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Abstract:

Abstract Introduction The aim of our study is to describe the epidemiology , clinical evolution , and the anatomical and neurological factors involved in polymicrogyria in 34 patients with this disorder . Subjects and methods We have compiled 34 patients diagnosed and/or in follow - up at the Department of Paediatric Neurology of the Hospital Infantil Niño Jesús between 1995 and 2010 . All the patients had a magnetic resonance imaging suggestive of polymicrogyria , and most of the patients still have periodic checks , thus their outcome is known . Results The large majority were male ( 76 .5% ) . The median age at presentation was 10 months ; the reason for the study was psychomotor or mental delay ( 44% ) followed by seizures ( 38 .2% ) . During the condition patients presented with epilepsy ( 61 .7% ) , infantile cerebral palsy ( 47% ) , psychomotor/mental retardation ( 94 .1% ) , pervasive developmental disorder ( 26 .4% ) , behavioural disturbances ( 38 .2% ) , neurosensory deficit ( 35 .2% ) and microcephaly 67 .6% . In 82 .3% of patients there was bilateral involvement ( 42 .8% perisylvian ) . Other abnormalities were observed in the MRI of 58 .8% of patients . The electroencephalogram at diagnosis showed changes in 41 .1% , and this rose to 67 .6% during follow - up . 61 .7% received antiepileptic treatment was received by 61 .7% of patients , with 52 .3% requiring ≥2 drugs . Epilepsy surgery was performed on two patients . Some type of sequelae was observed in 91 .1% of patients . The aetiology was unknown in 61 .7% ; a congenital infection was suspected in 10 patients and syndromic or polymalformative disorder in three patients . Conclusions This study shows the range of clinical and radiological expression in polymicrogyria , in addition to the possibilities for the future in terms of determining the aetiology of this pathology .
psychomotor or mental delay
infantile cerebral palsy
psychomotor/mental retardation
pervasive developmental disorder
behavioural disturbances
neurosensory deficit