• Anna Jansen
  • Frederick Andermann
  • R. Valerio
  • Renzo Guerrini
  • Richard Leventer
  • Y. Robitaille
  • H.j. Meencke
  • François Dubeau
  • E Andermann
Rationale: Polymicrogyria (PMG) is a relatively common malformation
of cortical development. However, the pathogenesis, epileptogenesis
and anatomo-clinical correlation of PMG require further clarification.
We aim to 1. study the extent and type of pathological changes in
polymicrogyric lesions as well as in adjacent non-polymicrogyric tissue
2. study the presence of other malformations of cortical development, as
well as other pathological conditions previously reported in association
with PMG 3. study the anatomo-clinical correlation in order to better understand
language impairment and motor involvement frequently seen
in PMG patients 4. study the correlation between pathology and imaging
findings 5. obtain further insight into the pathogenesis and epileptogenesis
of PMG.
Methods: Medical records, EEG, imaging and autopsy data on seven
patients from four tertiary centers were reviewed.
Results: Two patients had symmetrical and 2 had asymmetrical bilateral
perisylvian PMG, 1 had unilateral hemispheric PMG and a small
contralateral PMG lesion, 1 had parieto-occipital PMG. Multiple associated
CNS lesions were identified, including periventricular nodular heterotopia,
non-laminated heterotopic neurons in the cerebral white matter,
microscopic neuroglial heterotopias in the cerebellar white matter, and
DNT. Associated congenital malformations included cardiac malformations,
congenital hemiparesis, club foot, arthrogryposis, and cranio-facial
dysmorphism. When detailed seizure history was available, pathology
findings correlated with seizure patterns present during life. The cause
of death was seizure related in 2 patients.
Conclusions: This is the first series of brain autopsy findings in PMG
patients. Our study illustrates that PMG is often associated with diffuse
microscopic migration disorders that may contribute to the epileptogenesis
in PMG patients. The absence of underlying infectious or vascular
pathology reflects the developmental origin of PMG and supports the
involvement of genetic factors in its pathogenesis. The extent of the
polymicrogyric lesion can be more widespread than detected by current
MRI techniques. Associated congenital malformations seem more
frequent than recognized to date. (Supported by Savoy Foundation for
Epilepsy Research.)
Original languageEnglish
Pages (from-to)33-33
Number of pages1
Issue numbers7
Publication statusPublished - 2004
Event2004 Annual Meeting of the American Epilepsy Society - New Orleans, United States
Duration: 3 Dec 20047 Dec 2004

    Research areas

  • Polymicrogyria, Neuropathology, Epileptogenesis

ID: 2320338