Epilepsia, 47(4):766–772, 2006 Blackwell Publishing, Inc. C  2006 International League Against Epilepsy Language-induced Epilepsy, Acquired Stuttering, and Idiopathic Generalized Epilepsy: Phenotypic Study of One Family ∗ Maria Paola Valenti, ∗ Gabrielle Rudolf, ∗ Sophie Carr´ e, ∗ Pascal Vrielynck, ∗ Anne Thibault, †Pierre Szepetowski, and Edouard Hirsch ∗ Department of Neurology, Strasbourg, and †INSERM UMR491, Marseille, France Summary: Purpose: Language-induced epilepsy involves seizure precipitation by speaking, reading, and writing. Seizures are similar to those of reading epilepsy (RE). The nosologic po- sition of language-induced epilepsy is not clear. We performed a clinical and neurophysiological study in a multigenerational family with the association of idiopathic generalized epilepsy (IGE) with ictal stuttering as a manifestation of reflex language- induced epilepsy. Methods: Nine members on three generations were stud- ied. All patients underwent video-polygraphic EEG recordings (awake and during sleep). A standardized protocol was applied to test the effect of language and non–language-related tasks. Results: Six patients presented language-induced jaw jerking that mimicked stuttering and corresponded to focal myoclonus involving facial muscles. This was associated with an IGE pheno- type in four of these patients. Focal EEG spikes were found in all six patients by visual analysis and/or back-averaging techniques. The focal spikes were either asymptomatic (when followed by a slow wave) or symptomatic of facial myoclonia (when isolated). Levetiracetam, used as add-on or monotherapy in four patients, suppressed ictal stuttering. One additional case only had a phe- notype of IGE without focal features. Conclusions: This family study demonstrates the phenotypic heterogeneity of the association of IGE phenotype with ic- tal stuttering (language-related reflex seizure). Our data sug- gest that this particular form of reflex epilepsy related to lan- guage has more similarities with generalized epilepsies than with focal ones. Neurophysiological investigations should be performed more systematically in patients with acquired stutter- ing, especially if there is family history of IGE. Key Words: Stuttering—Language-induced epilepsy—Reading epilepsy— Idiopathic generalized epilepsy—Symptomatic spikes. Language-induced epilepsy, first described by Geschwind and Sherwin in 1967 (1), is a rare syndrome in which seizures are triggered by three modalities of language: reading, speaking, or writing. Seizures are characterized by speech arrest, myoclonic jerks of facial muscles, fol- lowed by staring. To our knowledge, no familial cases have ever been reported. Stuttering represents a speech- production disorder defined by involuntary repetition or prolongation of syllables, sometimes associated with brief speech arrest (2). Acquired stuttering as a manifestation of language-induced epilepsy has been recently described (3). Primary reading epilepsy (RE) is suggested to be a reflex epilepsy syndrome without specifying whether it is focal or generalized (4). In RE, seizures are precipi- tated by reading (5,6). Seizures are characterized by re- peated jaw jerks during reading, whether silent or aloud, that may progress to a generalized tonic–clonic seizure if Accepted November 10, 2005. Address correspondence and reprint requests to Dr. E. Hirsch at D´ epartement de Neurologie, 1 place de l’Hˆ opital, 67091 Strasbourg, France. E-mail: Edouard.Hirsch@chru-strasbourg.fr the triggering factor is continued. Spontaneous seizures may be present, and, in some cases, other nonspecific fac- tors than reading may trigger seizures (i.e., visual pat- tern, writing, and/or praxis). RE is often idiopathic, some- times symptomatic, and the occurrence of familial cases has been reported (7,8). RE is presently classified as “localization-related epilepsies and syndromes” (Com- mission on Classification and Terminology of Interna- tional League Against Epilepsy, 1989), but classification is still under debate; electroclinical data strongly suggest several similarities with idiopathic generalized epilepsies (IGEs). IGE syndromes are characterized by seizures in which clinical manifestations derive from the involvement of both cerebral hemispheres, with absences, generalized tonic–clonic seizures, and bilateral myoclonic jerks, pre- senting an EEG pattern of generalized abnormalities (9). The nosologic classification of language-induced epilepsy and its differentiation from RE remain unclear. Reported cases of language-induced epilepsy with seizures being precipitated by different linguistic activities resemble RE but are more heterogeneous with respect to precipitating factors than is “pure” RE. 766