Faculty Bibliography

A 10-year-old boy developed febrile convulsions at age 2 years, and multiple types of nonfebrile generalized seizures over the ensuing months and years. Gestation, birth, and early development were normal, as were initial electroencephalogram (EEG) and brain-imaging studies. By age 5 years, he had developed behavioral difficulties, and the EEG demonstrated both interictal and ictal generalized epileptiform patterns, as well as mild background slowing. Seizures were poorly controlled with multiple medication trials. There was a strong family history of clinically benign febrile seizures in the maternal lineage. Genetic analysis revealed a novel mutation in the voltage-gated neuronal sodium channel SCN1A. This patient's relatively malignant clinical course is consistent with 'extended spectrum' generalized epilepsy with febrile seizures plus (GEFS+), and along with the family history illustrates the phenotypic variability of this disease

Background. Spanish-speaking adults are the largest minority Population group in the United States and are disproportionately afflicted by epilepsy. Methods. A unique 78-item survey instrument conducted entirely in Spanish and devoted to the topic of epilepsy was administered to 760 Spanish-speaking adults in seven large U.S. Hispanic metropolitan areas representing a cross section of the U.S. Hispanic community. The answers were compared with those of 272 non-Hispanic controls administered the same survey in English in June 2004. Results. The Hispanic sample correlated well with U.S. Census data. Spanish-speaking adults are mostly unaware about epilepsy, with 21% reporting no familiarity with the condition (P = 0.0001). The vast majority of Hispanics use the term convulsiones or ataque to describe a seizure. Thirteen percent of Hispanics with less than high school education believe that epilepsy is contagious (P = 0.0001); 8% see 'sins' as a cause of seizures (P = 0.0001); and 10% agree that 'exorcism' would be a good remedy (P = 0.002). Conclusions. There is considerable misinformation about epilepsy in the U.S. Hispanic community. Neurologists must be made aware of U.S. Hispanic attitudes and beliefs regarding epilepsy to provide culturally competent care. (c) 2005 Elsevier Inc. All rights reserved

A 10-year-old boy developed febrile convulsions at age 2 years, and multiple types of nonfebrile generalized seizures over the ensuing months and years. Gestation, birth, and early development were normal, as were initial EEG and brain imaging studies. By age 5 years, he had developed behavioral difficulties, and the EEG demonstrated both interictal and ictal generalized epileptiform patterns, as well as mild background slowing. Seizures were poorly controlled with multiple medication trials. There was a strong family history of clinically benign febrile seizures in the maternal lineage. Genetic analysis revealed a novel mutation in the voltage-gated neuronal sodium channel SCN1A. This patient's relatively malignant clinical course is consistent with 'extended spectrum' generalized epilepsy with febrile seizures plus (GEFS+), and along with the family history illustrates the phenotypic variability of this disease

Many IV antiepileptic drugs administered in emergency situations to patients with prolonged seizures have serious adverse effects. For this reason, the authors conducted a multicenter, open-label, prospective, dose-escalation study of IV valproate sodium administered to patients with epilepsy at rates of infusion of up to 6 mg/kg/minute and doses of up to 30 mg/kg. Valproate sodium had no clinically significant negative effects on blood pressure and pulse rate and caused only mild-to-moderate, reversible adverse events.

BACKGROUND: Monotherapy is the goal for pharmacological treatment of epilepsy. Well-controlled trials have established the efficacy of some of the newer antiepileptic drugs (AEDs) as monotherapy. OBJECTIVE: To review clinical data and expert opinions pertinent to the evaluation of most of the newer AEDs as monotherapy for epilepsy. DATA SOURCES: The MEDLINE database was searched for clinical trials using newer AEDs. Reference sections of review articles were manually searched to identify relevant studies not retrieved in MEDLINE. STUDY SELECTION: The resulting list of references was manually reviewed to identify monotherapy studies. RESULTS: Lamotrigine and oxcarbazepine demonstrated efficacy in randomized active-control trials in patients with newly diagnosed epilepsy and in substitution trials in patients refractory to conventional AEDs. CONCLUSION: Lamotrigine and oxcarbazepine are as effective as conventional AEDs at controlling partial seizures and are better tolerated

Cerebral lateralization may be important in neural control of immune function. Animal studies have demonstrated differential effects of left and right brain lesions on immune function, but human studies are inconclusive. Here, we show that resections in the language dominant hemisphere of patients with epilepsy reduce lymphocytes, total T cells, and helper T cells. In contrast, resections in the language nondominant hemisphere increased the same cellular elements. T-cell responses to mitogens and microbial antigens were not differentially affected. Left/right arm histamine skin response ratios were altered in patients with left cerebral epileptic focus, and flare skin responses were reduced by left cerebral resections in contrast with an increase after right cerebral resections. The findings demonstrate a differential role of the left and right cerebral hemispheres on immune functions in humans.

Antiepileptic drugs (AEDs) are associated with bone disease. Early reports found rickets in children and osteomalacia in adults, but those reports were primarily in institutionalized persons. Studies in ambulatory adults and children taking AEDs do not reveal rickets or osteomalacia but do report abnormalities in biochemical indexes of bone mineral metabolism and density. In addition, fracture rates are increased in AED-treated patients. AEDs that induce the cytochrome P450 enzyme system are most commonly associated with abnormalities in bone. Emerging data suggest that valproate, an enzyme inhibitor, may also affect bone, and there is limited information on the newer AEDs. Several theories on the mechanism of AED-associated bone disease have been proposed, but no single one explains all the reported findings. Identifying AED-treated patients who are at risk for or have bone disease is important, as multiple therapies are available.

Studies in the general population demonstrate that anxiety disorders are associated with impaired quality of life and function. Available evidence suggests that comorbid anxiety disorders are frequent among patients with epilepsy but that neither the interrelationships between them nor the impact of anxiety disorders on functional outcome is well studied. The study and management of anxiety disorders are further complicated by the occurrence of seizures, the temporal relationship between seizures and anxiety symptoms/syndromes, and the influence of antiepileptic drugs. Increased recognition of anxiety disorders among patients with epilepsy and evaluation of the potential impact of these disorders on functional outcome and the beneficial and detrimental effects of antiepileptic drugs in clinical practice are needed