Diagnostic criteria for gelastic seizures with hypothalamic hamartoma

	Mandatory	Alerts	Exclusionary
Seizures	Gelastic seizures with mechanical, mirthless laughter, inappropriate to context	Seizure frequency less than daily
EEG		Interictal: Generalized or focal background slowing (excluding immediate postictal period) Ictal: Gelastic seizures may lack ictal EEG correlate
Age at onset		Onset >5 years of age
Development at onset		Clear developmental delay at seizure onset
Neurologic examination		Focal neurologic findings (other than Todd paresis) or generalized hypotonia
Imaging	Hypothalamic hamartoma (may require thin slices through the hypothalamic region to confirm)
Course of illness	Drug-resistant epilepsy	Lack of behavioral problems including aggression, impulsivity, and hyperactivity
Is MRI or ictal EEG required for diagnosis? An MRI is required for diagnosis. An ictal EEG is not required for diagnosis. Furthermore, gelastic seizures may lack ictal correlate on EEG.
Syndrome without laboratory confirmation: In resource-limited regions, HH-GS cannot be diagnosed in the absence of an MRI, as gelastic seizures may arise from other brain regions

EEG: electroencephalography; MRI: magnetic resonance imaging; HH-GS: hypothalamic hamartoma-gelastic seizures.

From: Zuberi SM, Wirrell E, Yozawitz E, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63:1349. Copyright © 2022 The Authors. Available at: https://onlinelibrary.wiley.com/doi/10.1111/epi.17239 (Accessed on November 14, 2022). Reproduced under the terms of the Creative Commons Attribution License 4.0.

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Diagnostic criteria for gelastic seizures with hypothalamic hamartoma