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Association of Morphological Characteristics with Precocious Puberty and/or Gelastic Seizures in Hypothalamic Hamartoma

Clinical Research, Internal Medicine, and Endocrinology (H.J.), Lilly Deutschland GmbH, Bad Homburg and University Children’s Hospital, Giessen, Germany; Department of Neuroradiology (E.N.P.), University Hospital Hamburg-Eppendorf, Germany; University Children’s Hospital (B.P.H.), Essen, Germany; University Children’s Hospital (C.-J.P.), Kiel, Germany; and Departments of Obstetrics and Pediatrics, Perinatal Infectiology (O.D.), Hanover Medical School, Hanover, Germany

Address all correspondence and requests for reprints to: Heike Jung, M.D., Clinical Research, Internal Medicine, Endocrinology, Lilly Deutschland GmbH, Saalburgstrassee 153, 61350 Bad Homburg, Germany. E-mail: Jung_Heike{at}Lilly.com.

The pathogenesis of central precocious puberty (PP) and/or gelastic seizures due to a hypothalamic hamartoma (HH) is still under debate. We evaluated the association of clinical symptoms with morphology and localization of the HH in 34 patients.

The majority (86.4%) of HHs in patients with isolated PP (n = 22; 68.2% females) revealed a parahypothalamic position without affecting the third ventricle (91%). Half of them were pedunculated, and 40.9% showed a diameter less than 10 mm. In contrast, 11 of 12 patients with seizures, eight of whom were male, presented with a sessile intrahypothalamic hamartoma, 10 of which distorted the third ventricle. Logistic regression analysis revealed an increased relative risk (RR) for epilepsy in males (RR, 4.3; 95% confidence interval, 0.96–19). However, combination of the risk factor gender with intrahypothalamic position (RR, 19; 1.3–285) and distortion of the third ventricle (RR, 10; 0.6–164) reduced the risk associated with male gender to 1.1.

The position of a HH and involvement of the third ventricle are likely to be more predictive for clinical characteristics than size and shape. Male gender was associated with an intrahypothalamic HH and epilepsy, suggesting a sexually dimorphic developmental pattern of this heterotopic mass.

Abbreviations: BA, Bone age; CI, confidence interval; CRF, clinical report form; CT, computerized tomography; E₂, estradiol; HH, hypothalamic hamartoma; MRI, magnetic resonance images; PP, precocious puberty; RR, relative risk; SDS, SD score.