Child's Nervous System

, Volume 16, Issue 10–11, pp 719–723 | Cite as

Dysgenetic mesial temporal sclerosis: an unrecognized entity

  • O. Vernet
  • Jean-Pierre Farmer
  • José Luis Montes
  • Jean-Guy Villemure
  • Kathleen Meagher-Villemure
Original Paper

Abstract 

Mesial temporal sclerosis (MTS) is the most frequently encountered lesion in adult patients with intractable temporal epilepsy; it is found in isolation in approximately two-thirds of surgically treated cases. Whereas the exact etiology of MTS is still controversial, several reports suggest that this pathologic lesion is both the cause and the consequence of chronic seizures and develops progressively during childhood secondary to recurrent seizures. In order to evaluate the clinical importance of MTS in children, we retrospectively reviewed the clinical charts of children who underwent surgery for medically intractable temporal epilepsy and report cases presenting an amygdalo-hippocampic dual pathology. Six children aged 1.5–16 years (mean±SD: 7.5±3 years) presenting with partial complex seizures (5 cases) or extension spasms (1 case), with onset from 6 months to the age of 8.5 years (mean seizure onset±SD: 3±5 years) underwent anterior temporal lobectomy including resection of the amygdala and hippocampus. All patients exhibited variable degrees of severity of neuronal loss and gliosis in the amygdala and/or hippocampus. The pathological picture of MTS was not isolated, however. Careful pathological examination has thus shown foci of amygdalo-hippocampic neuronal dysplasia in six patients, with concomitant bilaminated fascia dentata in two cases. Postoperatively, no mortality or morbidity was encountered. After a mean follow-up of 2.5 years, four patients are seizure free. One patient had a 80% rate of improvement in seizure frequency, though still having occasional febrile convulsions. In another patient, complex partial seizures resolved, but rare episodes of absence were still observed. These data are in keeping with the hypothesis that MTS could be secondary to repeated seizures. The analysis of this series of patients could suggest that mesiotemporal dysplastic lesions within the amygdalo-hippocampic structures induce seizures, which, in turn, will favor the development of MTS during childhood. MTS could then lead to synaptic reorganization, which can express abnormal hyperexcitability and result in more recurrent seizures. In this way a vicious circle is set up, which may explain the progression of seizures in some patients.

Keywords Mesial temporal sclerosis Pediatric epilepsy Hippocampus Dysgenesis 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • O. Vernet
    • 1
  • Jean-Pierre Farmer
    • 2
  • José Luis Montes
    • 2
  • Jean-Guy Villemure
    • 1
  • Kathleen Meagher-Villemure
    • 3
  1. 1.Service de Neurochirurgie, BH 10-163, Centre Hospitalier Universitaire Vaudois (CHUV), Rue du Bugnon 46, 1011 Lausanne, Switzerland e-mail: Olivier.Vernet@chuv.hospvd.ch Tel.: +41-21-3142604 Fax: +41-21-3142605CH
  2. 2.Service of Pediatric Neurosurgery, The Montreal Children’s Hospital, McGill University, Montréal, Québec, CanadaCA
  3. 3.Institut de Pathologie, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, SwitzerlandCH

Personalised recommendations