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Gelastic seizures in clusters in a case of West syndrome after perinatal hypothalamic hemorrhage

M Kito1, K Yabuta, S Kato

  • 1Department of Pediatrics, Minami Seikyo Hospital, Nagoya, Japan.

Journal of Perinatal Medicine
|September 22, 2001
PubMed

Insights

West syndrome in an infant presented with gelastic seizures, characterized by frequent smiling episodes. Early diagnosis using electroencephalogram (EEG) and neuroimaging is crucial for timely treatment.

Area of Science:

  • Neurology
  • Pediatrics
  • Epileptology

Background:

  • West syndrome is a severe form of epilepsy typically presenting in infancy.
  • Gelastic seizures, characterized by sudden, brief episodes of laughter or smiling, can be a rare manifestation of certain epilepsy syndromes.
  • Hypothalamic dysfunction has been implicated in some cases of epilepsy with behavioral changes.

Observation:

  • A 4-month-old infant experienced gelastic seizures manifesting as progressively intensifying smile-like episodes.
  • The infant displayed delayed development and a history suggestive of perinatal asphyxia.
  • Simultaneous video/electroencephalogram (EEG) monitoring captured the seizure evolution from smile-like episodes to generalized spasms.

Findings:

  • Positron emission tomography (PET) revealed hypoperfusion in the bilateral hypothalamus.
  • Interictal EEG demonstrated hypsarrhythmia, a hallmark of West syndrome.
  • Ictal EEG showed desynchronization during the gelastic seizures, indicating epileptic activity.

Implications:

  • This case highlights gelastic seizures as an unusual presentation of West syndrome.
  • Early recognition of these seizures through EEG and neuroimaging is vital for prompt diagnosis and intervention.
  • Treatment with adrenocorticotropic hormone (ACTH) proved effective, underscoring the importance of timely management in infantile spasms.

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