Aicardi Syndrome is an extremely rare genetic disorder characterized by infantile seizures/spasms, agenesis of the corpus callosum, chorioretinal lacunae, and learning disabilities. It is likely caused by a de novo mutation in a gene in the X chromosome. However, the gene that causes this syndrome is still not known. It is diagnosed based on clinical findings. Aicardi syndrome may present as a clinical spectrum, from mild to severe disease. In general, the younger the age at which epilepsy and learning disabilities are diagnosed, the more severe the epilepsy and learning difficulties become later in life. Hence, long-term surveillance and management are warranted. This paper presents a 6-month-old Filipino female who exhibited the classic triad of Aicardi Syndrome: profound seizure episodes; callosal agenesis and interhemispheric cysts; and chorioretinal lacunae. Several anti-epileptic drugs such as Phenobarbital, Clonazepam and Topiramate were given for the seizures. Ophthalmogic examination and retinal camera fluorescein angiogram were advised to be performed regularly as well as consistent neurodevelopmental follow-up.

Gelastic seizure is a rare seizure type, with laughter as the main ictal manifestation. In the presence of a hypothalamic hamartoma, laughing seizures are referred to as gelastic epilepsy which is seen in less than 5% of epilepsies [1]. These seizures begin during infancy with a progressive course and may present with precocious puberty and cognitive decline. In the absence of a hypothalamic hamartoma, gelastic seizures have a later onset and are more seldom encountered. These are seen in less than 1% of all epilepsies and occur as part of a frontal or temporal lobe epilepsy [1]. For gelastic seizures not associated with this lesion, prognosis is good since they are more responsive with AEDs and may be controlled by a single AED. This is a case report of a 7-year-old male who presented with recurrent attacks of spontaneous, mirthless, and inappropriate laughter associated with hyperkinetic movements. Workup did not show a hypothalamic hamartoma. Interictal EEG showed bilateral frontal lobe discharges in prolonged runs. He was given carbamazepine which provided adequate seizure control. This is the second case reported in this institution from 1992 until present.