Clinical Manifestations and MR Imaging Features In Hypothalamic Hamartomas

Hypothalamic hamartomas (HH) are rare non-neoplastic congenital malformations. The aims of this study are to demonstrate the brain MR findings and clinical manifestations in children with HH and the literature is reviewed. We have evaluated 6 children with HH diagnosed by brain MR imaging. We analyzed age at seizure onset, seizure types, neuropsychological assessment, and the presence of precocious puberty, electroencephalogram and MR findings. Among 6 HH patients, 2 manifested precocious puberty, 2 had experienced seizures, and 1 presented with both precocious puberty and seizures, and 1 presented with hyperactivity. MR imaging showed hypothalamic mass that was isointense to brain parenchyma on T1- weighted images and isointense-or hyperintense on T2-weighted images, without contrast enhancement. The size of the lesions does not change in the course of follow-up. Typical clinical presentation, peculiar location and MR imaging features strongly favour the HH diagnosis. Accurate diagnosis and classification is important for the selection of treatment modality.