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Transmantle Dysplasia in Tuberous Sclerosis

Clinical Features and Surgical Outcome in Four Children

Department of Child Neuropsychiatry, University of Turin, Turin, Italy, p.vigliano{at}infinito.it

Carlotta Canavese, MD

Department of Child Neuropsychiatry, University of Turin, Turin, Italy

Barbara Bobba, MD

Department of Child Neuropsychiatry, University of Turin, Turin, Italy

Lorenzo Genitori, MD

Department of Neurosurgery, Children's Hospital of Turin, Turn, Italy

Francesco Papalia, MD

Department of Radiology, Children's Hospital of Turin, Turin, Italy

Sergio Padovan

Department of Genetics, Biology and Biochemistry, University of Turin, and CNR-CIOS, Immunogenetic and Experimental Oncologic Center, Turin, Italy

Marco Forni, MD

In the literature, several malformations of cortical development have been described as additional lesions in tuberous sclerosis complex. Among these lesions, a very large focal cortical dysplasia has peculiar magnetic resonance imaging features: a signal abnormality that extends radially inward toward the lateral ventricle from the pachygyric cortical surface plus a homogeneous clinical picture. Affected patients have early-onset drug-resistant epilepsy and severe developmental delay. We describe the clinical, genetic, neurophysiologic, and neuroradiologic characteristics of four patients affected by tuberous sclerosis and this type of cortical dysplasia: these patients are of special interest because they have been operated on for their dysplastic lesions. Total control of seizures has been achieved in the three children who underwent a complete lesionectomy. This result cannot be permanent, however, because of the presence of other cortical tubers which could become epileptogenic. All things considered, our choice was to give these children at least temporary relief from severe epilepsy and possibly support for developmental progression. (J Child Neurol 2002;17:752—758).

Journal of Child Neurology, Vol. 17, No. 10, 752-758 (2002)
DOI: 10.1177/08830738020170101601


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