Language-induced epilepsy, acquired stuttering, and idiopathic generalized epilepsy: phenotypic study of one family.
OBJECTIVE
Language-induced epilepsy involves seizure precipitation by speaking, reading, and writing. Seizures are similar to those of reading epilepsy (RE). The nosologic position 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 studied. 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 phenotype 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 phenotype of IGE without focal features.


CONCLUSIONS
This family study demonstrates the phenotypic heterogeneity of the association of IGE phenotype with ictal stuttering (language-related reflex seizure). Our data suggest that this particular form of reflex epilepsy related to language has more similarities with generalized epilepsies than with focal ones. Neurophysiological investigations should be performed more systematically in patients with acquired stuttering, especially if there is family history of IGE.
Valenti
Maria Paola
MP
Department of Neurology, Strasbourg, France.
Rudolf
Gabrielle
G
Carré
Sophie
S
Vrielynck
Pascal
P
Thibault
Anne
A
Szepetowski
Pierre
P
Hirsch
Edouard
E
D000293:Adolescent; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D015897:Comorbidity; D004569:Electroencephalography / Q000706:statistics & numerical data*; D004576:Electromyography / Q000706:statistics & numerical data; D004829:Epilepsy, Generalized / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics*; D020195:Epilepsy, Reflex / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics*; D005190:Family; D005260:Female; D005602:France / Q000453:epidemiology; D018740:Genetic Heterogeneity; D006801:Humans; D007806:Language Disorders / Q000453:epidemiology / Q000235:genetics; D000077287:Levetiracetam; D008297:Male; D010375:Pedigree; D010641:Phenotype; D010889:Piracetam / Q000031:analogs & derivatives / Q000627:therapeutic use; D017286:Polysomnography; D013342:Stuttering / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics*; D016896:Treatment Outcome; D014743:Videotape Recording
D000927:Anticonvulsants; D000077287:Levetiracetam; D010889:Piracetam