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. 2016 Apr;57(4):648-59.
doi: 10.1111/epi.13325. Epub 2016 Feb 13.

Epilepsy in young Tsc1(+/-) mice exhibits age-dependent expression that mimics that of human tuberous sclerosis complex

Svetlana Gataullina  1   2   3   4 , Eric Lemaire  5   6 , Fabrice Wendling  7   8 , Anna Kaminska  1   2   3   9   10 , Françoise Watrin  11 , Audrey Riquet  12 , Dorothée Ville  13 , Marie-Laure Moutard  14 , Anne de Saint Martin  15   16 , Silvia Napuri  17 , Jean-Michel Pedespan  18 , Monika Eisermann  1   2   3   9   10 , Nadia Bahi-Buisson  19   20   21 , Rima Nabbout  1   2   3   10   19 , Catherine Chiron  1   2   3   10 , Olivier Dulac  1   2   3   10 , Gilles Huberfeld  1   2   3   22   23
Affiliations

Epilepsy in young Tsc1(+/-) mice exhibits age-dependent expression that mimics that of human tuberous sclerosis complex

Svetlana Gataullina et al. Epilepsia. 2016 Apr.

Abstract

Objective: To describe the epileptic phenotype of Tsc1(+/-) mice pups in comparison with age-related seizures in human tuberous sclerosis complex (TSC).

Methods: Tsc1(+/-) and control mice underwent intracranial electroencephalography (EEG) recording at postnatal ages (P)8 to P33, with linear silicon probe implanted in the somatosensory cortex of one or both hemispheres for 8-24 h. Ictal events were classified visually by independent analyzers; distinct EEG patterns were related to age and analyzed to quantify field potential characteristics and signal dynamics between hemispheres. We collected retrospectively 20 infants with prenatally diagnosed TSC and EEG before seizure onset, and analyzed the electroclinical course of epilepsy, taking into account a first-line treatment by vigabatrin.

Results: Spontaneous seizures were disclosed in 55% of Tsc1(+/-) mice at P9-18. Three ictal patterns were identified: from P9 to P12 "spike clusters" consisted of recurring large spikes without clinical correlate; "spasm-like" discharges dominated from P13 to P16 consisting of high amplitude large field potential superimposed with or followed by fast activity repeated every 2-10 s for at least 20 s, accompanied by rhythmic limb contractions; from P14 to P18 a "tonic-clonic like" pattern comprised rhythmic spikes of increasing amplitude with tonic-clonic movements. Early onset "spike clusters" were mainly unilateral, whereas "spasm-like" and "tonic-clonic like" patterns were bilateral. Interhemispheric propagation was significantly faster for "tonic-clonic like" than for "spasm-like" events. In infants diagnosed prenatally with TSC, clusters of sharp waves or spikes preceded the first seizure, and vigabatrin prevented the development of seizures. Patients treated after seizure onset developed spasms or focal seizures that were pharmacoresistant in 66.7% of cases.

Significance: Tsc1(+/-) mice pups exhibit an age-dependent seizure pattern sequence mimicking early human TSC epilepsy features. Spike clusters before seizure onset in TSC should be considered as a first stage of epilepsy reinforcing the concept of preventive antiepileptic therapy.

Keywords: Electroencephalogram; Epileptic spasms; Infants; Seizures; Spikes.

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