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PubMed2004

Efficacy and tolerability of the new antiepileptic drugs I: treatment of new onset epilepsy [RETIRED]: report of the Therapeutics and Technology Assessment Subcommittee and Quality Standards Subcommittee of the American Academy of Neurology and the American Epilepsy Society.

OBJECTIVE: To assess the evidence demonstrating efficacy, tolerability, and safety of seven new antiepileptic drugs (AEDs) (gabapentin, lamotrigine, topiramate, tiagabine, oxcarbazepine, levetiracetam, and zonisamide-reviewed in the order in which these agents received approval by the US Food and Drug Administration) in the treatment of children and adults with newly diagnosed partial and generalized epilepsies. METHODS: A 23-member committee, including general neurologists, pediatric neurologists, epileptologists, and doctors in pharmacy, evaluated the available evidence based on a structured literature review including MEDLINE, Current Contents, and Cochrane library for relevant articles from 1987 until September 2002, with selected manual searches up until 2003. RESULTS: There is evidence either from comparative or dose-controlled trials that gabapentin, lamotrigine, topiramate, and oxcarbazepine have efficacy as monotherapy in newly diagnosed adolescents and adults with either partial or mixed seizure disorders. There is also evidence that lamotrigine is effective for newly diagnosed absence seizures in children. Evidence for effectiveness of the new AEDs in newly diagnosed patients with other generalized epilepsy syndromes is lacking. CONCLUSIONS: The results of this evidence-based assessment provide guidelines for the prescription of AEDs for patients with newly diagnosed epilepsy and identify those seizure types and syndromes where more evidence is necessary.

Acetates↗
PubMed1996

Long-term prognosis of typical childhood absence epilepsy: remission or progression to juvenile myoclonic epilepsy.

OBJECTIVE: To determine the proportion and characteristics of children presenting with childhood absence epilepsy (CAE) who were not taking anti-epileptic drugs (AEDs) and were seizure-free over the last year of long-term follow-up. METHODS: For case finding, centralized EEG records for the province of Nova Scotia allowed identification of all children with typical CAE diagnosed between 1977 and 1985. Follow-up was done in 1994 to 1995. RESULTS: Of 81 children with CAE, 72 (89%) were contacted for follow-up. Mean age at seizure onset was 5.7 years (range, 1 to 14 years) and at follow-up was 20.4 years (range, 12 to 31 years). Forty-seven (65%) were in remission. Twelve others (17%) were not taking AEDs but continued to have seizures. Thirteen (18%) were taking AEDs; five were seizure-free over the last year (in four of these a trial without AEDs had previously failed). Fifteen percent of the total cohort had progressed to juvenile myoclonic epilepsy (JME). Multiple clinical and EEG factors were examined as predictors of outcome. Factors predicting no remission (p < 0.05) included cognitive difficulties at diagnosis, absence status prior to or during AED treatment, development of generalized tonic clonic or myoclonic seizures after onset of AEDs, abnormal background on initial EEG, and family history of generalized seizures in first-degree relatives. CONCLUSIONS: Only 65% of children presenting with CAE had remission of their epilepsy. Forty-four percent of those without remission had developed JME. At the time of diagnosis, remission is difficult to predict accurately in most patients. However, development of generalized tonic-clonic seizures or myoclonic seizures during AED treatment is ominous, predicting both lack of remission of CAE and progression to JME.

Child, Preschool↗
PubMed1998

Worsening seizures after surgery for focal epilepsy due to emergence of primary generalized epilepsy.

Our patient underwent right anteromesial temporal resection at 17 years of age for intractable complex partial seizures due to hippocampal sclerosis, and then developed juvenile myoclonic epilepsy after a change in medication. Postoperative seizures ceased after a change to valproate monotherapy. Our patient reminds us to remain aware that generalized and focal epilepsy may coexist as an unusual cause for surgical failure. We feel that these patients may still be favorable candidates for epileptic surgery, as long as the focal epileptogenic zone is amenable to resection and the generalized epilepsy appears to be readily controllable.

Adolescent↗
PubMed2000

Adult myoclonic epilepsy: a distinct syndrome of idiopathic generalized epilepsy.

The authors present 11 cases of idiopathic generalized epilepsy that began in adulthood at a mean age of 39 years. All patients had myoclonic jerks, five had absence seizures, and nine had infrequent generalized tonic-clonic seizures. A majority had a family history of seizures. EEG in all patients showed generalized epileptiform abnormalities, whereas neuroimaging and neurologic examination results were normal. This series appears to represent a previously undescribed idiopathic generalized epilepsy syndrome of adult myoclonic epilepsy.

Adult↗
PubMed2000

Rolandic discharges in benign childhood epilepsy with centrotemporal spikes, and in other forms of partial epilepsies.

Previous reports concerning benign childhood epilepsy with centrotemporal spikes (BECT) were re-investigated, including new data concerning both the presentation of the long-term clinical courses of three cases and the results of magnetoencephalography (MEG) in one patient, with respect to the following points; (1) the electro-clinical characteristics of sylvian seizures and rolandic discharges (RD), and (2) long-term outcome of idiopathic and symptomatic partial epilepsies with RD other than BECT. The epileptic focus of sylvian seizures is located either in the inferior rolandic cortex or underneath the sylvian fissure, which is strongly supported by the results of clinical seizure manifestations, ictal and interictal EEG findings, and interictal MEG findings. The presence of rolandic discharges is not a hallmark of benign outcome. Instead, the presence of sylvian seizures heralds a benign outcome of partial epilepsy regardless of the presence or absence of an organic lesion. The pathophysiological mechanism of BECT remains unknown. Further neurophysiological as well as genetic investigations are needed.

Adolescent↗
PubMed2002

[Idiopathic generalized epilepsies misdiagnosed as partial epilepsies].

Idiopathic generalized epilepsy (IGE) is often not recognized with serious consequences on the sufferers. We examined factors contributing to the missed diagnosis of IGE in 41 adults attending our epilepsy clinic with diagnosis of partial epilepsy who had semiology or EEG findings suggesting a possible differential diagnosis. After careful re-evaluation, the diagnosis of IGE was established in 25 patients: 22 (88%) with JME, one with juvenile absence, one with perioral myoclonia with absences, one with eyelid myoclonia with typical absences. Myoclonic jerks, the hallmark of the JME and other IGE, were not usually reported by patients or misdiagnosed as focal motor seizures. Brief and infrequent absence seizures and focal EEG abnormalities were other factors contributing to not recognizing JME. All 25 patients did not achieve seizure control before re-evaluation of diagnosis. After appropriate diagnosis of IGE and change of AED to valproate or valproic acid, 19 (76%) became seizure free and six (24%) had a significant improvement on seizure control. Association with lamotrigine provided further improvement in three of these patients. An appropriate questioning to identify myoclonic and absence seizures and a proper interpretation in the context of whole clinical constellation are essential for a correct seizure classification and diagnosis of IGE in adults.

Adolescent↗
PubMed2003

[Juvenile myoclonic epilepsy misdiagnosed as focal epilepsy: variability of myoclonic seizures].

We report two male patients with juvenile myoclonic epilepsy. They had been diagnosed as having partial epilepsy for three years. They had various myoclonic seizures characterized by truncal and head torsion, stepping backward, and inability to reach objects, as well as asymmetric myoclonic jerks of the upper extremities. For early diagnosis of juvenile myoclonic epilepsy, it is important to take account of the variability of myoclonic seizures.

Child↗
PubMedDate not supplied

[The Andalusia Epilepsy Society's Guide to Epilepsy Therapy 2005: II. Chronic therapy with antiepileptic drugs in adults and in children].

AIMS: The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy. DEVELOPMENT: A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology's Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies' criteria for producing Clinical Practice Guidelines. CONCLUSIONS: The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care.

Adult↗
PubMed1993

[MRI hippocampal volumetric measurements in benign childhood epilepsy with centrotemporal spike(s) and temporal lobe epilepsy of childhood onset].

Benign childhood epilepsy with centrotemporal spike(s) (BCECT) is characterized by rolandic discharges (RD) on EEG. As the origin of RD, the lower rolandic cortex was proposed by Lombroso, but recently the hippocampus was proposed by some reports, based on the character of RD or the pharmacological effects of anticonvulsants. On the other hand, MRI hippocampal volumetric measurements revealed the relationship between the hippocampal atrophy and EEG foci in intractable temporal lobe epilepsy (TLE) of adults, but there are a few reports of the hippocampal volumetric study in children. Therefore, in this paper, we reported about MRI hippocampal volumetric measurements in BCECT and TLE of childhood onset. The statistical examination was performed by t-test. MRI hippocampal volumetric measurements were performed in 27 cases with BCECT (6-19 years, 9.9 +/- 2.7 years, 11 males, 16 females), 22 cases with TLE (5-22 years, 12.9 +/- 5.5 years, 15 males, 7 females) and 17 normal control subjects (6-15 years, 10.4 +/- 2.7 years, 10 males, 7 females). The age was not significantly different among the 3 groups. The clinical course of TLE group was significantly longer than BCECT group, but the onset age of epilepsy was not significantly different between the 2 groups. The hippocampal volume and the right-side minus left-side volume were not significantly different in 3 groups, but these values of TLE were more scattered than the others. There was no abnormal signal intensity in hippocampus on MRI, or no specific abnormality in the surrounding structures in 3 groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2003

Chronic epilepsy and cognition: a longitudinal study in temporal lobe epilepsy.

It remains unclear whether uncontrolled epilepsy causes mental decline. This longitudinal study contrasts change of memory and nonmemory functions in 147 surgically and 102 medically treated patients with temporal lobe epilepsy. All participants were evaluated at baseline (T1) and after 2 to 10 years (T3). Surgical patients underwent additional testing 1 year postoperatively (T2). Data were analyzed on an individual and group level. Sixty-three percent of the surgical and 12% of the medically treated patients were seizure-free at T3. Fifty percent of the medically treated and 60% of the surgical patients showed significant memory decline at T3 with little change in nonmemory functions (difference not significant). Surgery anticipated the decline seen in the medically treated group and exceeded it when surgery was performed on the left, or if seizures continued postoperatively. Seizure-free surgical patients showed recovery of nonmemory functions at T2 (p < 0.001) and of memory functions at T3 (T3, p = 0.03). Multiple regression indicated retest interval, seizure control, and mental reserve capacity as predictors of performance changes. In addition, psychosocial outcome was better when seizures were controlled. In conclusion, chronic temporal lobe epilepsy is associated with progressive memory impairment. Surgery, particularly if unsuccessful, accelerates this decline. However, memory decline may be stopped and even reversed if seizures are fully controlled.

Adult↗
PubMed1998

Familial partial epilepsy with variable foci: a new partial epilepsy syndrome with suggestion of linkage to chromosome 2.

Familial partial epilepsy with variable foci (FPEVF) joins the recently recognized group of inherited partial epilepsies. We describe an Australian family with 10 individuals with partial seizures over four generations. Detailed electroclinical studies were performed on all affected and 17 clinically unaffected family members. The striking finding was that the clinical features of the seizures and interictal electroencephalographic foci differed among family members and included frontal, temporal, occipital, and centroparietal seizures. Mean age of seizure onset was 13 years (range, 0.75-43 years). Two individuals without seizures had epileptiform abnormalities on electroencephalographic studies. Penetrance of seizures was 62%. A genome-wide search failed to demonstrate definitive linkage, but a suggestion of linkage was found on chromosome 2q with a LOD score of 2.74 at recombination fraction of zero with the marker D2S133. FPEVF differs from the other inherited partial epilepsies where partial seizures in different family members are clinically similar. The inherited nature of this new syndrome may be overlooked because of relatively low penetrance and because of the variability in age at onset and electroclinical features between affected family members.

Adult↗
PubMed1997

Patterns of language dominance in focal left and right hemisphere epilepsies: relation to MRI findings, EEG, sex, and age at onset of epilepsy.

The present study evaluates results of language testing during intracarotid amobarbital procedures in 167 patients with either left (LHE, n = 81) or right hemisphere epilepsies (RHE, n = 86). In both groups there was a high rate of deviance from complete left hemisphere dominance of 24 and 31%, respectively. Whilst complete right hemisphere or incomplete left hemisphere language dominance were the prominent atypical patterns in LHE, RHE was associated with either bilateral dominance or incomplete left dominance. In LHE, atypical language dominance was frequently associated with an extratemporal localization of lesions or epileptic foci. The age at onset of epilepsy and the degree of right hemisphere language dominance correlated significantly in LHE but not in RHE. Finally, atypical dominance in LHE but not in RHE was associated with poorer language and nonlanguage functions, the latter being negatively correlated with the degree of right hemisphere language dominance. Conclusions are: (1) The data contradict the assumption of equipotentiality and favor the supposition of a predetermined left hemisphere superiority in language processing. (2) Atypical language dominance in LHE can largely be explained in terms of a plasticity dependent language shift as a consequence of early left hemisphere epilepsies and lesions. (3) Atypical dominance patterns in RHE appear to reflect the prevalence of genetically determined variants and the possibility of a language transfer from the right to the left hemisphere.

Age of Onset↗
PubMed1997

Low grade glioma in intractable epilepsy: lesionectomy versus epilepsy surgery.

In approximately 30% of patients with intractable partial epilepsy, an intra-axial cerebral lesion is the aetiology of the seizure disorder. Lesions adjacent to mesiotemporal structures often result in secondary epileptogenicity in the same region. The authors present 22 cases of low-grade gliomas associated with intractable epilepsy. In 15 cases the location was temporal (8 extra-hippocampal and 7 with invasion of the amygdalo-hippocampus), 7 cases were extratemporal in eloquent areas. The eight extra-hippocampal tumours were originally treated with lesionectomy. The seizure outcome was class 1 in only 4 cases, the remaining 4 were class 4 according to Engel's classification. The 4 cases with class 4 outcome required additional temporal lobectomy associated with amygdalo-hippocampectomy for seizure control. The 2 cases with associated hippocampal atrophy at MRI after lobectomy had outcome class 1. The 2 cases without hippocampal atrophy at MRI presented outcome class 2. The 7 cases with invasion of amygdala and hippocampus were treated with selective lesionectomy+amygdalo-hippocampectomy. In all these cases convergence of focal structural abnormality, ictal onset of epileptiform EEG abnormality and interictal epileptiform EEG abnormality provided powerful evidence of focal epileptogenicity. All these patients had a favourable epilepsy outcome (class 1-2). In the seven extratemporal cases the first step was lesionectomy. In 1 case located in the parietal region intraoperative mapping was required. 5 had class 1 outcome, one case had outcome class 2 and one case had an outcome class 4. The last patient required a second step operation with intraoperative strip and deep electrode monitoring that led subsequently to a frontal lobectomy. This patient is seizure free 2 years after surgery. There was no perioperative mortality and post-operative morbidity was 3/22. This study indicates that lesionectomy may be the first step procedure if the structural abnormality is localized to extra-temporal eloquent cortex and concordance is documented. Patients may subsequently be candidates for a cortical resection as a second step procedure if the lesionectomy does not provide an adequate reduction in seizure tendency. Since MRI identified hippocampal atrophy was predictive in this study of an unsatisfactory seizure outcome after lesionectomy, MRI defined dual pathologies consisting of a temporal lesion plus hippocampal atrophy necessitate temporal lobectomy+amygdalo-hippocampectomy. In patients with negative MRI findings of hippocampal atrophy and temporal lobe lesions, intraoperative electrocorticography and deep electrode monitoring are indicated for planning the surgical strategy.

Adolescent↗
PubMed2000

Pure lesionectomy versus tailored epilepsy surgery in treatment of cavernous malformations presenting with epilepsy.

Cerebral cavernous malformations (CM) are well-circumscribed vascular malformations that often present with epileptic seizures. Although patients may initially benefit from antiepileptic drugs, surgical treatment may become necessary due to medically intractable seizures. However, it is unclear whether lesionectomy alone or tailored epilepsy surgery with previous invasive monitoring is the optimal strategy in such cases. We report two patients with epileptic seizures due to CM. One patient with few seizures prior to surgery became seizure-free following resection of the CM and the surrounding tissue. In the second patient with long-lasting epilepsy, lesionectomy was performed because of the proximity to a functioning left hippocampus. This limited resection failed and the patient still had seizures. Subsequently, invasive monitoring with intracranial depth and strip electrodes was performed in order to localize the epileptogenic area and determine whether the left hippocampus could be spared. The invasive study showed the seizure origin in the tissue around the former CM but no epileptic discharges in the hippocampus. In a second operation, an anterior temporal resection was performed with removal of the epileptogenic surrounding tissue and the patient became seizure-free without cognitive deficits. The optimal surgical strategy for CM presenting with epileptic seizures must take into account various factors such as underlying mechanisms and duration of epilepsy, and location of the lesion.

Adult↗
PubMed2005

Epilepsy surgery: perioperative investigations of intractable epilepsy.

Recent advances in our understanding of the basic mechanisms of epilepsy have derived, to a large extent, from increasing ability to carry out detailed studies on patients surgically treated for intractable epilepsy. Clinical and experimental perioperative studies divide into three different phases: before the surgical intervention (preoperative studies), on the intervention itself (intraoperative studies), and on the period when the part of the brain that has to be removed is available for further investigations (postoperative studies). Before surgery, both structural and functional neuroimaging techniques, in addition to their diagnostic roles, could be used to investigate the pathophysiological mechanisms of seizure attacks in epileptic patients. During epilepsy surgery, it is possible to insert microdialysis catheters and electroencephalogram electrodes into the brain tissues in order to measure constituents of extracellular fluid and record the bioelectrical activity. Subsequent surgical resection provides tissue that can be used for electrophysiological, morphological, and molecular biological investigations. To take full advantage of these opportunities, carefully designed experimental protocols are necessary to compare the data from different phases and characterize abnormalities in the human epileptic brain.

Brain↗
PubMed2006

Therapeutic effects of vagus nerve stimulation in epilepsy and implications for sudden unexpected death in epilepsy.

Vagus nerve stimulation (VNS) is a non-pharmacological therapy approved by the FDA for treatment of patients with partial-onset epilepsy. The most frequently encountered adverse effects typically occur during stimulation, are usually mild to moderate in severity, and resolve with reduction in current intensity or spontaneously over time. There are no apparent effects of VNS on vagally mediated visceral function. Though the precise mechanism of action of VNS remains unknown, available evidence suggests that central autonomic nervous system pathways are involved, which have also been implicated in sudden unexpected death in epilepsy (SUDEP). Studies to date of VNS and SUDEP are limited and do not conclusively show an association between VNS and SUDEP rates independent of other epilepsy-specific variables.

Anticonvulsants↗
PubMed2006

Health-related quality of life in youth with epilepsy: theoretical model for clinicians and researchers. Part I: the role of epilepsy and co-morbidity.

Children and adolescents with epilepsy are at increased risk for poor health-related quality of life (HRQL) even in the absence of active seizures. Clinicians who aim to achieve optimal seizure control also need to focus on improving HRQL. This can only be accomplished by recognizing how different features of the epilepsy itself, co-morbid conditions, as well as psychosocial factors can all make a difference to HRQL. These psychosocial factors include child, family and community/society variables that play a role in the well-being of these children. Conceptual models are relevant insofar as they provide us with a basis for hypothesizing the causal processes that lead to improved HRQL. They delineate what is meant by HRQL and highlight factors relevant to this important outcome. Researchers can use such models to test statistically and clinically relevant relationships. Clinicians can use this knowledge to direct the planning and implementation of services that are informed by these relationships, critically evaluate and then choose HRQL measures for use in clinical practice that most closely approximate what they understand HRQL is. The purpose of this review is to identify what is meant by HRQL as well as outline the theoretical and empirical basis for what it is about epilepsy, co-morbidity, and its treatment that we believe make a difference to HRQL.

Adolescent↗
PubMed1994

Cardiovascular causes of loss of consciousness in patients with presumed epilepsy: a cause of the increased sudden death rate in people with epilepsy?

UNLABELLED: BACKGROUND, METHODS, AND RESULTS: Syncope and seizures are often indistinguishable clinically. We present a series of 12 patients diagnosed as having epilepsy. Despite normal or nonspecific electroencephalographic findings, 11 of 12 patients were treated or offered treatment with long-term anticonvulsant agents. Subsequently, diagnoses of arrhythmic or neurally mediated syncope were made in all patients using Holter monitoring, long-term ambulatory loop electrocardiographic recording, or tilt-table studies. Arrhythmias included torsades de pointes (four patients), atrioventricular nodal reentrant supraventricular tachycardia (one patient), and sinus arrest (two patients). The remaining five patients had neurally mediated syncope with hypotension and bradycardia, including asystole in two patients. Treatment for the documented cardiovascular abnormalities resulted in the alleviation of syncopal symptoms. CONCLUSIONS: Because the observed cardiovascular abnormalities are potentially fatal, this series suggests that undiagnosed cardiac syncope may contribute to the documented increased sudden death rate in patients with presumed epilepsy. Cardiac causes of loss of consciousness should be considered in patients with presumed epilepsy, atypical premonitory symptoms (such as nausea, lightheadedness, or palpitations), nondiagnostic electroencephalograms, and failure to respond to anticonvulsant therapy.

Adolescent↗