Hypothalamic hamartoma

Case contributed by Luke Berger

Presentation

Childhood gelastic seizures, and developmental delay, in the setting of Pallister-Hall syndrome.

Patient Data

Age: 7 years
Gender: Female
MRI

Large, lobulated, non-enhancing mass arising from the tuber cinereum, predominantly isointense to gray matter, but with a faint thin curvilinear layer of T2 hypointense/T1 hyperintense signal, consistent with a layer of white matter. The mass follows normal brain tissue signal on all sequences, including DWI and perfusion imaging. 

Case Discussion

This case represents findings of a hypothalamic hamartoma with evidence of gray and white matter differentiation in the setting of Pallister-Hall syndrome.

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