Atypical clinical schizencephaly

Article type: Research Article

Authors: Arantes, Mavilde | Perdigao, Sandra | Pereira, Jorge Resende | Costa, Manuela

Affiliations: Department of Neuroradiology, Hospital Pedro Hispano, Matosinhos, Portugal | Department of Neurology, Hospital Pedro Hispano, Matosinhos, Portugal

Note: [] Correspondence: Dr. Mavilde Arantes, M.D., Department of Neuroradiology, Hospital Pedro Hispano, 4464-513, Matosinhos, Portugal. Tel.: +351 229391000; Fax: +351 229391654; E-mail: [email protected]

Abstract: Schizencephaly is an uncommon disorder of cell migration characterized by communication clefts between ventricular system and subarachnoid space. The clefts may be unilateral or bilateral and may be closed or separated. Patients with bilateral clefts typically present with severe mental deficits, motor anomalies and seizures. We report a 16-year-old girl whose cranial magnetic resonance imaging revealed bilateral open-lip schizencephaly, but with an atypical clinical course. She had minimally recognizable neurological deficits and epilepsy for last one year. Such mild clinical phenotypes were not proportional to the magnetic resonance imaging of the cerebral malformation.

Keywords: Migration disorder, schizencephaly, magnetic resonance image

Journal: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 343-345, 2007