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Hypothalamic hamartoma

Case contributed by Mehmet Yağtu
Diagnosis probable

Presentation

Ongoing headache for a month.

Patient Data

Age: 65 years
Gender: Female

Brain

mri

There is an oval-shaped mass which is lengthening craniocaudal in the hypothalamic region (CC:21mm, AP:11mm TS: 10 mm). The mass has minimal lower intensity on T2W images according to CSF and also isointense to cortex on T1W images, which has no diffusion restricted on DWI and ADC images. The mass is compressing the inferior aspect of third ventricular and also optic chiasma that best can be seen on sagittal and coronal images. 

There is also a subacute-chronic period intra-axial hematoma in the right lenticular region.

Case Discussion

Also, findings or appearances of this mass are typical for hypothalamic hamartoma. Without contrasted images make diagnosis less favorable. The differential diagnosis is hypothalamic-chiasmatic glioma.

Lesions can cause gelastic seizures, visual problems, early onset of puberty and behavioral problems. Gelastic seizures are typically of short duration (2-30 seconds) and characterized by uncontrollable laughter, without impairment of consciousness, which contrasts with frontotemporal gelastic seizures which usually are longer lasting and can result in loss of consciousness. Very rarely children with hypothalamic hamartomas can enter into 'status gelasticus'. (gelastic seizure now is more meaningful for me, after I watched JOKER movie.)

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