Gastaut syndrome. Each patient acted as his or her own control. LTG and placebo were randomly added to existing antiepileptic medication (AEDs). The LTG dosage was individualized in an open phase preceding the placebo/treatment phase. Patients who responded to LTG in the open phase went on to the double‐blind phase. “Responders” were defined as patients with a >50% seizure reduction or less severe seizures or both, or improved behavior or improved motor skills or both. “Nonresponders” were defined as children who showed no positive effects of LTG with plasma levels of 10 μg/ml or children who had adverse events during the open phase.

Results: There was a clear statistically significant reduction of seizure frequency in LTG compared with placebo treatment. None of the children studied showed abnormal biochemical or hematologic findings, or changes in plasma levels of concomitantly administered AEDs.

Conclusions: LTG is a well‐tolerated and effective treatment in children with intractable generalized epilepsies, including those with Lennox‐Gastaut syndrome. The study design allowed a double‐blind placebo‐controlled assessment of LTG although the participating children used 19 different AED combinations at entry.
[31P]/[1H] Nuclear Magnetic Resonance Study of Mitigating Effects of GYKI 52466 on Kainate‐Induced Metabolic Impairment in Perfused Rat Cerebrocortical Slices
Authors: Tang Pei , Liachenko Serguei , Melick John A. , Xu Yan
Published: August 2005

Summary: Purpose: Kainic acid (KA) has long been used in experimental animals to induce status epilepticus (SE). A mechanistic implication of this is the association between ex‐citotoxicity and brain damage during or after SE. We evaluated KA‐induced metabolic impairment and the potential mitigating effects of GYKI 52466 [1‐(4‐aminophenyl)‐4‐methyl‐7,8‐methylenedioxy‐5H‐2,3‐benzodiazepine] in superfused rat cerebral cortical slices.

Methods: Interleaved [31P]/[1H] magnetic resonance spectroscopy (MRS) was used to assess energy metabolism, intra‐cellular pH (pHi), N‐acetyl‐L‐aspartate (NAA) level, and lactate (Lac) formation before, during, and after a 56‐min exposure to 4 mM KA in freshly oxygenated artificial cerebrospinal fluid (OXY‐ACSF).

Results: In the absence of GYKI 52466 and during the KA exposure, NAA, PCr, and ATP levels were decreased to 91.1 ± 0.8, 62.4 ± 3.9, and 59.1 ± 4.3% of the control, respectively; Lac was increased to 118.2 ± 2.1%, and pH, was reduced from 7.27 ± 0.02 to 7.13 2 0.02. During 4‐h recovery with KA‐free ACSF, pHi rapidly and Lac gradually recovered, NAA decreased further to 85.5 ± 0.3%, and PCr and AW showed little recovery. Removal of Mg2+ from ACSF during KA exposure caused a more profound Lac increase (to 147.1 ± 4.0%) during KA exposure and a further NAA decrease (to 80.4 ± 0.5%) during reperfusion, but did not exacerbate PCr, ATP, and pH, changes. Inclusion of 100 μM GYKI 52466 during KA exposure significantly improved energy metabolism: the PCr and ATP levels were above 76.6 ± 2.1 and 82.0 & 2.9% of the control, respectively, during KA exposure and recovered to 101.4 ± 2.4 and 95.0 ± 2.4%, respectively, during reperfusion. NAA level remained at 99.8 ± 0.6% during exposure and decreased only slightly at a later stage of reperfusion.

Conclusions: Our finding supports the notion that KA‐induced SE causes metabolic disturbance and neuronal injury mainly by overexcitation through non‐N‐methy1‐D‐aspartate (NMDA) receptor functions.
Symptoms of Depression and Anxiety in Pediatric Epilepsy Patients
Authors: Ettinger Alan B. , Weisbrot Deborah M. , Nolan Edith E. , Gadow Kenneth D. , Vitale Susan A. , Andriola Mary R. , Lenn Nicholas J. , Novak Gerald P. , Hermann Bruce P.
Published: August 2005

Summary: Purpose: We assessed rates of symptoms of anxiety and depression among pediatric patients with epilepsy.

Methods: We administered the Revised Child Manifest Anxiety Scale (RCMAS), and Child Depression Inventory (CDI) to 44 epilepsy patients aged 7–18 years (mean age 12.4 years). Demographic, socioeconomic, and epilepsy‐related information was examined in relation to depression and anxiety scores.

Results: No patients had been previously identified to have depression or anxiety. However, 26% had significantly increased depression scores and 16% met critieria for significant anxiety symptomatology.

Conclusions: Symptoms of depression and anxiety are common among pediatric patients with epilepsy and appear to be overlooked by care providers.
Real‐Time Automated Detection and Quantitative Analysis of Seizures and Short‐Term Prediction of Clinical Onset
Authors: Osorio Ivan , Frei Mark G. , Wilkinson Steven B.
Published: August 2005

Summary:Purpose:We describe an algorithm for rapid realtime detection, quantitation, localization of seizures, and prediction of their clinical onset.

Methods:Advanced digital signal processing techniques used in time‐frequency localization, image processing, and identification of time‐varying stochastic systems were used to develop the algorithm, which operates in generic or adaptable “modes.” The “generic mode” was tested on (a) 125 partial seizures (each contained in a 10‐min segment) involving the mesial temporal regions and recorded using depth electrodes from 16 subjects, and (b) 205 ten‐minute segments of randomly selected interictal (nonseizure) data. The performance of the algorithm was compared with expert visual analysis, the current “gold standard.”

Results:The generic algorithm achieved perfect sensitivity and specificity (no false‐positive and no false‐negative detections) over the entire data set. Seizure intensity, a novel measure that seems clinically relevant, ranged between 35.7 and 6129. Detection was sufficiently rapid to allow prediction of clinical onset in 92% of seizures by a mean of 15.5 s.

Conclusions:This algorithm, which was implemented with a personal computer, represents a definitive step toward rapid and accurate detection and prediction of seizures. It may also enable development of intelligent devices for automated seizure warning and treatment and stimulate new study of the dynamics of seizures and of the epileptic brain.
Prolonged Coma and Severely Attenuated EEG After a Single Seizure
Authors: Arnoldus Edo P. J. , Bloem Bastiaan R. , van Dijk J. Gert , Lammers GertJan
Published: August 2005

Summary: A 63‐year‐old woman presented with a comatose state after a fall. Results of cranial computed tomography (CT) and magnetic resonance imaging (MRI) scans were normal. An EEG recorded 5 h after admission was very severely attenuated and slowed. Consciousness and EEG were improved the next day. No cause was detected initially. After sleep deprivation, the patient had a generalized seizure followed by a similar coma and EEG. Even a single seizure may cause a prolonged coma with a very severely attenuated and slowed EEG.
Antiepileptic Drugs, Learning, and Behavior in Childhood Epilepsy
Authors: Bourgeois Blaise F. D.
Published: August 2005

Summary: Cognitive and behavioral impairments are found more often among epileptic children than among their peers. The cause of these impairments is multifactorial. Identifying the relative contribution of antiepileptic drugs (AEDs) to these problems has been the object of a large number of clinical investigations. This area of research has been characterized by an unusually high number of methodological challenges and pitfalls. Accordingly, results have often been inconsistent and contradictory, except for the more obvious observations that can be derived from clinical experience. Overall, the effects of AEDs on cognition and behavior in children have been overrated in the past. More recent research has benefited from the methodological lessons of previous studies and it suggests that the majority of children taking AEDs do not experience clinically relevant cognitive of behavioral adverse effects from these medications. In addition, some of the newer AEDs may indeed have a better cognitive profile. Nevertheless, clinical experience must be used to identify the subgroup of children who remain at risk for significant and clinically relevant cognitive and behavioral adverse effects of AEDs.
Chromosome 20 Ring: A Chromosomal Disorder Associated with a Particular Electroclinical Pattern
Authors: Canevini Maria Paola , Sgro Vincenzo , Zuffardi Orsetta , Canger Raffaele , Carrozzo Romeo , Rossi Elena , Ledbetter David , Minicucci Fabio , Vignoli Aglaia , Piazzini Ada , Guidolin Loredana , Saltarelli Amalia , Bernardina Bernardo dalla
Published: August 2005

Summary: Purpose: The chromosome 20 ring [r(20)] is a rare chromosomal disorder without clear phenotypical markers. We describe the electroclinical pattern in a group of patients with r(20).

Methods: We observed 3 patients (a boy, patient 1; his mother, patient 2; and an unrelated man, patient 3), performing prolonged video‐EEG and cytogenetic studies and fluorescent in situ hybridization (FISH) with chromosome‐specific telomeric probes.

Results: All 3 patients had a very similar abnormal electroclinical pattern characterized by long bursts or trains of rhythmic theta waves, which were sharply contoured or had a notched appearance (with no detectable clinical correlate), and generalized spike waves (SW) associated with seizures of probable frontotemporal origin (SFT). In all 3 patients, the cytogenetic analysis of T lymphocytes showed mosaicism with a normal cell line and a second cell line with a chromosome 20, although the latter was little represented in patients 2 and 3. A few cells with a single chromosome 20 were also found. The same cytogenetic findings were confirmed in the lymphoblastoid cell line of patient 1 and in the fibroblasts of patient 3. FISH with chromosome‐specific telomeric probes and TTAGGG sequences demonstrated the integrity of the ring chromosomes.

Conclusions: The clinical picture of these patients appears to be related to the instability of the r(20)‐generating cells monosomic for chromosome 20 and is thus haploinsufficient for a gene. In these patients, the electroclinical pattern of theta waves (probably unrelated to epilepsy) and the SW and SFT, even with mild mental retardation (MR) or no MR and without dysmorphic features, suggest that the r(20) syndrome may be present.
Acute Symptomatic Seizure Disorders in Young Children—A Population Study in Southern Taiwan
Authors: Huang Chao‐Ching , Chang Ying‐Chao , Wang Shan‐Tair
Published: August 2005

Summary: Purpose: To determine the incidence, etiology, and prognosis of acute symptomatic seizures in children by age 3 years.

Methods: In a population‐based birth cohort study of all 15,209 neonatal survivors born in Tainan City between October 1989 and December 1991, parents or caretakers of 13,493 children aged 3 years were surveyed by telephone regarding any provoked convulsive disorder, particularly acute symptomatic seizure, in the children; medical records were reviewed.

Results: Sixty‐three children (39 boys, 24 girls) had acute symptomatic seizures (incidence O.46 in 100). The leading causes of acute symptomatic seizures were acute gastroenteritis, encephalitis/encephalopathy, and bacterial meningitis. Age‐specific incidence was highest in the group aged 1–12 months. Intracranial hemorrhage, bacterial meningitis, and metabolic disturbance were the major causes of acute symptomatic seizures in children aged 1–12 months. Acute gastroenteritis, encephalitis/encephalopathy, and bacterial meningitis accounted for 85% of the causes in children aged 13–24 months, and gastroenteritis and encephalitislencephalopathy were the predominant causes in those aged 25–36 months. By age 5 years, subsequent unprovoked seizures developed in 14% of the survivors of acute symptomatic seizures.

Conclusions: Many acute symptomatic seizures are preventable. The risk of subsequent unprovoked seizures is determined by underlying precipitating factors. Public education regarding the danger of shaken‐baby syndrome and excessive water supplement, as well as and nationwide vaccination against bacterial meningitis in young children, is necessary.
Nonepileptic Seizures After Head Injury
Authors: Westbrook Lauren E. , Devinsky Orrin , Geocadin Romergryko
Published: August 2005

Summary: Purpose: To examine the role of head injury as a risk factor in the development of nonepileptic seizures (NES). Specifically, we will determine the relative frequency of head injury among NES patients referred to our center and will describe several pertinent clinical features and personal characteristics.

Methods: Retrospective record review of patients referred to our center for evaluation of seizures over a 4‐year period. All patients with NES were evaluated as in a previously described protocol, which included intensive video EEG monitoring, provocation by suggestion, and psychiatric interview. All NES patients with a history of head injury were extracted for this report.

Results: Of 102 patients with NES, nearly one‐third (32%) had an antecedent head injury; 52% were male, mean age was 34 years, and 12% had coexisting epilepsy. Multiple psychiatric disorders were not uncommon (79%), and a history of abuse was found in 35%. All but four patients had documented financial gain from their injury. Follow‐up at 1 year found poor long‐term outcome with lasting disability; despite that, the majority (91%) of head injuries were minor.

Conclusions: Our preliminary findings suggest that prior head injury is associated with the development of NES and may contribute to the pathogenesis of NES in vulnerable patients. Head injury and sexual or physical abuse appear to occur in comparable proportions in patients with NES. This suggests that head injury and abuse may be equally important risk factors in the development of NES.
Classifications of the International League Against Epilepsy: Time for Reappraisal
Authors: Engel Jerome
Published: August 2005

Summary: Recent advances in neurodiagnostic technology, new insights into fundamental neuronal mechanisms of epilepsy, and rapid developments in molecular genetics have greatly improved our understanding of epileptic seizures and epileptic disorders since 1981, when the current International Classification of Epileptic Seizures was adopted, and since 1989, when the International Classification of Epilepsies, Epileptic Syndromes, and Related Disorders was adopted. Although both have been universally accepted and have proven to be of considerable clinical value, it has become clear that more is needed for specific applications of growing importance, such as presurgical evaluation, clinical pharmacology trials, and epidemiological studies. Therefore, an International League Against Epilepsy (ILAE) task force has evaluated the need for revising our current classification and terminology and has begun developing four specific documents. The first is a descriptive terminology for ictal phenomena, incorporating some of the concepts included in the preceding paper by Dr. Hans Lüders; however, this will be a glossary, not a classification. The second is a classification of epileptic seizures based on known or presumed pathophysiological and anatomic substrates, to replace the current classification, which is based entirely on phenomenology. The third is a classification of epileptic syndromes and epileptic diseases, adapted from the current Classification of Epilepsies, Epileptic Syndromes, and Related Disorders, which will take into account the fact that disorders with unique etiologies, such as a single gene, should be considered diseases, and which will be reorganized for maximum practical application to differential diagnosis. The fourth is a new classification of functional disability due to seizures or epilepsy, based on a new impairment classification for neurological disorders being developed by the World Health Organization. Working groups are currently in the process of developing each of these documents, and their chairs would benefit from any comments or suggestions from those who ultimatelly will be using these systems of classification and terminology.
Lamotrighe‐Induced Severe Cutaneous Adverse Reactions
Authors: Schlienger Raymond G. , Shapiro Lori E. , Shear Neil H.
Published: August 2005

Summary: Purpose: We systematically reviewed and analyzed published and unpublished cases of Stevens‐Johnson syndrome (SJS), or toxic epidermal necrolysis (TEN) associated with lamotrigine (LTG) therapy to identify characteristics of these reactions.

Methods: We performed a MEDLINE search (January 1985 to April 1998) and citation tracking for published reports. In addition, reports were requested from the Uppsala Monitoring Centre of the World Health Organization (WHO). Published and WHO cases of LTG‐associated SJS or TEN were included if the causal relationship was assessed as either possible, probable, or definite.

Results: We identified a total of 57 cases (43 cases of SJS, 14 cases of TEN), of which 13 (23%) were published. Cases in the SJS group were significantly younger than in the TEN group (21 years vs. 31 years). The median time to onset (17 days for SJS and TEN) and the median dosage at onset (50 mg vs. 87.5 mg) for SJS and TEN did not differ significantly. Concomitant use of valproate (VPA) was reported in 74% of the SJS cases and 64% of the TEN cases. In three cases, TEN was the cutaneous manifestation of the antiepileptic drug hypersensitivity syndrome (AHS).

Conclusions: The main features of severe cutaneous drug reactions, such as dosage, onset, and concomitant VPA use, do not differ in patients with LTG‐induced SJS or TEN. SJS or TEN may also be the cutaneous manifestations of LTG‐induced AHS. Further epidemiologic studies are needed to identify the incidence of severe LTG‐induced cutaneous adverse reactions and the relative risk compared with other AEDs.
The Prognosis for Control of Seizures with Medications in Patients with MRI Evidence for Mesial Temporal Sclerosis
Authors: Kim Won‐Joo , Park Soo‐Chul , Lee Se‐Jin , Lee Joon‐Hong , Kim Jung‐Yeon , Lee Byung‐In , Kim Dong‐Ik
Published: August 2005

Summary: Purpose: Mesial temporal sclerosis (MTS) is the most common and important pathology in temporal lobe epilepsy (TLE), and its presence in magnetic resonance imaging (MRI) scans is strongly correlated with a successful surgical outcome. Despite the general assumption that patients with MTS respond poorly to medication, the long‐term prognosis for such patients has not yet been investigated. We studied the overall clinical prognosis of patients with MTS and analyzed the factors related to the degree of medical responsiveness.

Methods: Case patients were actively followed up at the Yonsei Epilepsy Clinic in Seoul, Korea, for >2 years. A structured interview and a thorough clinical evaluation were conducted. MRI scans, at the field strength of 1.0 or 1.5 Tesla, were performed with T1‐ and T2‐weighted coronal and axial spin‐echo images. All coronal slices were ≤5‐mm thick with no gap. Two neurologists and one radiologist determined the presence of MTS in MRIs by visual analysis.

Results: The MTS group consisted of 104 patients. Of these, 26 (25%) were completely controlled with adequate therapy, and 40 (38%) were intractable, despite aggressive anticonvulsant polytherapy. The remaining 37% had their seizure frequencies reduced by ≥50%, but were not seizure free. The age of seizure onset was significantly younger in the intractable group than in the well‐controlled group. Patients with a history of febrile convulsions or with epileptiform discharges in their electroencephalogram (EEG) had poorer seizure control (p < 0.05) than those who did not. Among the 16 patients who had no previous treatment, five (31%) became seizure free, and two were intractable.

Conclusions: Not all patients with MTS are medically intractable; 25% of the patients in our study achieved complete control while receiving medication. Poor seizure control was related to an early age of seizure onset, a history of febrile convulsions, and epileptiform discharges on the EEG.
Gelastic Epilepsy: Symptomatic and Cryptogenic Cases
Authors: Striano Salvatore , Meo Roberta , Bilo Leonilda , Cirillo Sossio , Nocerino Cristofaro , Ruosi Patrizia , Striano Pasquale , Estraneo Anna
Published: August 2005

Summary: Purpose: To describe the etiology, characteristics, and clinical evolution of epilepsy in patients with gelastic seizures (GSs).

Methods: Nine patients whose seizures were characterized by typical laughing attacks were observed between 1986 and 1997. Patients were selected based on electroencephalogram (EEG) or video‐EEG recordings of at least one GS and on magnetic resonance imaging (MRI) study.

Results: Five patients were affected by symptomatic localization‐related epilepsy (LRE), with four of the patients' disorders related to a hypothalamic hamartoma (HH) and one to tuberous sclerosis (TS) without evident hypothalamic lesions. In four patients (the cryptogenic cases) MRI was negative also in these cases, clinical and EEG data suggested a focal origin of the seizures. The epileptic syndrome in the HH cases was usually drug‐resistant, and was surgically treated in two of the patients. The patient with TS became seizure free with vigabatrin. In the cryptogenic cases, the ictal, clinical, and EEG semiology were similar to the symptomatic cases; the clinical evolution was variable, with patients having transient drug resistance or partial response to treatment. No cognitive defects were observed in the cryptogenic patients. None of the nine patients had precocious puberty.

Conclusions: We confirm the frequent finding of HHs in GSs and further underline how GSs may also be observed in patients without MRI lesions and with normal neurologic status. In these patients, clinical and EEG seizure semiology is similar to symptomatic cases, but the clinical evolution is usually more benign.
Allergic Skin Reactions to Anticonvulsant Medications in Patients Receiving Cranial Radiation Therapy
Authors: Mamon Harvey J. , Wen Patrick Y. , Burns Anne C. , Loeffler Jay S.
Published: August 2005

Summary: Purpose: Erythema multiforme and Stevens‐Johnson syndrome have been associated with anticonvulsant medications (AEDs) in patients with brain tumors receiving cranial irradiation. AEDs are also known to cause mild drug rashes. The incidence of these complications has not been well studied among patients with brain tumors. We reviewed the records of patients with brain tumors treated with cranial radiation and AEDs to assess the frequency of both severe and mild skin reactions.

Methods: Retrospective review of 289 radiotherapy records of consecutively treated patients from 1988 to 1993.

Results: Only one of 289 patients developed erythema multiforme. Milder rashes, however, occurred in 18% of exposures to AEDs including 22% of exposures to phenytoin, compared with the expected rate of 5‐10%. Most of the mild drug rashes occurred before the initiation of radiotherapy, suggesting that radiation was not the cause of these reactions.

Conclusions: Severe skin rashes are rare among patients with brain tumors receiving radiation therapy and AEDs. There is, however, an increased frequency of mild drug rashes among patients with brain tumors that does not appear related to radiation.
Elevated Plasma Concentrations of Homocysteine in Antiepileptic Drug Treatment
Authors: Schwaninger Markus , Ringleb Peter , Winter Ralph , Kohl Brigitte , Fiehn Walter , Rieser Peter A. , Walter‐Sack Ingeborg
Published: August 2005

Summary: Purpose: Homocysteine is an experimental convulsant and an established risk factor in atherosclerosis. A nuritional deficiency of vitamin B6, vitamin B12, or folate leads to increased homocysteine plasma concentrations. During treatment with carbamazepine (CBZ), phenytoin, or phenobarbital, a deficiency in these vitamins is common. The objective of the study was to test the hypothesis that antiepileptic drug (AED) treatment is associated with increased homocysteine plasma concentrations.

Methods: A total of 51 consecutive outpatients of our epilepsy clinic receiving stable, individually adjusted AED treatment and 51 sex‐ and age‐matched controls were enrolled in the study. Concentrations of total homocysteine and vitamin B6 were measured in plasma; vitamin B12 and folate were measured in the serum of fasted subjects.

Results: Patients and controls differed significantly in concentrations of folate (13.5 ± 1.0 vs. 17.4 ± 0.8 nM and vitamin B6 (39.7 ± 3.4 vs. 66.2 ± 7.5 nM), whereas serum concentrations of vitamin B12 were similar. The homocysteine plasma concentration was significantly increased to 14.7 ± 3.0 μM in patients compared with controls (9.5 ± 0.5 μM; p < 0.05, Wilcoxon rank‐sum test). The number of patients with concentrations of >15 μM was significantly higher in the patient group than among controls. The same result was obtained if only patients with CBZ monotherapy were included. Patients with increased homocysteine plasma concentrations had lower folate concentrations.

Conclusions: These data support the hypothesis that prolonged AED treatment may increase plasma concentrations of homocysteine, although the alternative explanation that increased homocysteine plasma concentrations are associated with the disease and not the treatment cannot be completely excluded at the moment.
Payer Costs of Patients Diagnosed with Epilepsy
Authors: Griffiths Robert I. , Schrammel Peggy N. , Morris George L. , Wills Stacey H. , Labiner David M. , Strauss Michael J.
Published: August 2005

Summary: Purpose: To identify the annual cost to a third‐party payer of inpatient and outpatient services and prescription drugs for patients diagnosed with epilepsy or convulsions.

Methods: Retrospective study using administrative and claims data from a private insurer in the Northeast United States with >1.8 million covered lives. Health plan members were included if they had a claim for epilepsy or convulsions and a claim for an antiepileptic drug (AED) between January 1992 and December 1996. Annual costs and frequencies of all medical services, and of services related to epilepsy, were compared among five groups of patients defined by the most intensive procedure they received: invasive therapeutic procedure (group I); invasive diagnostic procedure without an invasive therapeutic procedure (group 2); noninvasive diagnostic procedure without an invasive procedure (group 3); neurologist or neurosurgeon visit without an invasive procedure or noninvasive diagnostic procedure (group 4); or none of the preceding services (group 5).

Results: In the cohort of 9,090 patients meeting the inclusion criteria, mean age was 38 years, 53% were female, 30% had malignant disease, and 25% had cardiac disease. The mean annual cost of all medical services was $9,617. Mean annual costs of all services were $43,333, $29,847, $11,300, $4,362, and $5,855, and annual costs of inpatient and outpatient encounters coded as epilepsy plus AEDs were $24,369, $10,330, $3,127, $1,079, and $1,086, in groups 1‐5, respectively. Services used to stratify patients into the groups accounted for 37% of the total costs.

Conclusions: The annual costs of medical services for patients with epilepsy are high and vary considerably because of treatment of epilepsy and management of comorbidities.
Surgical Treatment of a Case of Early Infantile Epileptic Encephalopathy with Suppression‐Bursts Associated with Focal Cortical Dysplasia
Authors: Komaki Hirofumi , Sugai Kenji , Sasaki Masayuki , Hashimoto Toshiaki , Arai Nobutaka , Takada Eiko , Maehara Taketoshi , Shimizu Hiroyuki
Published: August 2005

Summary: We report a surgically treated case of early infantile epileptic encephalopathy (EIEE) with supression‐bursts associated with focal cortical dysplasia. Tonic‐clonic seizures followed by a series of spasms occurred about a hundred times a day at a few days of age. Interictal electroencephalogram (EEG) revealed a suppression‐burst pattern that was predominant in the left hemisphere. Magnetic resonance imaging (MRI) suggested focal cortical dysplasia in the left prefrontal area. Combination therapies with antiepileptic treatments showed only partial efficacy. The patient underwent lesionectomy at age 4 months, after which he gradually showed psychomotor development and a decrease of spasms to 0‐2 series daily. In cases of EIEE with focal cortical dysplasia, surgical treatment may have beneficial effects on both psychomotor development and seizure control.
Clinical Significance of EEG Abnormalities During Photic Stimulation in Patients with Photosensitive Epilepsy
Authors: Fylan F. , Edson A. S. , Harding G. F. A.
Published: August 2005

Summary: Purpose: The continued presence of EEG abnormalities in patients with a history of photosensitive seizures is used to signify the persistence of photosensitive epilepsy. The extent to which this approach places patients at risk of seizures is unclear, however. We describe those EEG abnormalities that may be tolerated with low risk of further seizures, and those that are indicative of poor seizure control.

Methods: Fifty patients with EEG evidence of persistent photosensitive epilepsy underwent photosensitivity testing with diffuse and patterned light; 58% of patients continued to experience seizures, and 42% were seizure free. The incidence of EEG abnormalities to diffuse and patterned intermittent photic stimulation (IPS) was analysed as a function of recent seizures.

Results: All patients showed EEG abnormalities to patterned IPS; there was no significant association between patterned IPS and poor seizure control. EEG abnormalities to diffuse IPS occurred in 58% of patients, and 76% of these patients had experienced a seizure within the previous year. These patients were more than twice as likely to be poorly controlled than those who showed abnormalities only to patterned IPS. These results were consistent for both medicated and unmedicated patients.

Conclusions: EEG abnormalities to patterned IPS can be used to signify the persistence of photosensitive epilepsy, but abnormalities to diffuse IPS are more likely to indicate the patient is poorly controlled and at risk of further seizures.
Morbus Sacer in Africa: Some Religious Aspects of Epilepsy in Traditional Cultures
Authors: Jilek‐Aall Louise
Published: August 2005

Summary: Epilepsy when manifested as grand mal seizure provokes strong and ambivalent feelings in those witnessing it. Terms such as morbus sacer, denoting both a sacred and demoniac condition, or folk names indicating divine punishment, have expressed these feelings in European societies from antiquity to the Middle Ages and beyond. An atmosphere of fear, shame and mysticism surrounds epilepsy even in our days in many non‐Western and also in Western cultures. In the course of work and studies in Tanzania, where I organized the Mahenge Clinic for Epilepsy in 1960, and in other parts of Africa. I found that epilepsy is conceived of as an “African” affliction, a manifestation of supernatural forces that makes it difficult to reach epilepsy sufferers with modern medical treatment. Epilepsy is traditionally looked on as caused by ancestral spirits or attributed to possession by evil spirits. It is also thought to be due to witchcraft, and “poisoning,” and often taken to be contagious. Epilepsy may, under Christian missionary teaching, have come to be considered as due to demoniac possession or divine punishment for sins, in accordance with biblical examples. In many parts of Africa, syncretic amalgamation of indigenous traditions with Judeo—Christian doctrines influenced popular attitudes toward epilepsy. We demonstrated that persistent efforts at health education in the context of organized treatment of epilepsy can result in a change of popular notions about epilepsy and consequently lead to significant improvement in the quality of life of epilepsy sufferers.
Normative Volumetric Data of the Developing Hippocampus in Children Based on Magnetic Resonance Imaging
Authors: Pfluger Thomas , Weil Sabine , Weis Serge , Vollmar Christian , Heiss Dirk , Egger Joseph , Scheck Roland , Hahn Klaus
Published: August 2005

Summary: Purpose: To acquire normative data of the hippocampus and its postnatal growth in 50 children (age, 1 month to 15 years) without epilepsy.

Methods: Morphometry of the hippocampus was carried out by using a spoiled FLASH 3D sequence (sagittal orientation), whereas the volume of the brain was assessed with a T2‐weighted spin‐echo sequence (transverse orientation). The volume of the hippocampus and the brain was determined by following Cavalieri's principle. Growth curves of the brain and hippocampus were fitted to a nonlinear Boltzmann sigmoidal equation.

Results: Intra‐/interobserver coefficient of variation was 2.0/4.9% for hippocampal volume measurements and 2.0/2.1% for brain volumetry. A significant difference in volume was noted between the right and left hippocampus (p < 0.001), with the right side being larger on average by 0.10 cc. Correlation coefficients of growth curves ranged between 0.71 and 0.94. Growth curves demonstrated a faster development of the hippocampus in girls. A steeper slope of hippocampal growth as compared with brain growth was found in girls, whereas in boys, the slope of brain growth was steeper.

Conclusions: Our findings will be of help in evaluating vulnerable phases of the hippocampal formation with accelerated growth, thereby leading to a better understanding of the development of hippocampal sclerosis in early childhood.
GABA Changes with Vigabatrin in the Developing Human Brain
Authors: Novotny Edward J. , Hyder Fahmeed , Shevell Michael , Rothman Douglas L.
Published: August 2005

Summary: Purpose: Changes in γ‐aminobutyric acid (GABA) physiology are important in determining seizure susceptibility in the developing nervous system. Noninvasive measurements of brain GABA in adults with epilepsy have demonstrated important relations among seizure control, brain GABA levels, and changes in brain GABA with drugs designed to alter GABA metabolism. The purpose of this study was to demonstrate the changes in GABA in the occipital lobes of children with epilepsy after treatment with vigabatrin (VGB).

Methods: Ten proton nuclear magnetic resonance spectroscopic (NMRS) studies were obtained on four subjects with epilepsy. The subjects were between ages 1 and 5 years. Occipital lobe GABA levels were measured before and after treatment with VGB.

Results: Brain GABA levels increased significantly in these subjects after VGB treatment (p < 0.05, paired Student's t test). In one subject, brain GABA was decreased in the region of the epileptic focus compared with the homologous region of the opposite hemisphere. A nearly fivefold increase in GABA occurred in the epileptic region after VGB treatment in this subject.

Conclusions: VGB increases brain GABA levels in children with epilepsy. NMRS can be used to monitor the response of brain GABA levels to drugs known to alter GABA physiology and serve as an important tool to understand the role of GABA‐mediated inhibition in pediatric epilepsies.
Epilepsy in Ehlers‐Danlos Syndrome
Authors: Jacome Daniel E.
Published: August 2005

Summary: Purpose: Ehlers‐Danlos syndrome (EDS) is a complex hereditary connective tissue disorder infrequently reported in association with epilepsy. Seven patients with ages ranging from 28 to 70 years with EDS and epilepsy are described.

Methods: Case review of clinical and diagnostic data.

Results: Two patients had occipital horn syndrome (EDS type IX) and partial seizures of probable supplementary motor area origin. Of these two, one had an area of frontal gliosis and was able to abate his seizures by hyperextending his neck; the other had a Dandy Walker malformation and also had pseudoseizures. The third patient of the series had complex partial seizures, pain asymbolia, and basilar artery hypoplasia. The fourth had ictal aphasia, left hemispheric hypotrophy, and distal right arm and left leg atrophy. The fifth patient had focal seizures, a venous parietal angioma, hyperekplexia, nocturnal head oscillations (jactatio capitis nocturna), monoclonal gammopathy‐associated neuropathy, and Tourette syndrome. The sixth had affective illness, chronic fatigue, and complex partial seizures with autoscopic phenomena after intracranial bleed. The seventh patient had a previous stroke, peripheral neuropathy, and grand mal seizures.

Conclusions: EDS may be accompanied by congenital or acquired central nervous system disorders and epilepsy. Additional neurologic conditions that are unrelated to EDS may be present.
Male Infertility: Possible Association with Valproate Exposure
Authors: Yerby Mark S. , McCoy Gregory B.
Published: August 2005

Summary: Purpose: To describe a potential association between male infertility and valproate (VPA) exposure. VPA has been implicated in the development of polycystic ovarian disease and subsequent menstrual and infertility problems in women with epilepsy. Infertility has been well described in population‐based studies of persons with epilepsy. The low marital rates for men with epilepsy have previously been thought to play a major contributing role.

Methods: We report a case of a 32‐year‐old man whose wife and he were able to bear a child before the development of his epilepsy. With VPA monotherapy, the family were unable to conceive despite 4 years of unprotected intercourse. An infertility evaluation of the man revealed a very low sperm count of <50,000/ml, no motile sperm, <10% viability, and 100% with abnormal structure. Follicle‐stimulating hormone, luteinizing hormone, and testosterone levels were normal.

Results: Felbamate (FBM) was initiated and VPA discontinued for improved seizure control. Within 4 months, the couple conceived their second child. A seminal analysis revealed a sperm count of >16 million, 50% motility, 78% viability, and 72% with abnormal structure.

Conclusions: One must be cautious in extrapolating from a case report, but these findings strongly suggest a direct effect of VPA on spermatic structure and function.