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Biomedical subjects

Mary L Zupanc

Publications and source records attributed to Mary L Zupanc.

8 recordsLinked to original sources
PubMed2006

Felbamate: consensus of current clinical experience.

An expert panel convened to evaluate data and review current clinical practices regarding the novel antiepileptic drug (AED) felbamate. Felbamate has demonstrated efficacy against a variety of refractory seizures types, including seizures associated with Lennox-Gastaut syndrome, but postmarketing experience revealed serious idiosyncratic adverse effects that were not observed during clinical trials. Although felbamate is not indicated as first-line antiepileptic therapy, its utility in treating seizures that are refractory to other AEDs is undisputed, as shown by the number of patients who continue to use it. New exposures to felbamate number approximately 3200-4200 patients annually, and it is estimated that over the past 10 years, approximately 35,000 new starts have occurred. Recommendations by the American Academy of Neurology and a review of felbamate literature were evaluated in conjunction with the clinical experience of the expert panel to determine current medical opinion and practice regarding felbamate. The past 10 years of clinical experience have demonstrated that when used in accordance with existing recommendations and close clinical monitoring, felbamate is an effective treatment for some patients with seizures refractory to other AEDs. This review of clinical data and discussion of the current understanding of the risk:benefit of felbamate therapy supports its use as an important therapeutic option for some patients with refractory epilepsy.

Anticonvulsants↗
PubMed2006

Antiepileptic drugs and hormonal contraceptives in adolescent women with epilepsy.

Both estrogen and progesterone influence seizure activity in women with epilepsy, with estrogen generally demonstrating proconvulsant and progesterone anticonvulsant effects. Women with epilepsy exhibit a variety of endocrine disturbances, probably due to a combination of factors, including the epilepsy syndrome and the effect of interictal and ictal epileptic discharges in the brain. The direct effects of some antiepileptic drugs (AEDs) further increase this risk, apparently related to a specific drug's effect on hepatic microsomal enzymes of the cytochrome P-450 system. AEDs that induce hepatic microsomal enzymes also interact with hormonal contraception to increase estrogen's metabolism and progesterone's protein binding, decreasing concentrations of both hormones and thus reducing contraceptive efficacy. Some evidence indicates that concurrent use of hormonal contraceptives and lamotrigine significantly decreases the plasma concentration of lamotrigine, suggesting that close monitoring is warranted. Nevertheless, hormonal contraception confers comparable or superior efficacy compared with such other contraceptives as the intrauterine device and barrier methods and remains an appropriate option in women with epilepsy. Importantly, concurrent use of hormonal contraception and AEDs does not adversely affect seizure control. Careful patient management, including the use of increased estrogen doses (> or =50 microg) in patients receiving enzyme-inducing AEDs, may further minimize the risk for unintended pregnancy. Special considerations in women of childbearing age include decreased compliance and disease prevention. Although adequate seizure control is the critical requirement of an AED, the potential for interactions with hormonal contraception and the increased risk for endocrine disturbances caused by drugs that alter hepatic microsomal enzymes suggest additional potential advantages for AED treatment that does not affect these enzymes. Both the constellation of physicians treating women with epilepsy and the patients themselves have a poor understanding of the spectrum of reproductive health issues involved, and increased awareness is needed to improve patient management.

Adolescent↗
PubMed2004

Neonatal seizures.

Neonatal seizures typically indicate significant underlying disease.They are poorly classified, under-recognized, and often difficult to treat. Recognition of etiology is often helpful in prognosis and treatment; the most common is hypoxic-ischemic encephalopathy. Patients generally have a poor prognosis, with most developing a severe encephalopathy and epilepsy. Studies suggest that neonatal seizures and their etiology have a significant impact on the developing brain; it is critical to recognize seizures early and initiate immediate antiepileptic therapy. Continuous computerized simultaneous video electroencephalograph monitoring is imperative;at-risk infants will frequently have electrographic seizures without clinical manifestations. Although there are antiepileptic therapies for neonatal seizures, they are ineffective in over 35% of cases. The goal of research should be the development of more effective therapies for neonatal seizures, regardless of etiology.

Electroencephalography↗
PubMed2004

Ketogenic diet: outpatient initiation, without fluid, or caloric restrictions.

Although the ketogenic diet has been used for more than 80 years, the optimal methods of initiating the diet and its maintenance have not been clearly defined. This retrospective study was performed to review our experience with initiation of the ketogenic diet in the outpatient and inpatient settings and maintenance of the diet without fluid or caloric restriction. We analyzed 54 patients who had medically intractable epilepsy of whom 44% manifested some degree of mental retardation, 80% had multiple seizure types, and failed on average 4.8 antiepileptic drugs. Forty-four patients underwent induction of the ketogenic diet on an outpatient basis and 21 as inpatients. Three patients in each group were fasted at the initiation of the diet. No significant differences were observed with regard to seizure control in that 62% and 71% had greater than 50% improvement in the outpatient and inpatient groups, respectively. Both groups manifested improvement in alertness and social interaction. The efficacy of a ketogenic diet in the symptomatic epilepsies was confirmed, and benefit for medically refractory childhood absence epilepsy was documented. We conclude that a prospective, randomized trial is necessary to compare outpatient vs inpatient initiation of the ketogenic diet and the utility of fluid and caloric restriction.

Ambulatory Care↗
PubMed2004

Progressive myoclonic epilepsy.

Progressive myoclonic epilepsies (PMEs) are a group of rare disorders characterized by the occurrence of seizures, myoclonus, and progressive neurological dysfunction. This article discusses epidemiology, genetics, pathology, clinical manifestations, EEG characteristics, methods of diagnosis and treatment of the most common causes of PME, including Unverricht-Lundborg Disease (Baltic Myoclonus), MERRF, neuronal ceroid lipofuscinosis, dentatorubropallidoluysan atrophy, Gaucher disease, Lafora disease, and sialidosis. The aim of this paper is to provide clinicians with useful clinical information in order to facilitate the diagnosis and treatment of these rare diseases.

Humans↗
PubMed2004

Neurodevelopmental outcomes of children born to mothers with epilepsy.

Most children born to women with epilepsy are normal, but there is an increased risk of abnormal functional neurodevelopment in these children. Although there are many contributing factors, antiepileptic drugs (AEDs) may play a role. Most women with epilepsy must take AEDs during pregnancy because the potential for injury from seizures to both mother and fetus is a greater risk. AEDs are also used to treat other disorders, including depression and pain. Thus, an understanding of the effects of AEDs on the unborn child is relevant to physicians who treat nonepileptic mothers as well. This review discusses animal and human studies of the neurodevelopmental effects of AEDs and briefly reviews the possible mechanisms underlying these effects. Flaws in the methodology of some studies of these effects require that the results be interpreted cautiously and highlight the need for well-designed studies to explore this issue further.

Anticonvulsants↗
PubMed2003

Infantile spasms.

Infantile spasms is a catastrophic form of epilepsy found only in infants and young toddlers, with the peak incidence between 4 - 7 months of age. Estimated prevalence is 1 in 2000 - 6000 live births. There are many causes of infantile spasms, including tuberous sclerosis, hypoxic-ischaemic injury, congenital infectious diseases, inborn errors of metabolism, malformations of cortical development, genetic syndromes such as Aicardi's syndrome and chromosomal abnormalities. A small percentage of patients have idiopathic infantile spasms, with normal growth and development prior to the onset of infantile spasms and no known aetiology. Because of the poor prognosis of infantile spasms, treatment is usually aggressive and immediate, with the hopes of altering the natural history of the disease. The majority of patients with infantile spasms have a poor prognosis with intractable epilepsy, severe developmental delays and/or significant cognitive impairments. Of all patients with infantile spasms, 70 - 90% have mental retardation. Furthermore, 20 - 50% of patients with infantile spasms develop Lennox-Gastaut syndrome with multiple seizure types, cognitive impairments and a markedly abnormal electroencephalogram, arguably one of the most difficult epilepsy syndromes to treat. Infantile spasms are resistant to most of the standard antiepileptic drugs. Adrenocorticotropin hormone (ACTH) or oral steroids result in a significant reduction of seizures, as well as an improvement in the electroencephalogram. Some studies have indicated that infants treated with ACTH within the first month of onset have a more favourable prognosis. Vigabatrin has also been shown to be effective in the treatment, although it is not yet FDA-approved in the US. Valproate has also been used in the treatment of infantile spasms, with an efficacy of approximately 25 - 40%. However, in the very young infant, it does carry a high risk of fatal hepatotoxicity. Surgical resection may be the treatment of choice for those infants with focal cortical dysplasia and intractable infantile spasms. Emerging therapeutic possibilities include topiramate, felbamate, lamotrigine, zonisamide and perhaps levetiracetam. With the advancements in molecular biology, genetics and neuroimaging, there is the hope of novel therapies in the future.

Anti-Inflammatory Agents↗