[Polymicrogyria: epidemiology, neurological and anatomical factors and clinical outcome in a series of 34 cases]

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.