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Leukoencephalopathy With Bilateral Anterior Temporal Lobe Cysts: A Further Case of This New Entity

Division of Child Neurology and Psychiatry Instituto Ricera Cura Carattere Scientifico (IRCCS) Stella Maris and University of Pisa Pisa, Italy

M. Cristina Bianchi, MD

Unit of Neuroradiology S. Chiara Hospital Pisa, Italy

Michela Tosetti, PhD

Neuroimaging Laboratory IRCCS Stella Maris Pisa, Italy

Andrea Guzzetta, MD

Division of Child Neurology and Psychiatry IRCCS Stella Maris and University of Pisa Pisa, Italy

Giovanni Cioni, MD

Division of Child Neurology and Psychiatry IRCCS Stella Maris and University of Pisa Pisa, Italy, cioni{at}inpe.unipi.it

The introduction of magnetic resonance imaging (MRI) and magnetic resonance spectroscopy into clinical practice has permitted advances in the definition and categorization of unknown leukodystrophies in children. We report a new type of leukodystrophy, defined by particular MRI and clinical findings, in a child with uneventful pre- and perinatal histories, spasticity, severe mental impairment with absence of language, and deafness. Brain MRI showed lobar white-matter signal abnormalities with extensive cysts in the anterior temporal lobe. The results of all metabolic screening tests, including the specific investigations for leukodystrophy, have been negative. Six years of clinical and MRI—magnetic resonance spectroscopy patient follow-up indicate a nonprogressive clinical and magnetic resonance picture. Owing to the striking similarities with the previous eight patients reported, this additional case supports the identification of a new leukodystrophy. (J Child Neurol 2002;17:773—776).

Journal of Child Neurology, Vol. 17, No. 10, 773-776 (2002)
DOI: 10.1177/08830738020170101803


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