Advertisement

Welcome to the new AJNR, Updated Hall of Fame, and more. Read the full announcements.

AJNR is seeking candidates for the position of Associate Section Editor, AJNR Case Collection. Read the full announcement.

 

Case of the Week

Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada

Sign up to receive an email alert when a new Case of the Week is posted.

Submit a Case Previous Cases ASPNR Pediatric Cases

November 1, 2010 (Supplemental)

Hypothalamic Hamartoma

  • Hypothalamic hamartomas are developmental malformations consisting of tumor like masses located in the tuber cinereum of the hypothalamus.
  • Clinical presentation varies from precocious puberty, diabetes insipidus to diencephalic syndrome or epilepsy. Gelastic seizures (laughing fits) are frequently associated with hamartomas of the tuber cinereum.
  • Key Diagnostic Features: MRI demonstrates well-defined hypothalamic lesions isointense on T1WI, iso- to hyperintense on T2WI, with no contrast enhancement. Single voxel spectroscopy of the hamartomas shows reduced NAA/Cr ratio, increased mI/Cr and Cho/Cr ratios when compared with controls. Increased T2W signal intensity and higher mI/Cr on proton MRS correlates with tumors of increased glial content on pathology.
  • DDx: Glioma, Craniopharyngioma
  • Treatment: Surgical excision

Suggested Reading

Saleem SN, Said AHM, Lee DH. Lesions of the Hypothalamus: MR Imaging Diagnostic Features. RadioGraphics 2007;27:1087-108.

Amstutz DR, Coons SW, Kerrigan JF, et al. Hypothalamic Hamartomas: Correlation of MR Imaging and Spectroscopic Findings with Tumor Glial Content. AJNR Am J Neuroradiol 2006;27:794-8.

Current Issue

Advertisement

Case Collections

Case of the Week Archive
Case of the Month Archive
Advertisement