Epilepsy syndromes associated with hypothalamic hamartomas

T1 weighted MRI of HH in all cases. (A) Patient 1, coronal view. (B) Patient 2, sagittal view. (C) Patient 3, sagittal view. (D) Patient 4, coronal view. (E) Patient 5, coronal view. (F) Patient 6, sagittal view. Arrows point to HH, in each case.

Ictal EEG during a tonic seizure (patient 6) showing a high-amplitude, diffuse slow (delta) wave, followed by diffuse attenuation of the background activity, and superimposed fast activity (beta) more prominently noted in the fronto-temporal electrodes. Referential montage, with average reference. The arrow points to the clinical and EEG onset.

Ictal EEG during a complex partial seizure (patient 1) showing sharply contoured (theta) activity in the left temporal and subtemporal electrodes. Referential montage, with average reference. The arrow points to the clinical and EEG onset.

Interictal and ictal SPECT obtained during a gelastic seizure evolving to a tonic seizure (patient 6). Note ictal increased flow in the HH (white arrow), left frontal lobe (yellow arrow). Increased flow was also seen in the right cerebellum (not shown). MRI to provide anatomical correlation: sagital T1 weighted image, axial FLAIR (arrows point to HH).