A NEW LEUKOENCEPHALOPATHY WITH BILATERAL ANTERIOR TEMPORAL-LOBE CYSTS

We describe an identical syndrome of cystic leukoencephalopathy in thr ee Turkish children, including two siblings. The neurological findings were noted within the first months of life and include severe intelle ctual impairment, motor retardation, and spasticity. Magnetic resonanc e imaging of the brain showed extensive cysts within the anterior temp oral lobes, ventricular enlargement and white matter disease. The sign al intensities of the cysts' content were identical to those of the ce rebrospinal fluid. The patients' screening for known inborn errors of metabolism, especially those characterised by white matter involvement , did not reveal any abnormality. The clinical picture and the magneti c resonance imaging characteristics are unique diagnostic features of a new disease entity so far not described in the literature.