Endoscopic Treatment of Hypothalamic Hamartomas

Abstract

Hypothalamic hamartoma (HH) is a benign indolent lesion despite the presentation of refractory epilepsy. Behavioral disturbances and endocrine problems are additional critical symptoms that arise along with HHs. Due to its nature of generating epileptiform discharge and spreading to cortical region, various management strategies have been proposed and combined. Surgical approaches with open craniotomy or endoscopy, stereotactic approaches with radiosurgery and gamma knife surgery or radiofrequency thermos-coagulation, and laser ablation have been introduced. Topographical dimension and the surgeon’s preference are key factors for treatment modalities. Endoscopic disconnection has been one of the most favorable options performed in treating HHs. Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications.

Hypothalamic hamartomas and Gelastic seizures. Chilean experience in a pediatric epilepsy surgery program during 13 years - Review of the literature

“…This rare non-neoplastic abnormal mixture of neuronal and glial tissue of the inferior hypothalamus has been postulated to be derived from the mammilo-thalami-cingulate tract, from which HH is networking other brain areas associated with GS or the pathway from the HH to the brainstem and cerebellum [1,11,12].…”

“…Using a recent electroencephalography-functional MRI (EEG-fMRI) studies revealed that the ipsilateral hypothalamus is associated with activation of brainstem tegmentum and contralateral cerebellum; in contrast the cunei, bilateral thalami, caudate nuclei, hippoccampi, paracentral gyri and default mode network (precunei and inferior lateral parietal lobes) are deactivated [1,13]. This activated-deactivated networks result in epileptic discharges through the premotor and frontal opercular areas (voluntary system), which are located on the same side of the broader attachment [1,14] of the HH on the hypothalamus, as well as the ipsilateral thalamus is activated, mainly V A nucleus with the cingulate gyri and the limbic circuit is activated too leading to DM activation (dorso medial thalamic nucleus) with the subsequent "on" activity of orbitofrontal cortex, mesial part of the premotor area and parietal lobe [15] (Figure 7).…”

“…Gelastic seizures (GS) have been well known as part of the hypothalamic hamartoma (HH) syndrome from sessile HH, while endocrinological disturbances [1][2][3] and or visual impairment may arise from pedunculated HH [1,4,5]. Compulsive burst of laughter is a typical and classical feature of GS [1,6,7]. Drop attacks and other secondary generalized epilepsy symptoms usually develop as the patient ages.…”

“…Drop attacks and other secondary generalized epilepsy symptoms usually develop as the patient ages. Other types of seizure, cognitive deterioration and behavioral problems follow and frequently develop late in the first decade of life [1,8,9]. It has been well known that epileptic discharge originates from the hamartoma itself and propagates to subcortical and cortical areas.…”

“…Partial and generalized seizures result along with cognitive/ behavioral problems, including memory deficit and mental retardation [1,7,10].…”