Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
Book Review
Brief Report
Case Report
Case Series
Commentary
Editorial
Erratum
Guest Editorial
Images
Images in Neurology
Images in Neuroscience
Images in Neurosciences
Letter to Editor
Letter to the Editor
Letters to Editor
Letters to the Editor
Media and News
None
Notice of Retraction
Obituary
Original Article
Point of View
Position Paper
Review Article
Short Communication
Technical Note
Techniques in Neurosurgery
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
Book Review
Brief Report
Case Report
Case Series
Commentary
Editorial
Erratum
Guest Editorial
Images
Images in Neurology
Images in Neuroscience
Images in Neurosciences
Letter to Editor
Letter to the Editor
Letters to Editor
Letters to the Editor
Media and News
None
Notice of Retraction
Obituary
Original Article
Point of View
Position Paper
Review Article
Short Communication
Technical Note
Techniques in Neurosurgery
View/Download PDF

Translate this page into:

Letter to the Editor
11 (
4
); 675-675
doi:
10.1055/s-0040-1715080

Gelastic Seizures and Hypothalamic Hamartoma

Serviço de Neurologia, Universidade Federal do Paraná, Hospital de Clínicas, Curitiba, PR, Brasil
InNeuro, Hospital das Nações, Curitiba, PR, Brasil
Departamento de Medicina, Universidade Positivo, Curitiba, PR, Brasil
Address for correspondence Otto Jesús Hernandez Fustes, MD, MSC Hospital de Clínicas da Universidade Federal do Paraná Rua General Carneiro 181, 80060-900, Curitiba/PR, Brasil otto.fustes@hc.ufpr.br
Licence
This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
Disclaimer:
This article was originally published by Thieme Medical and Scientific Publishers Pvt. Ltd. and was migrated to Scientific Scholar after the change of Publisher; therefore Scientific Scholar has no control over the quality or content of this article.

In 1877, Trousseau first described a laughter crisis in a young man. In 1957, Daly and Mulder proposed the term gelastic seizures (GS), from the Greek word gelos meaning “laughter,” emphasizing this condition’s the main characteristic.1 2

The accepted diagnostic criteria for GS include recurrent stereotyped fits of laughter, the absence of precipitating external factors, laughter inappropriate to context, laughter concomitant with other epileptic clinical manifestations, and ictal or interictal epileptiform electroencephalographic (EEG) changes.3

GS are frequently associated with hypothalamic hamartomas (HHs), rare non-neoplastic congenital malformations composed of ectopic neurons and glial cells. HH has an estimated prevalence of 1 in 50,000 to 100,000, more frequently in males. HH occurs concurrently with precocious puberty in 63% of patients, epileptic seizures in 61%, and both in 25%.4 5

We present a case of GS associated with an HH in a 19-year-old male with retarded psychomotor development. At the age of 4 years, he presented with short-duration disconnection crises (focal impaired-awareness seizures) followed by generalized tonic–clonic seizures that were refractory to multiple antiepileptic drugs (AEDs). The seizures morphed, at 10 years, into focal seizures of unmotivated, uncontrollable, and explosive laughter lasting for seconds without precipitating factors and occurring several times a day. Examination revealed slow, monotonous, and monosyllabic language without other peculiarities. EEG showed acute paroxysmal left temporoparietal and right temporal interictal epileptiform waves and right temporal ictal epileptiform activity. A computed tomography (CT) scan of the brain was normal. Magnetic resonance imaging (MRI) of the brain revealed a right hypothalamic mass with spectroscopy compatible with an HH. There was partial seizure control, without the occurrence of tonic–clonic seizures, and a 70% decrease in the frequency of focal and GS with the use of carbamazepine 1200 mg/day.

HH is associated with endocrine and neurological manifestations, commonly occurring in childhood with generalized early-onset dysperceptive and tonic seizures that are resistant to AEDs.5 The onset of seizures in adolescence, as in our case, is infrequent; however, the refractory behavior of GC was similar to previous reports. The EEG supported the diagnosis of epilepsy and was hemispherically related to the lesion on brain MRI. We deduced that an HH had caused all crises in the patient, including the intellectual deficit and developmental delay of school skills.

Our patient emphasizes the need for imaging, preferably brain MRIs, in patients with epileptic seizures due to the 14-year delay in identifying the etiology of epilepsy.

Conflict of Interest

None declared.

References

  1. , , . Clinique Medicale de L’Hotel-Dieu de Paris. 187789-155.
    [Google Scholar]
  2. , , . Gelastic epilepsy. Neurology. 1957;7(3):189-192.
    [Google Scholar]
  3. , , . Epileptic (gelastic) laughter. Epilepsia. 1971;12(1):63-76.
    [Google Scholar]
  4. , , , et al . Hypothalamic hamartomas in adulthood: clinical spectrum and treatment outcome-a unicenter experience. Brain Behav. 2019;9(11):e01412.
    [CrossRef] [Google Scholar]
  5. , , , et al . Hypothalamic hamartomas: seven cases and review of the literature. Epilepsy Behav. 2003;4(3):246-258.
    [Google Scholar]

Fulltext Views
48

PDF downloads
1
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections