Gelastic epilepsy associated with lesions other than hypothalamic hamartoma

Panagariya, A.; Sharma, Bhawna; Tripathi, Gautam; Kumar, Hrisikesh; Agarwal, Vinay

doi:10.4103/0972-2327.33218

Cited by 2 publications

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“…Patients with GS and HH have been reported previously from India both in adults and children [7][8][9]; however, the other varieties have rarely been reported [10]. In most cases, the laughter lacks any sensation but occasionally a feeling of mirth may be associated with the laughter [3].…”

Section: Discussionmentioning

confidence: 99%

Cryptogenic Gelastic Epilepsy: A Pediatric Case Vignette

Mishra

Juneja

Chutani

et al. 2014

JCDR

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Gelastic seizures, characterized by epileptic laughter, are rare and the majority is associated with hypothalamic hamartomas. We report a case with cryptogenic Gelastic seizure (without hypothalamic hamartoma), as the MRI was normal and, EEG and clinical data suggested a focal origin of the seizures. CASe RepoRTA 4-year-old child, growing well, started having seizures, which were atonic in nature. After three such seizures over a period of one month, the child started having two distinct type of seizures: (i) abnormal tonic-clonic movements initiated on the right half of body and then becoming generalized and followed by post-ictal drowsiness (right simple partial seizures with secondary generalization), and (ii) sudden episodes of inappropriate laughter lasting for 15-20 seconds (gelastic seizures). A CT scan of the head was normal. The initial frequency was 10-15/day (gelastic seizures) and 3-4/week for the partial seizures. The seizures remained controlled on sodium valproate 50mg/kg/day for 3½ months (May 2009 onwards), but restarted. He was referred to us for the management of the epilepsy.He had a normal birth and perinatal history. No family history of epilepsy reported. There was no evidence of precocious puberty. Development quotient was within normal range. EEG showed abnormal awake record consistent with left hemispheric temporal origin of discharges. Video-EEG showed similar findings. MRI head with epilepsy protocol was normal. Interictal PET scan was normal.The seizures were refractory to valproate monotherapy (maximum dose 62mg/kg/day, pre-dose serum valproate level 96 mg/dL). Sequential addition of carbamazepine (maximum 30mg/kg/d), clobazam (maximum dose of 1.5 mg/kg/day), and levetiracetam (68 mg/kg/day) did not achieve satisfactory control of gelastic seizures (3-4 seizures/day), although the partial seizures responded. Discontinuing levetiracetam and carbamazepine, followed by addition of phenobarbitone (6 mg/kg/day) led to control of seizures. At last follow-up, 17 months after complete seizure control, child was seizure-free and developmentally normal.Interestingly, at the last follow-up, the younger male sibling (2½-yearold) also started having complex partial seizures with left temporal focus, but is well-controlled on carbamazepine monotherapy.

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Section: Discussionmentioning

confidence: 99%