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Epilepsy in twins

Insights from unique historical data of William Lennox

From the Epilepsy Research Centre (Drs. Vadlamudi and Berkovic), Department of Medicine (Neurology), University of Melbourne, Austin Health, Australia; Montreal Neurological Institute (Drs. E. Andermann and F. Andermann), Canada; Harvard Medical School (Dr. Lombroso), Boston; Beth Israel Deaconess Medical Center (Dr. Schachter), Boston, MA; and Centre for Genetic Epidemiology (R.L. Milne and Dr. Hopper), University of Melbourne, Australia.

Address correspondence and reprint requests to Professor Samuel F. Berkovic, Epilepsy Research Centre, First Floor, Neurosciences Building, Repatriation Campus, Austin Health, Banksia Street, Heidelberg West, Victoria, Australia 3081; e-mail: s.berkovic{at}unimelb.edu.au

Objective: To classify the Lennox twin pairs according to modern epilepsy classifications, use the classic twin model to identify which epilepsy syndromes have an inherited component, search for evidence of syndrome-specific genes, and compare concordances from Lennox’s series with a contemporary Australian series.

Methods: Following review of Lennox’s original files describing twins with seizures from 1934 through 1958, the International League Against Epilepsy classifications of seizures and epileptic syndromes were applied to 169 pairs. Monozygous (MZ) and dizygous (DZ) pairs were subdivided into epilepsy syndromes and casewise concordances estimated.

Results: The authors excluded 26 pairs, with 71 MZ and 72 DZ pairs remaining. Seizure analysis demonstrated strong parallels between contemporary seizure classification and Lennox’s terminology. Epilepsy syndrome diagnoses were made in 75%. The MZ and DZ casewise concordance estimates gave strong evidence for a major genetic influence in idiopathic generalized epilepsies (0.80 versus 0.00; n = 23). High MZ casewise concordances also supported a genetic etiology in symptomatic generalized epilepsies and febrile seizures. The pairs who were concordant for seizures usually had the same syndromic diagnoses in both twins (86% in MZ, 60% in DZ), suggesting syndrome-specific genes. Apart from partial epilepsies, the MZ casewise concordances were similar to those derived from Australian twin data.

Conclusions: The authors were able to apply contemporary classifications to Lennox’s twins. The data confirm genetic bases for common generalized epilepsies as well as febrile seizures and provide further support for syndrome-specific genes. Finally, comparable results to our Australian series were obtained, verifying the value of twin studies.

Received July 24, 2003. Accepted in final form November 26, 2003.