Hypothalamic hamartoma

Case contributed by Hala M Qasrawi
Diagnosis almost certain

Presentation

Developmental delay and seizures.

Patient Data

Age: 2 years
Gender: Female
mri

There is a 1.2 cm mass lesion in the hypothalamic region arising from the tuber cinereum, related to the left optic tract, and bulging into the third ventricle.

It appears isointense on T1- and T2-weighted images without post-contrast enhancement.

There is minimal hyperintense FLAIR signal in the periventricular white matter.

There are multiple non-specific tiny hyperintense FLAIR foci in the subcortical cerebral white matter.

Case Discussion

The MR appearance and clinical picture are highly suggestive of a hypothalamic hamartoma, arising from the tuber cinereum; usually patients present with gelastic seizures and precocious puberty.

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