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Hypothalamic Hamartoma

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Atlas of Sellar and Parasellar Lesions

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Abstract

Hypothalamic hamartomas are congenital lesions that present in the region of the floor of the third ventricle, hypothalamus, tuber cinereum, mammillary bodies, and infundibulum.

The mean age at presentation is 10 years.

Patients typically present with a classic triad:

  • Precocious puberty

  • Gelastic seizures (uncontrollable laughter)

  • Developmental delay

Central precocious puberty occurs in 30–45 % of patients and is often associated with obesity.

Serum levels of FSH, LH, estradiol, and testosterone can be elevated.

Gelastic seizures often progress to more complex seizure disorders and finally to intractable epilepsy.

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Author information

Authors and Affiliations

  1. Department of Neurological Surgery, Keck School of Medicine, University of Southern California, 1200 N State Street, #3300, Los Angeles, CA, 90033, USA

    Gabriel Zada MD, MS

  2. Department of Pathology (Neuropathology), University of Virginia School of Medicine, 1215 Lee Street, Room 3060–HEP, Charlottesville, VA, 22908, USA

    M. Beatriz S. Lopes MD, PhD

  3. Department of Radiology, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA, 02115, USA

    Srinivasan Mukundan Jr. PhD, MD

  4. Department of Neurosurgery, Brigham and Women’s Hospital, 15 Francis Street, Boston, MA, 02115, USA

    Edward Laws Jr. MD

Authors
  1. Gabriel Zada MD, MS
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  2. M. Beatriz S. Lopes MD, PhD
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  3. Srinivasan Mukundan Jr. PhD, MD
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  4. Edward Laws Jr. MD
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Corresponding author

Correspondence to Gabriel Zada MD, MS .

Editor information

Editors and Affiliations

  1. Department of Neurosurgery, University of Southern California, LOS ANGELES, California, USA

    Gabriel Zada

  2. Department of Pathology, University of Virginia, CHARLOTTESVILLE, Virginia, USA

    M. Beatriz S. Lopes

  3. Department of Radiology, Brigham and Women's Hospital, BOSTON, Massachusetts, USA

    Srinivasan Mukundan Jr.

  4. Department of Neurosurgery, Brigham and Women’s Hospital, BOSTON, Massachusetts, USA

    Edward R. Laws Jr.

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Zada, G., Lopes, M.B.S., Mukundan, S., Laws, E. (2016). Hypothalamic Hamartoma. In: Zada, G., Lopes, M., Mukundan Jr., S., Laws Jr., E. (eds) Atlas of Sellar and Parasellar Lesions. Springer, Cham. https://doi.org/10.1007/978-3-319-22855-6_39

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  • DOI: https://doi.org/10.1007/978-3-319-22855-6_39

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-22854-9

  • Online ISBN: 978-3-319-22855-6

  • eBook Packages: MedicineMedicine (R0)

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