Hypothalamic Hamartoma, Gray Matter Heterotopia, and Polymicrogyria in a Boy: Case Report and Literature Review

World Neurosurg. 2020 Oct:142:396-400. doi: 10.1016/j.wneu.2020.07.098. Epub 2020 Jul 22.

Abstract

Background: Hypothalamic hamartomas (HHs) are rare, and it is even rarer when combined with gray matter heterotopia (GMH) and polymicrogyria (PMG).

Case description: A 5-year-old boy with HH, GMH, and PMG was retrospectively evaluated. The clinical data, including the symptoms, examinations, diagnosis, and treatment, were collected. The patient had a chief complaint of gelastic seizures and intellectual deficiency. Brain magnetic resonance imaging showed HH, paraventricular nodular heterotopia, and PMG. Video electroencephalographs were normal. The patient underwent resection of the HH via transcallosal transseptal interforniceal approach. Seizures disappeared immediately after complete resection of HH, and the intellectual development improved.

Conclusions: In this extremely rare case, resection of the HH eliminated the symptoms. Nonetheless, we still need to be cautious about the possible epilepsy that may be caused by GMH and PMG.

Keywords: Epilepsy; Gray matter heterotopia; Hypothalamic hamartoma; Polymicrogyria.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Gray Matter / pathology*
  • Hamartoma / pathology*
  • Hamartoma / surgery
  • Humans
  • Hypothalamic Diseases / pathology*
  • Hypothalamic Diseases / surgery
  • Intellectual Disability / etiology
  • Male
  • Neurosurgical Procedures / methods
  • Polymicrogyria / pathology*
  • Seizures / etiology

Supplementary concepts

  • Hypothalamic hamartomas