Seizure Syndrome Research Articles

Neurological complications of liver transplantation in pediatric patients: A single center experience

Neurologic complications (NCs) are a significant cause of morbidity and mortality in patients who undergo liver transplantation (LT). The aim of this study was to evaluate the incidence and type of NCs and associated factors in pediatric LT patients. We retrospectively reviewed NCs in the medical records of 40 consecutive infants, children, and adolescents who underwent LT at our institution. The subjects consisted of 23 boys and 17 girls (median age, 8.5 +/- 0.85 yr; range, 11 months to 17 yr). The indications for LT were Wilson's disease in 10 patients, fulminant hepatic failure (FHF) in nine, and other types of chronic liver disease in 21. NCs were found in 14 patients (35%). Those 14 individuals experienced a total of 16 episodes of NCs (two separate episodes in two of the patients). The most common NCs were seizure (seven episodes in six patients) and posterior leukoencephalopathy syndrome (PLES; five episodes in four patients). Seizure was the presenting symptom in three episodes of PLES. Two episodes of diffuse encephalopathy were observed in two patients, and two episodes of psychiatric symptoms occurred in two patients. We also noted one episode of tremor in one patient, one episode of acute dystonic reaction in one patient, and one episode of headache in one patient. Patients with Wilson's disease had a higher incidence of NCs (60%) than did patients without Wilson's disease (26.7%); however, this difference was not significant. The incidence of NCs was 44% in patients with FHF and 35% in those without FHF. That difference also was not significant. Immunosuppressive agents were the primary cause of 13 of the 16 episodes of NC. Uremia with hypertension, hypoxia, and hypomagnesemia caused one neurologic episode each. NCs, which are frequent in the first 30 days after pediatric LT, did not affect survival in this group. NCs were reversed by the discontinuation or reduction of immunosuppressive agents in 12 episodes, correction of hypomagnesemia and the reduction of immunosuppressive agents in one episode, and the correction of uremia and hypertension in one episode. Refractory epilepsy developed in one patient, and death unrelated to NCs occurred in one. The mortality rate was 7.1% (n = 1) in patients with NCs and 15.4% (n = 4) in those without NCs (p = 0.64). NCs are an important complication after LT. It is essential that each transplantation team collaborate with pediatric neurologists to ensure the rapid and accurate diagnosis of NCs in infants, children, and adolescents after LT and to prevent the delay of appropriate treatment.

Report of the ILAE Classification Core Group

A Core Group of the Task Force on Classification and Terminology has evaluated the lists of epileptic seizure types and epilepsy syndromes approved by the General Assembly in Buenos Aires in 2001, and considered possible alternative systems of classification. No new classification has as yet been proposed. Because the 1981 classification of epileptic seizure types, and the 1989 classification of epilepsy syndromes and epilepsies are generally accepted and workable, they will not be discarded unless, and until, clearly better classifications have been devised, although periodic modifications to the current classifications may be suggested. At this time, however, the Core Group has focused on establishing scientifically rigorous criteria for identification of specific epileptic seizure types and specific epilepsy syndromes as unique diagnostic entities, and is considering an evidence-based approach. The short-term goal is to present a list of seizure types and syndromes to the ILAE Executive Committee for approval as testable working hypotheses, subject to verification, falsification, and revision. This report represents completion of this work. If sufficient evidence subsequently becomes available to disprove any hypothesis, the seizure type or syndrome will be reevaluated and revised or discarded, with Executive Committee approval. The recognition of specific seizure types and syndromes, as well as any change in classification of seizure types and syndromes, therefore, will continue to be an ongoing dynamic process. A major purpose of this approach is to identify research necessary to clarify remaining issues of uncertainty, and to pave the way for new classifications.

Incidence of emergencies and equipment to treat emergencies in dental practices: A statewide survey

Purpose of the study: Dental practice teams have to manage medical emergencies adequately to provide optimal patient safety. The German Society of Dental, Oral and Craniomandibular Sciences recommends ECG monitoring and readiness for defibrillation during electrosurgery in patients with pacemakers. Guidelines published by the Association of the Scientific Medical Societies in Germany demand adequate equipment to treat medical emergencies in all patients treated under analgesia or sedation. Objective on the study was to evaluate the incidence of emergencies as well as available equipment in dental practices in the state Saxonia (Germany). Materials and methods: A survey was mailed to all 3000 dentists who were registered with the state dentist board of Saxonia in January 2005. It contained questions on medical emergencies during a 12 months period (01/2004–12/2004) and on emergency equipment which is provided actually in the respective practice. The survey was completely anonymous, all questionnaires which had been returned within 4 weeks were evaluated. Results: We received 573 questionnaires. 21% of the dentists provide treatment under sedation. During the evaluated 12 months 1435 emergencies occurred. The most frequent emergencies were vasovagal syncope (1208), hypertensive crisis (67), grand mal seizure (44), hypoglycaemia (30) and acute coronary syndrome (22). Two cardiac arrests were reported. 83% of the dentists who had responded have an emergency bag. Eighty eight percent have an airway device and 71% oxygen in their practice. Only 1.7% have an automated external defibrillator, 17% plan to buy an AED. Five percent of the dentists do not provide any equipment to treat emergencies. Thirty-five percent of the responding dentists had invested in emergency equipment in 2004 and 22% plan to buy new equipment in 2005. Conclusions: Emergencies occur frequently in dental practices. There are deficits as some dentists do not provide any emergency equipment and AED are not standard in dental practices.

Duloxetine-induced syndrome of inappropriate antidiuretic hormone secretion and seizures

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) and hyponatremia is a well known side effect of older selective serotonin reuptake inhibitors (SSRIs) such as paroxetine, sertraline, fluoxetine, citalopram, escitalopram, and fluvoxamine.1,2 The frequency of hyponatremia is around 8 per 1,000 among elderly women receiving fluoxetine.2 Although the second-generation dual blockers, selective serotonin–norepinephrine reuptake inhibitors (SNRIs) such as venlafaxine and duloxetine, are touted to have a wider therapeutic index, hyponatremia is encountered even with venlafaxine. To date, Medline searches do not reveal any reports of hyponatremia associated with duloxetine. We describe a woman who developed severe hyponatremia on exposure to duloxetine and recurrence on inadvertent rechallenge, suggesting the causative relationship of this drug to hyponatremia. A 48-year-old woman was hospitalized for acute severe headache and treated with IV fluids and analgesics. Over the next few days, she had multiple aches and pains and …

Management of Epilepsy in Women of Childbearing Age

Women with epilepsy should not be discouraged from becoming pregnant as the likelihood of having a healthy baby is very high. However, in such women, early and individualised counselling about pregnancy and contraception is essential. Ideally, pregnancies should be planned, folic acid (5 mg/day) given and antiepileptic drug (AED) treatment optimised well before conception to ensure that the lowest dosage that controls seizures is administered. When initiating AEDs in a woman of childbearing age, the most appropriate drug for the seizure type and syndrome should be chosen, although it is preferable to avoid valproate, because of a possible elevated risk of fetal malformations, when equi-effective agents are available for a given syndrome. In women who become pregnant while taking AEDs, fetal monitoring should include high-resolution ultrasonography before week 20 and measurement of serum alpha-fetoprotein levels. Amniocentesis is not routinely indicated. The measurement of blood concentrations of AEDs can be useful to ensure that the lowest possible maintenance dosage is being used, especially for those drugs whose pharmacokinetics are likely to change during pregnancy. Breastfeeding should be encouraged whatever the treatment administered.

Asthma Is Associated with Mortality in Children with Sickle Cell Anemia.

Background: Acute chest syndrome (ACS) is one of the leading causes of premature death among people with sickle cell anemia (SCA). Children with SCA and asthma have an increased risk of ACS (Boyd et al, Pediatric Pulmonology , 2004). The Cooperative Study of Sickle Cell Disease (CSSCD) has previously reported the median age at death to be approximately 45 years, 42 years for males and 48 years for females (Platt et al, New England Journal of Medicine, 1994). The objective of this study is to determine if asthma in patients with SCA is associated with mortality.Methods: The cohort was comprised of African American participants with SCA anemia enrolled in the CSSCD from birth to 66 years of age who were followed beyond five years and were evaluated for asthma. Patients who died before age 5 years were not included in the cohort. Asthma was classified by physician diagnosis, documentation of an acute asthma event, and/or record of use of prescribed asthma medication. Mortality was determined for patients with and without asthma. Cox regression analysis included the following established risk factors for death (Platt et al, 1994): covariates age, white blood cell count level, renal failure, hemoglobin F level, seizure, and acute chest syndrome.Results: A total of 1963 African American individuals with SCA were classifiable for asthma, enrolled in the study and followed for a total of 18,496 patient-years. The average age at study entry was 13.9 years, and the mean length of follow-up was 9.4 years. A total of 138 individuals (7.0%) had asthma; 70% were classified by a physician diagnosis, 10% by documentation of an acute asthma event, and 20% by record of using a prescribed asthma medication. Individuals with asthma had a median lifespan of 52.5 years in comparison to 64.3 years and over a two-fold higher risk of mortality after controlling for age, white blood cell count, renal failure, hemoglobin F level, seizure, and acute chest syndrome rate (hazard ratio 2.36 by Cox regression, 95% CI 1.21 to 4.62, p=0.01).Conclusion: Asthma was associated with a significant increase in the risk of all-cause mortality among patients with SCA who lived to 5 years of age. In addition to previously identified risk factors, asthma is an independent predictor of mortality.

Surgery: All Ages

Surgery: All Ages

Hypertensive Encephalopathy in Cats with Reduced Renal Function

The clinical, hemodynamic, and pathologic features of hypertensive encephalopathy in two cats with reduced renal mass are described. The cats developed a progressive syndrome of lethargy, ataxia, blindness, stupor, and seizures following an abrupt increase in blood pressure associated with a surgical reduction in renal mass. The cats had severe gross brain edema, evidenced by cerebellar changes of caudal coning and cranial displacement over the corpora quadrigemina and cerebral changes of widening and flattening of the gyri. Histologically, interstitial edema was most pronounced in the cerebral white matter. Hypertensive vascular lesions were present as hyaline arteriolosclerosis in one cat and hyperplastic arteriolosclerosis in the other. Rare foci of parenchymal microhemorrhages and necrosis were also observed. Systemic hypertension (especially severe or rapidly developing) accompanied by neurologic signs and the pathologic findings of diffuse brain edema with cerebral arteriolosclerosis are consistent with an etiologic diagnosis of hypertensive encephalopathy.

Familial clustering of seizure types within the idiopathic generalized epilepsies

To examine the genetic relationships among epilepsies with different seizure types--myoclonic, absence, and generalized tonic-clonic--within the idiopathic generalized epilepsies (IGEs). Careful phenotype definition in the epilepsies may allow division into groups that share susceptibility genes. Examination of seizure type, a phenotypic characteristic less complex than IGE syndrome, may help to define more homogeneous subgroups. Using the approach that found evidence of distinct genetic effects on myoclonic vs absence seizures in families from the Epilepsy Family Study of Columbia University, the authors examined an independent sample of families from Australia and Israel. They also examined the familial clustering of generalized tonic-clonic seizures (GTCs) within the IGEs in two combined data sets. Families were defined as concordant if all affected members had the same type of seizure or IGE syndrome, as appropriate for the analysis performed. The proportion of families concordant for myoclonic vs absence seizures was greater than expected by chance in the Australian families. In addition, GTCs clustered in families with IGEs to a degree greater than expected by chance. These results provide additional evidence for distinct genetic effects on myoclonic vs absence seizures in an independent set of families and suggest that there is a genetic influence on the occurrence of generalized tonic-clonic seizures within the idiopathic generalized epilepsies.

The Epileptic Encephalopathies of Infancy and Childhood

The epileptic encephalopathies comprise a group of devastating seizure syndromes which begin in infancy and early childhood and usually result in intractable epilepsy. While some syndromes are relatively easily diagnosed early in their course, others take time to evolve, hampering an early, confident diagnosis. Epileptic encephalopathies are associated with slowing of cognitive function and evolution of severe behavioral disorders, which are often more distressing to families than the epilepsy. While an underlying etiology may explain some of this co-morbidity, many children have no identifiable etiology found for their seizures. In these "idiopathic" cases, recurrent subtle seizures, frequent epileptiform discharge and non-convulsive status epilepticus probably all play a role in deterioration of cognitive function and evolution of behavior disorders. This paper will review the most common epileptic encephalopathy syndromes, discuss the cognitive and behavioral co-morbidities and review current therapeutic options.

Treatment of Alcohol Withdrawal Syndrome with Combined Carbamazepine and Tiapride in a Patient with Probable Sleep Apnoe Syndrome

The most frequently used agents for treatment of alcohol withdrawal syndrome are benzodiazepines and clomethiazole. Both have the main disadvantage of potential misuse and respiratory depression. Therefore their use in patients with respiratory diseases is limited. In recent years a treatment strategy with combined carbamazepine and tiapride was reported to be an effective alternative in alcohol withdrawal without the risk of respiratory depression. We report the successful treatment with carbamazepine and tiapride of a patient with probable sleep apnoe syndrome and history of withdrawal-related epileptic seizures.

Reversible posterior leukoencephalopathy syndrome: a misnomer reviewed

Reversible posterior leukoencephalopathy is a syndrome of headache, seizures and visual loss, often associated with an abrupt increase in blood pressure. Prompt diagnosis and therapy with antihypertensives, anticonvulsants, removal of any offending medication and treatment of associated disorders is essential since early treatment might prevent progression to irreversible brain damage. We present six illustrative cases presenting to Christchurch Hospital and review the condition. All were hypertensive, two were receiving immunosuppressant therapy after transplantation and one chemotherapy. Only three made a full recovery. The term reversible posterior leukoencephalopathy is a misnomer as the condition is not always reversible, is not necessarily confined to the posterior regions of the brain and can affect both white and grey matter. Magnetic resonance imaging findings of increased T2 and fluid attenuated inversion recovery signal predominantly involving the posterior regions of the cerebral hemispheres should alert the clinician to the possibility of this diagnosis.

Benign Focal Seizures of Adolescence: A Prospective Study

To characterize the clinical and EEG findings and evolution of the syndrome of benign focal seizures of adolescence (BFSA), as described by Loiseau et al. A prospective study was performed in adolescents with normal clinical and neurologic examinations and normal neuroradiologic studies who had focal seizures that occurred isolated or in a cluster, with or without secondary generalization in the first 24 to 48 h after onset. None of the patients was treated with antiepileptic drugs (AEDs). Between January 1996 and January 2002, 15 patients with BFSA were enrolled in the study. Median age at onset of BFSA was 14 years. Thirteen patients had focal sensory or motor seizures. In two patients, the ictal manifestation was motion arrest associated with oral automatisms. Eight of them evolved to generalized tonic-clonic seizures. Thirteen patients had seizures only when awake, and the other two, both when awake and during sleep. Repeated interictal EEGs were normal, but in four of the patients who had seizures in a cluster, we were able to record an EEG within 8 h after seizure onset. Two of these four patients had focal seizures, and their waking EEG showed focal centroparietal theta activities. The other two patients had secondarily generalized seizures, and their waking EEG showed bilateral theta activities instead. Prognosis was excellent. BFSA is a well-defined seizure syndrome, recognizable by clinical and EEG features, as described by Loiseau et al. In teenagers with these electroclinical features with a normal neurologic examination and normal neuroradiologic findings, AEDs should be avoided.

Memory in Patients with Drug‐responsive Mesial Temporal Lobe Epilepsy and Hippocampal Sclerosis

Mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE-HS) is the most common of the antiepileptic drug (AED)-resistant seizure syndromes that are remediable mostly with surgery, although a small group of patients have benign prognosis with fewer seizures. Material-specific memory impairment is an important feature in these patients and may be related to both the structural abnormality and the frequent seizures. In this study, we investigated the relation between memory deficit and HS by taking seizure frequency into account. The patients were evaluated according to a standard protocol and divided into two groups, considering their response to AEDs: the good-responder group (GRg, n = 18) and the pharmacoresistant group (PRg, n = 95). They were administered a neuropsychological test battery that included verbal and nonverbal memory tests, compared with each other and with a normal control group (n = 29). The responder group was evaluated by the same battery once again (mean, 23 months; SD, 8.25; range, 14-38 months). Both GR and PR patient groups had poorer memory than the normal controls in all memory tests (p < 0.05). However, the comparison of GRg with PRg revealed that only the digit-span test was significantly worse in PRg (p = 0.0061), and no difference was found in any other memory scores. The reevaluation of the GRg showed no significant difference between the first and second evaluation. We concluded that the memory impairment in patients with MTLE-HS was permanent and might be related to the direct effect of HS itself. Therefore patients with good response to AEDs can be used as a model for investigating the memory problems in patients with MTLE-HS.

SURGICAL TREATMENT OF THERAPY-RESISTANT EPILEPSY

ABSTRACT Epilepsy neurosurgery is a therapeutic option for patients with seizures resistant to medical therapy. Surgical therapy should be considered in therapy-resistant patients with epilepsy because of the increased mortality (conservatively 0.5% per year), and progressive cognitive and psychosocial morbidity associated with uncontrolled seizures over many years. Patients who continue to have disabling seizures after failure of two to three antiepileptic drugs should be evaluated at a specialty epilepsy center that offers surgery. In particular circumstances, such as catastrophic epilepsy in young children, patients ought to be urgently referred because of the risk of severe developmental disability. Epilepsy surgery encompasses a variety of resective and palliative operative procedures, and no age or gender restrictions exist. Depending on the seizure syndrome and operative procedure, 50% to 70% of patients can expect to become seizure free after resective surgery, which is superior to continued medical therapy. Surgical therapy has improved with time because of new technologies, such as neuroimaging and better operative techniques. Operative-related morbidity and mortality are generally under 5%, which is less than the risk of death from refractory epilepsy over the course of many years. Epilepsy neurosurgery, therefore, is not the treatment of last resort. Instead, surgery is a tool in the treatment of patients who are medically intractable and should be considered early in those patients with a high risk of therapy resistance to reduce seizure-related morbidity and mortality.

Intractable reflex audiogenic epilepsy successfully treated by peri-insular hemispherotomy

We report a case of an infantile hemiplegia seizure syndrome (IHSS) that presented with intractable reflex audiogenic startle epilepsy which in itself is an uncommon form of seizure disorder. Peri-insular hemispherotomy provided complete seizure control. Also of particular interest was that this syndrome resulted from an iatrogenic brain injury sustained during the course of a caesarian section. We review the different mechanisms of birth injury reported in the literature and, discuss the physiopathogenesis of the hemispheric damage in this patient. We also review the literature on “reflex epilepsy” as it applies to this case. Intractable reflex audiogenic (startle) epilepsy in IHSS submitted to hemispherotomy has not previously been reported.

Behavioral aspects of pediatric epilepsy syndromes

Apart from control of the seizures, two of the most important factors in determining how well a child with epilepsy progresses toward independence are cognition and behavior. The diagnosis of the correct epilepsy syndrome often provides information with regard to probability of good seizure control and intellectual outcome. However, relatively little has been published on the behavioral aspects of the various epilepsy syndromes. In West syndrome there is emerging evidence that early effective treatment might improve outcome in terms of both cognition and behavior. The work on this syndrome in children with tuberous sclerosis has demonstrated an association between temporal lobe tubers and autism. In Dravet syndrome, a variety of psychiatric disorders have been reported, including hyperactivity and autistic features. This is another epilepsy syndrome that tends to be resistant to treatment, implying that the prognosis has to be guarded. The behavioral problems reported with Lennox–Gastaut syndrome also include autistic features, as well as generally sluggish behavior. It is very likely that these characteristics largely reflect the effect of ongoing seizure activity. Autistic features, aggression, and hyperkinesis have been described with Landau–Kleffner syndrome. The behavior may improve dramatically with appropriate medical treatment or after multiple subpial transection. Although the syndrome of benign partial seizures with centrotemporal or rolandic spikes is said to have a very good prognosis, it is becoming increasingly evident that behavioral problems such as concentration difficulties, tempers, hyperactivity, and impulsivity might occur. Juvenile myoclonic epilepsy has been associated with very variable behavioral traits, sometimes with immature personality features and poor social adjustment suggesting frontal lobe dysfunction. Because many of the reports of behavioral disturbance associated with epilepsy syndromes are anecdotal and do not include validated measures of behavior it would be unwise to draw firm conclusions from them at this stage. Carefully conducted prospective studies, paying particular attention to any behavioral improvements that occur with successful treatment of the epilepsy, are required.

Effect of vagus nerve stimulation on adults with pharmacoresistant generalized epilepsy syndromes

Introduction: Although vagus nerve stimulation (VNS) therapy is approved for the treatment of partial onset seizures, its efficacy for generalized seizures has not been fully evaluated. This Investigational Device Exemption assessed the outcome of VNS therapy among patients with generalized epilepsy syndromes. Methods: Sixteen patients with pharmacoresistant generalized epilepsy syndromes and stable antiepileptic drug (AED) regimens were implanted with the VNS therapy device and were evaluated for changes in seizure frequency and type between baseline and follow-up of 12–21 months. Results: The patients experienced a statistically significant overall median seizure frequency reduction of 43.3% ( P=0.002, Wilcoxon signed rank test) after 12–21 months of VNS therapy. Types of seizures that may involve a fall or collapse decreased with reductions in the frequency of myoclonic (60% reduction, n=9; P=0.016, Wilcoxon signed rank test), tonic (75% reduction, n=8, NS), atonic (98.6%, n=3, NS), and clonic seizures (86.7%, n=1, NS). Conclusion: The benefits of reduced seizure frequency and reduced risk of injury merit consideration of VNS therapy for patients with pharmacoresistant generalized seizure syndromes.

The complex epilepsy patient: intricacies of assessment and treatment.

The aim of treatment of all patients with epilepsy is the same: a complete remission of seizures with minimal or no adverse events. However, the means to reach that common goal may differ among patients, not only because of differences in their epileptic syndrome or type of seizures within the same syndrome, but also because of a variety of comorbid pathologic entities that are commonly identified in patients with epilepsy. In this article, we discuss some of the complexities inherent in the planning of therapeutic regimens for epilepsy patients. Comorbid conditions, whether intrinsic or extrinsic to the epileptic disorder, must play a paramount role in the choice of the therapeutic regimen for seizure control. We review the impact of psychiatric comorbidities on the quality of life of patients with epilepsy, with special attention to the tight relationship between depression and epilepsy. Yet, despite this close relationship, comorbid depression is more often than not underrecognized and undertreated. Next, we review the impact of antiepileptic drugs (AEDs) on comorbid medical disorders and give clear examples of the potentially deleterious impact of several AEDs on the therapeutic effects of concomitant medications used for their treatment. Clearly, treating epilepsy is not only about controlling seizures.

Role of levetiracetam in the treatment of epilepsy

ABSTRACT Physicians treating patients with epilepsy have a host of therapeutic options. Drug choice is dictated first by the seizure(s) and/or epilepsy syndrome. Age is also a factor. Special considerations apply to women, particularly during their childbearing years, and to patients who are learning‐disabled. Drug selection is further influenced by such characteristics as spectrum of activity, rapid response, low potential for drug‐drug interactions, and ease of use. In addition to clinical trial data, postmarketing assessments of the new antiepileptic drugs provide useful clinical information on efficacy and safety. Levetiracetam has specific characteristics that make it an optimal choice for many patient populations.