Background Geographical differences in the incidence of amyotrophic lateral sclerosis (ALS) have been reported in the literature but comparisons across previous studies are limited by different methods in case ascertainment and by the relatively small size of the studied populations. To address these issues, the authors undertook a pooled analysis of European population based ALS registries. Methods All new incident ALS cases in subjects aged 18 years old and older were identified prospectively in six population based registries in three European countries (Ireland, UK and Italy) in the 2 year period 1998–1999, with a reference population of almost 24 million. Results Based on 1028 identified incident cases, the crude annual incidence rate of ALS in the general European population was 2.16 per 100 000 person years; 95% CI 2.0 to 2.3), with similar incidence rates across all registries. The incidence was higher among men (3.0 per 100 000 person years; 95% CI 2.8 to 3.3) than among women (2.4 per 100 000 person years; 95% CI 2.2 to 2.6). Spinal onset ALS was more common among men compared with women, particularly in the 70–80 year age group. Disease occurrence decreased rapidly after 80 years of age. Conclusions ALS incidence is homogeneous across Europe. Sex differences in incidence may be explained by the higher incidence of spinal onset ALS among men, and the age related disease pattern suggests that ALS occurs within a susceptible group within the population rather than being a disease of ageing.

Objectives: Previous studies that have assessed the risk of developing epilepsy have failed to account for the competing risk of death, significant in the elderly where epilepsy incidence is highest. We report the lifetime risk for epilepsy, accounting for the competing risk of mortality. Methods: Lifetime risk and cumulative incidence of epilepsy were examined among Rochester, MN, residents between 1960 and 1979. Age-, gender-, and calendar year–specific deaths were obtained for Rochester, MN. Lifetime risk was calculated as the conditional probability of developing epilepsy by a specific age for a person reaching that age who had not yet developed epilepsy. Lifetime risk and cumulative incidence were compared for age and time period. Results: We identified 412 individuals with incident epilepsy diagnosed between January 1, 1960, and December 31, 1979. Lifetime risk was 1.6% to age 50 and 3.0% to age 80; cumulative incidence was 1.7% to age 50 and 3.4% to age 80. Similar differences were seen across epilepsy etiologies. Lifetime risk through 87 years of age increased over time from 3.5% in 1960–1969 to 4.2% in 1970–1979. Conclusions: One in 26 people will develop epilepsy during their lifetime. Lifetime risk provides an estimate of an individual9s risk for epilepsy over his or her remaining lifetime, translates into the number of people who are expected to develop epilepsy, and assists health care planners as they estimate service needs for epilepsy.

Summary: Purpose:  In an analysis of four case–control studies of sudden unexpected death in epilepsy (SUDEP), we found that yearly frequency of generalized tonic–clonic seizures (GTCS) and antiepileptic drug (AED) polytherapy were associated with an increased risk for SUDEP. The prior analysis, however, did not evaluate AEDs and GTCS frequency concurrently.Methods:  We combined data from the three case–control studies with information on the frequency of GTCS and AED therapy, that is, carbamazepine, phenytoin, valproic acid, and other AED therapy. Number of AEDs was also considered. Lamotrigine and GTCS frequency were considered separately in two of the case–control studies. Logistic regression analysis was used to evaluate GTCS frequency, each of the AEDs, and number of AEDs. Adjusted analysis of the different AEDs accounted for study, age at death, gender, and GTCS frequency.Key Findings:  In crude analysis, GTCS frequency, AED polytherapy, and number of AEDs were associated with an increased risk for SUDEP. Analysis of individual AEDs and of number of AEDs, adjusting for GTCS frequency, revealed no increased risk associated with AEDs as monotherapy, polytherapy, or total number. GTCS frequency remained strongly associated with an increased risk for SUDEP.Significance:  Our findings—that none of the AEDs considered were associated with increased SUDEP risk as monotherapy or as polytherapy when GTCS frequency was taken into account—provide a consistent message that number of GTCS increases SUDEP risk and not AEDs. These results suggest that prevention of SUDEP must involve increased efforts to decrease GTCS frequency in order to avert the occurrence of this devastating epilepsy outcome.

Summary: Purpose:  In a prospective study, Consequences of Prolonged Febrile Seizures in Childhood (FEBSTAT), we determined the frequency of human herpesvirus (HHV)‐6 and HHV‐7 infection as a cause of febrile status epilepticus (FSE).Methods:  Children ages 1 month to 5 years presenting with FSE were enrolled within 72 h and received a comprehensive assessment including specimens for HHV‐6 and HHV‐7. The presence of HHV‐6A, HHV‐6B, or HHV‐7 DNA and RNA (amplified across a spliced junction) determined using quantitative polymerase chain reaction (qPCR) at baseline indicated viremia. Antibody titers to HHV‐6 and HHV‐7 were used in conjunction with the PCR results to distinguish primary infection from reactivated or prior infectionKey Findings:  Of 199 children evaluated, HHV‐6 or HHV‐7 status could be determined in 169 (84.9%). HHV‐6B viremia at baseline was found in 54 children (32.0%), including 38 with primary infection and 16 with reactivated infection. No HHV‐6A infections were identified. HHV‐7 viremia at baseline was observed in 12 children (7.1%), including eight with primary infection and four with reactivated infection. Two subjects had HHV‐6/HHV‐7 primary coinfection at baseline. There were no differences in age, characteristics of illness or fever, seizure phenomenology or the proportion of acute EEG or imaging abnormalities in children presenting with FSE with or without HHV infection.Significance:  HHV‐6B infection is commonly associated with FSE. HHV‐7 infection is less frequently associated with FSE. Together, they account for one third of FSE, a condition associated with an increased risk of both hippocampal injury and subsequent temporal lobe epilepsy.