Absence epilepsy beyond adolescence: an outcome analysis after 45 years of follow-up.

OBJECTIVES: Depending on patient age at onset, absence epilepsy is subdivided into childhood and juvenile forms. Absence seizures can occur several times per day (pyknoleptic course) or less frequently than daily (non-pyknoleptic course). Seizures typically terminate before adulthood, but a quarter of patients need ongoing treatment beyond adolescence. Little is known about their long-term seizure and psychosocial outcome. METHODS: Files of 135 outpatients with absence epilepsy (76 females; 123 had additional generalised tonic-clonic seizures) were retrospectively analysed after a median follow-up of 45.4 years (IQR: 31.9-56.2). Eighty-two subjects completed an additional interview. Patients were dichotomised according to age at epilepsy onset (childhood: n=82; juvenile: n=53) and course of absence seizures (pyknoleptic: n=80; non-pyknoleptic: n=55). RESULTS: Among all patients, 53% achieved 5-year terminal seizure remission, 16% without antiepileptic medication. Median age at last seizure was lower in patients with childhood onset of absence epilepsy (37.7 years) versus juvenile onset (44.4 years; P≤0.01). However, rates and duration of terminal seizure remission were similar. Pyknoleptic versus non-pyknoleptic course of absence seizures made no difference for long-term seizure outcome. Multivariate analysis identified only higher age at investigation to be associated with terminal 5-year seizure remission. Regarding aspects of psychosocial outcome, there were no significant differences between the respective subgroups. CONCLUSIONS: These data indicate that if absence epilepsy persists beyond adolescence, long-term seizure and psychosocial outcome do not differ between childhood and juvenile onset or between pyknoleptic and non-pyknoleptic course of absence epilepsy. However, higher patient age increases the chance of terminal seizure remission.

Long-term outcome in adolescent-onset generalized genetic epilepsies.

OBJECTIVE: Until now, it has been unclear if the three subsyndromes of adolescent-onset generalized genetic epilepsy (GGE) differ in long-term prognosis. Therefore, this study aimed to compare long-term seizure outcome in juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and epilepsy with generalized tonic-clonic seizures alone (EGTCS). METHODS: This retrospective study is based on the archive of an institutional tertiary care outpatient clinic for adult patients with epilepsy. Charts of 870 epilepsy outpatients were reviewed among whom 176 had adolescent-onset GGE (53 JAE, 66 JME, 57 EGTCS). Median patient age at investigation was 60 years; median follow-up time was 42.5 years. If possible, GGE patients were additionally interviewed on psychosocial and clinical variables. RESULTS: Age at first seizure was significantly higher in EGTCS patients (median 18 years) than in patients with JAE or JME (14 years each; p ≤ 0.001). Long-term seizure outcome hardly differed between the three subsyndromes. At the end of follow-up, 60% of all patients were in 5-year terminal seizure remission, and in 14%, epilepsy even had resolved (>10 years without seizures, >5 years without pharmacotherapy). Twenty percent of patients had persistent seizures during the last year of follow-up. Across all patients, 23% reported a psychiatric comorbidity, 87% had married, and 57% had achieved university entrance qualification. SIGNIFICANCE: Long-term outcome was shown to be highly similar across all subsyndromes of adolescent-onset GGE. Even in a selection of difficult-to-treat epilepsy patients still attending an adult epilepsy clinic, most become seizure-free. To confirm these findings, prospective studies are needed.

Psychosocial long-term outcome in juvenile myoclonic epilepsy.

PURPOSE: Juvenile myoclonic epilepsy (JME) is a well-defined subsyndrome of idiopathic generalized/genetic epilepsy. It is allegedly related to specific personality characteristics and has been associated with unfavorable social outcome. We aimed to analyze psychosocial outcome in patients with JME. To delineate consequences of the chronic seizure disorder from possible neurobiologic contributions being inherent to the condition itself, we compared social outcome in JME subjects with that of age- and sex-matched control patients with absence epilepsy (AE). METHODS: Patients with an epilepsy course of at least 20 years were included. All JME and AE patients (n = 41 in each group) answered a structured questionnaire asking about seizures, treatment, and psychosocial variables. In addition, patients with JME were assessed with the Quality of Life in Epilepsy Inventory 31 (QOLIE-31). RESULTS: In JME, 46.3 years (20-69) after onset of epilepsy, the overall psychosocial long-term outcome was favorable (80.5% of patients had never been unemployed for more than 1 year, 90.2% were well integrated into social context). Quality of life in all inquired subdomains revealed high scores. Compared with AE controls, JME patients did not perform worse regarding psychosocial outcome; rate of university access and degrees in JME patients was even higher (70% vs. 34%, p = 0.001). JME patients showed a high level of quality of life, and current or previous psychiatric comorbidity was associated significantly with lower overall quality of life scores (p = 0.02). SIGNIFICANCE: Our long-term study on JME patients demonstrated favorable psychosocial outcome that contrasted previous findings. This is the first study to compare social outcome in JME with another genetically determined form of epilepsy. Similar outcomes in JME and AE patients argue against specific neurobiologic alterations in JME that may predispose to social deficits. In JME, reduced quality of life seems to be associated with psychiatric comorbidity.

Discontinuing antiepileptic drugs in long-standing idiopathic generalised epilepsy.

BACKGROUND: Once adults with long-standing idiopathic generalised epilepsy have achieved stable seizure remission, patients or physicians may attempt to discontinue their antiepileptic drug treatment. To date, risk of subsequent seizure relapse across the four idiopathic generalised epilepsy syndromes is largely unknown, and so are the clinical variables associated. METHODS: For this retrospective observational study, 256 adult outpatients with idiopathic generalised epilepsy were evaluated. Data were obtained from outpatient charts and, if possible, from additional telephone or mail interviews. RESULTS: In 84 patients (33%), antiepileptic medication was discontinued at least once. Median patient age at antiepileptic drug withdrawal was 33 years, and median duration of subsequent follow-up was 20 years. Seizures recurred in 46% of patients after a median latency of 11 months. Following multivariable analysis, seizure relapse was independently associated with short duration of seizure remission beforehand. If medication was withdrawn after < 5 years of seizure freedom, two-thirds of patients had a seizure relapse, while among those in remission for ≥ 5 years, only one-third relapsed. CONCLUSIONS: Discontinuation of antiepileptic drug treatment can be successful in every other adult with long-standing idiopathic generalised epilepsy. Short duration of prior seizure remission appears to be a relevant predictor of seizure recurrence.