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Neuropediatrics
DOI: 10.1055/a-2561-8410
Videos and Images in Neuropediatrics

Combined Gelastic and Dacrystic Seizures in a Child with Hypothalamic Hamartoma

Lorenz Kronast    *
1   LMU University Hospital Munich, Ludwig-Maximilians-Universität in Munich, München, Germany
,
Sarah Schweikart   *
1   LMU University Hospital Munich, Ludwig-Maximilians-Universität in Munich, München, Germany
,
Marcus Jakob
2   Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, University Hospital of Regensburg, Regensburg, Germany
,
Friederike Studt
3   Center for Pediatric Neurology, Neurorehabilitation, and Epileptology, Schoen-Clinic, Vogtareuth, Germany
,
Gerhard Kluger
3   Center for Pediatric Neurology, Neurorehabilitation, and Epileptology, Schoen-Clinic, Vogtareuth, Germany
4   Research Institute “Rehabilitation, Transition and Palliation” PMU Salzburg, Austria
,
Till Hartlieb
3   Center for Pediatric Neurology, Neurorehabilitation, and Epileptology, Schoen-Clinic, Vogtareuth, Germany
4   Research Institute “Rehabilitation, Transition and Palliation” PMU Salzburg, Austria
› Author Affiliations Funding None.
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Hypothalamic hamartomas (HH) are congenital malformations developing during the fetal period. The core symptom in patients with HH is frequent stereotypical gelastic seizures (sz), in addition to other focal epileptic sz, developmental delay, cognitive decline, and endocrinologic disorders.[1] [2] Stereo-EEG studies showed that HH causes epilepsy, although the exact pathophysiology remains unclear.[3] Dacrystic sz are rare (0.06–0.53% of long-term monitoring patients)[4] and occur in combination with gelastic sz in patients with HH. According to the literature, patients after radiofrequency or laser coagulation of HH show the highest rates of seizure freedom and fewer complications compared with microsurgical resection.[5]

We report on a 34-month-old boy with combined gelastic and dacrystic sz (4–5 daily, 20–30 seconds) started at the age of 12 months, with uncontrollable, stereotyped laughter followed by crying out of situational context (see [Video 1]).


Quality:
Video 1 Stereotypical episode of inadequate laughing followed by crying (combined gelastic and dacrystic seizure) with MRI findings at the end of the video.

Developmental delay in motor function, behavior, and speech was evident, compared with the family, he showed increased longitudinal growth as a likely symptom of endocrinological malfunction. Magnetic resonance imaging confirmed the diagnosis of HH (see [Video 1]) with right-sided, sessile HH type 4 according to Delalande and Fohlen. Genetic testing on GLI3-mutation (Pallister–Hall syndrome) and NF1 was negative, no further information on therapy or outcome is currently available.

* Both authors contributed equally.




Publication History

Received: 16 November 2024

Accepted: 17 March 2025

Accepted Manuscript online:
19 March 2025

Article published online:
07 April 2025

© 2025. Thieme. All rights reserved.

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