Three pediatric cases of hypothalamic hamartoma presenting different clinical courses

Endo Megumi, Takahata Asuka, Takeda Kenta, Hashimoto Kahoko, Kaneshi Yosuke, Suzuki Yasuto, Nakanishi Masanori, Nakamura Akie, Egawa Kiyoshi, Shirozu Hiroshi

doi:10.11251/ojjscn.54.343

<p> Hypothalamic hamartoma (HH) pertains to non-neoplastic, heterotopic nodules of the hypothalamus. They have been well known for their characteristic symptoms, which include precocious puberty (PP) and epileptic seizures, such as gelastic seizures (GSs), and mental developmental disorders. Thus, we need to choose the treatment for each case. We report three cases of HH, wherein the patients were treated with different therapeutic strategies and showed good progress. Case 1 was a 4-year-old boy who presented with GSs. He was treated with stereotactic radiofrequency thermocoagulation 4 months after diagnosis, and complete remission of seizures was achieved. The patient presented with PP but no intellectual disability, and was treated with a gonadotropin-releasing hormone (GnRH) analog. Case 2 was a 3-year-old girl who presented with PP but no epileptic seizures. After treatment with a GnRH analog, her progression of puberty was arrested. Case 3 was a 9-year-old girl who was suspected to have PP, but did not fulfill the relevant criterion on GnRH stimulation test. Although watchful waiting without therapeutic intervention was done, the patient had no seizures and intellectual disability, and reached the target height range. It is important to classify HH based on the magnetic resonance imaging findings because it might be helpful in predicting the clinical courses, and to select individualized treatment for each case.</p>

Three pediatric cases of hypothalamic hamartoma presenting different clinical courses

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