Perisylvian syndrome, bilateral,  congenital

See also polymicrogyrias

It is the most frequent form of polymicrogyria: it is bilateral, symmetric, and localized around the sylvian fissures (bilateral perisylvian polymicrogyria). Most cases are sporadic but there are familial cases with all modes of heredity.


Two genetic forms have been identified:

1.        an X-linked form (Xq28)

2.        a form associated with mutations of the GPR56 gene on chromosome 16. There are non-genetic forms due to intrauterine   cytomegalovirus infections or to placental perfusion abnormalities.


Clinical picture:

-        epilepsy, most often resistant to treatment: tonico-clonic seizures and/or frequent absence seizures

-        slight to moderate mental retardation

-        diplegia of the muscles of the face, jaws and pharynx causing feeding and/or speech learning difficulties.


Anesthetic implications:

mental retardation, epilepsy, side-effects of the antiepileptic treatment.


References : 

-        Quelin C, Poirier K, Bahi-Buisson N.
Les polymicrogyries : des formes isolées aux syndromes polymicrogyriques.
Neurone 2012 ; 17 : 16-22.


Updated: June 2019