Hypothalamic Hamartomas: Two Cases

Tushar Kalekar

doi:10.4236/ojmi.2015.51003

Abstract

This is a MRI study of two patients presented with history of gelastic seizures since many years ago. Plain and post intravenous gadolinium multiplanar MRI imaging of the brain is performed. It showed well-defined non-enhancing mass lesions in the region of hypothalamus and tuber cinereum. It showed signal intensity similar to the gray matter and imaging diagnosis of hypothalamic hamartoma is made.

Highlights

Hypothalamic hamartomas, rare lesions of the floor of the third ventricle, are manifested with a spectrum of clinical symptoms that include precocious puberty, gelastic seizures, and behavioral disorders
Multiplanar multiecho magnetic resonance imaging (MRI) study was performed on 1.5 T GE scanner with high resolution, thin coronal and sagittal images were performed in the region of thalamus and this was followed by post intravenous gadolinium study
A hamartoma of the tuber cinereum or hypothalamus is a congenital malformation consisting of neuronal tissue in an ectopic location

Summary

Introduction

Hypothalamic hamartomas, rare lesions of the floor of the third ventricle, are manifested with a spectrum of clinical symptoms that include precocious puberty, gelastic seizures, and behavioral disorders. Hypothalamic hamartomas represent neoplastic proliferation of well-organized brain tissue similar to heteropias [1]. Classical location and magnetic resonance imaging (MRI) features strongly favor the diagnosis. As these lesions are usually small in size and isointense signal as compared to adjacent gray matter of hypothalamus, they can be missed on routine MRI imaging. High resolution MRI sequences with special attention in the region of hypothalamus should be performed whenever scanning patients with these types of seizures or associated precocious puberty. MRI is the most important modality for the diagnosis of this entity

Case Reports

Discussion