E-ISSN 2636-865X
Case report
Clinical Manifestations and MR Imaging Features In Hypothalamic Hamartomas
1 Başkent Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, Adana, Türkiye  
2 Başkent Üniversitesi Tıp Fakültesi, Pediatri Anabilim Dalı, Adana, Türkiye  
Neurol Sci Neurophysiol 2009; 26: 335-342

Key Words: Hypothalamic hamartoma, MR imaging, gelastic seizure, precocious puberty

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.


How to cite this article:
Ö. ALKAN, O. KIZILKILIÇ, T. KARSANTIÖZÜ, T. YILDIRIM, N. TOKMAK : Clinical Manifestations and Mr Imaging Features In Hypothalamic Hamartomas. J Neurol Sci [Turk] 2009;26:335-342


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