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Bilateral frontal polymicrogyria

A newly recognized brain malformation syndrome

From the Division of Neurology (Dr. Guerrini), King’s College, University of London, UK, and the Institute of Child Neurology and Psychiatry, University of Pisa-IRCCS Stella Maris Foundation, Pisa, Italy; the Departments of Radiology (Neuroradiology), Neurology, and Pediatrics (Dr. Barkovich), University of California–San Francisco; the Department of Pediatrics (Dr. Sztriha), FMHS, UAE University, United Arab Emirates; and the Departments of Human Genetics (Dr. Dobyns), Neurology, and Pediatrics, The University of Chicago, IL.

Address correspondence and reprint requests to Dr. Renzo Guerrini, Academic Neuroscience Centre, King’s College Hospital Denmark Hill, London SE 59 RS, UK; e-mail: R.Guerrini{at}iop.kcl.ac.uk

Background and Objective: Polymicrogyria is a brain malformation characterized by abnormal cortical lamination, excessive cortical folding, and fusion of the cortical molecular layer. Two distinct bilateral localized forms have been described: bilateral perisylvian polymicrogyria, which has proved to be genetically heterogeneous, and bilateral parasagittal parieto-occipital polymicrogyria, which has been described only in sporadic patients. We describe 13 patients with symmetric polymicrogyria of both frontal lobes back to the precentral sulcus: bilateral frontal polymicrogyria (BFP).

METHODS: Review of clinical records, brain MRI, and EEG results of 13 patients; correlation with other regional polymicrogyrias.

RESULTS: The abnormal cortex extended from the frontal poles anteriorly to the precentral gyrus posteriorly and to the frontal operculum inferiorly and was relatively symmetric in all 13 patients. All patients presented with developmental delay and mild spastic quadriparesis, but variably impaired language development (12/13), mental retardation (11/13), and epilepsy (5/13) also occurred. BFP was sporadic in 13 of 13 patients, but 2 of 13 had consanguineous parents.

CONCLUSIONS: BFP extends the spectrum of the recognized bilateral symmetric regional polymicrogyria syndromes.

Key words: Bilateral frontal polymicrogyria—Brain malformation syndrome—Epilepsy—Mental retardation

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