Hypothalamic Hamartoma (HH) as a Bystander of Epileptogenicity - Case Study with StereoEEG and Neurostimulation
Abstract number :
1.419
Submission category :
18. Case Studies
Year :
2023
Submission ID :
229
Source :
www.aesnet.org
Presentation date :
12/2/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Carolina Cuello-Oderiz, MD – University of Pittsburgh Medical Center
Joanna Fong-isariyawongse, MD – University of Pittsburgh Medical Center; Thandar Aung, MD – University of Pittsburgh Medical Center; Niravkumar Barot, MD – Beth Israel Deaconess Medical Center; Anto Bagić, MD, PhD, FAES, FACNS – University of Pittsburgh Medical Center; Jorge Gonzalez-Martinez, MD, PhD – University of Pittsburgh Medical Center; Alexandra Urban, MD, FAAN, FAES – University of Pittsburgh Medical Center
Rationale:
The role of HH in epileptogenicity remains controversial. Genetic abnormalities are in the Sonic hedgehog pathway and most of the mutations are sporadic (1). Different types of interictal/ictal scalp patterns have been described (2). Patients usually undergo HH surgical interventions with variable outcomes. Long-standing history of epilepsy in patients with HH may result in independent epileptogenesis (3).
Methods:
Case Report
Results:
We report a 33-year-old right-handed male with drug resistant epilepsy (DRE) since infancy, developmental delay and HH status post two prior surgeries during pediatric age: 1) a subtotal resection of a HH and the mid-corpus callosum; 2) a right sided HH laser ablation. He used to have gelastic/atonic that resolved and currently having uncontrolled tonic seizures. Recently, patient developed psychotic symptoms and encephalopathy without EEG changes. SEEG showed multifocal interictal discharges. Seizures captured were poorly localized with diffuse attenuation. Seizures and epileptiform discharges did not involve the residual HH. Patient underwent Responsive Neurostimulation Implantation (RNS) in bilateral anterior thalamus (ANT) to target limbic circuit of Papez (4). Post-implantation seizures were captured from both leads, left preponderant.
Conclusions:
Our case demonstrates using SEEG recordings that HH can be a bystander of epileptogenicity; specifically in long-standing epilepsy and absence of gelastic seizures. Genetic abnormalities may predispose to multifocal epilepsy. Encephalopathy, depression, and psychosis have been described in patients with HH and chronic epilepsy further supporting a diffuse process (1). Neurostimulation may be considered as a first line of surgical options in this highly selective population.
References:
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Mullatti N, Selway R, Nashef L, Elwes R, Honavar M, Chandler C, Morris R, Jarosz J, Buchanan C, Polkey C. The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma. Epilepsia. 2003;44(10):1310-9.
Valentin A, Lazaro M, Mullatti N, Cervantes S, Malik I, Selway RP, Alarcón G. Cingulate epileptogenesis in hypothalamic hamartoma. Epilepsia. 2011;52(5):e35-9.
Roa JA, Abramova M, Fields M, Vega-Talbott M, Yoo J, Marcuse L, Wolf S, McGoldrick P, Ghatan S, Panov F. Responsive Neurostimulation of the Thalamus for the Treatment of Refractory Epilepsy. Front Hum Neurosci. 2022 15;16:926337.
Funding: NA
Case Studies