Abstract :
Aim: To highlight the clinical and electroencephalographic features, treatment strategies and outcome in atypical variant of benign childhood epilepsy with centrotemporal spikes (BECTS).
Materials and Methods: Subject underwent video electroencephalograph (EEG), high-resolution magnetic resonance imaging (MRI), neuropsychological evaluation and language assessment and response to medication was followed up. In addition to sodium valproate, treatment with intravenous methylprednisolone (given as monthly bolus doses) was given as the seizures remained refractory to anti-seizure medications (ASM) alone.
Results: Earlier onset, increased frequency and duration of focal seizures compared to prototype Rolandic epilepsy are cardinal features suggestive of atypical variant of BECTS. Head drop and truncal sway due to axial or axiorhizomelic atonia occurring several times per day or week was the key manifestation. When the atypical seizures commenced, one or more of the following problems can occur: hyperactivity, attention deficit, and mild cognitive or language dysfunction. Child became seizure free on a combination of intravenous methylprednisolone, with sodium valproate and his hyperactivity reversed.
Conclusion: BECTS in children with an early age of onset, frequent and prolonged seizures and scholastic decline pari passu with seizure onset are more likely to evolve into atonic-BECTS. Carbamazepine, oxcarbazepine and some benzodiazepines may worsen these seizures. They can become seizure free with immunomodulatory therapy, namely methylprednisolone (given as monthly bolus doses) or intravenous immunoglobulin (IVIG), and have complete resolution of the transient motor and cognitive impairment. Atonic-BECTS needs to be differentiated from Lennox–Gastaut syndrome since it is potentially treatable and children recover with no sequel.
Keyword :
Benign Rolandic epilepsy, Mental retardation, Atypical benign epilepsy, CSWS