Author(s): Aamir Jalal Al-Mosawi
Background: Childhood Lennox-Gastaut syndrome is a heterogeneous epileptic encephalopathy with onset between the age of one and seven years. It is associated with more than one seizure type that are drug-resistant, a slow spike-wave activity in the EEG recording and mental retardation .The syndrome was first described by William Gordon Lennox in 1950, and later by Henri Gastaut in 1966. The condition was first called “Lennox-Gastaut syndrome” by Ernst Niedermeyer in 1968. The association of Lennox-Gastaut syndrome with low set ears and cryptorchidism has not been reported before.
Patients and Methods: A two and three months boy was referred late during February, 2022 because of seizures that started during the last week of December, 2021 and were not controlled with multiple anti-epileptic drugs. The diagnosis was made and an evidence-based therapeutic approach is described.
Results: When the boy was first seen he was still experiencing frequent generalized tonic clonic seizures that may last for about ten minutes and myoclonic seizures, despite he was receiving four anti-seizure drugs in good doses including valproic acid, clobazam, levetiracetam, topiramate. The drugs made the boy sleepy most of the day. Brain MRI was performed during August, 2021 for the evaluation of developmental retardation showed normal findings. The presence of more than seizure type, the EEG recording, and the developmental and retardation together with resistance of seizures to polytherapy allowed confident diagnosis of Lennox-Gastaut syndrome. Anti-epileptics were replaced with other drugs and evidence-based agents were used for neuroprotection and improving development and mental retardation. Treatment was associated with a significant improvement.
Conclusion: A new variant of childhood Lennox-Gastaut syndrome is reported and the experience with its early treatment is described.
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