Brain functional imaging SPECT in agyria-pachygyria

Chiron, C.; Nabbout, R.; Pinton, F.; Nuttin, C.; Dulac, O.; Syrota, A.

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Volume 24, Issue 2 , Pages 109-117, June 1996

Brain functional imaging SPECT in agyria-pachygyria

C. Chiron
- Corresponding author. Service de Neuropediatrie, Hopital Saint-Vincent de Paul, 82 avenue Denfert-Rochereau, 75674 Paris Cedex 14, France. Tel.: +33 (1) 40 48 80 45; fax: + 33 (1) 40 48 80 46.
- Neuropediatric Department, INSERM U29, and University René Descartes, Hospital Saint-Vincent de Paul, Paris, France
- Service de Hopital, Frederic Joliot, Atomic Energy Commission, Orsay, France
R. Nabbout
- Neuropediatric Department, INSERM U29, and University René Descartes, Hospital Saint-Vincent de Paul, Paris, France
F. Pinton
- Service de Hopital, Frederic Joliot, Atomic Energy Commission, Orsay, France
C. Nuttin
- Service de Hopital, Frederic Joliot, Atomic Energy Commission, Orsay, France
O. Dulac
- Neuropediatric Department, INSERM U29, and University René Descartes, Hospital Saint-Vincent de Paul, Paris, France
A. Syrota
- Service de Hopital, Frederic Joliot, Atomic Energy Commission, Orsay, France

Received 21 July 1995; received in revised form 15 December 1995; accepted 16 December 1995.

Abstract

Agyria-pachygyria or lissencephaly type I, a diffuse cortical malformation, provides infantile spasms (IS) which are refractory and persisting after the first decade, an age at which IS have disappeared in the other causes. In order to study the functional postnatal development of the lissencephalic cortex, we measured regional cerebral blood flow (rCBF) using SPECT (Single photon emission computed tomography) and ¹³³Xe in 14 children with lissencephaly, aged from 4 months to 12 years (mean = 40 months) compared to normal children of the same age range and to children with cryptogenic IS aged from 3 months to 3 years (mean = 13 months). rCBF was calculated in frontal (FR) and parieto-temporo-occipital (PTO) cortex as well as the ratio FR/PTO. FR/PTO was higher in lissencephalic patients than in controls (P < 0.001) due to higher FR rCBF (P < 0.001), particularly in patients aged less than 3 years. FR/PTO was also higher in lissencephalic patients than in patients with cryptogenic IS (P < 0.001) also due to higher FR rCBF (P < 0.001). The values of FR/PTO and FR rCBF remained stable during the first years of life and did not exhibit any age- or topography-related changes as they do in controls or in patients with cryptogenic IS. These results suggest that the normal process of postnatal development in the brain is lacking in agyria-pachygyria. That could play a role in determining the persistance of epileptic spasms, the specific seizure type of this malformation.

Keywords: Agyria, Lissencephaly, SPECT, Child, Infantile spasm

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