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A14 month old child with Seizure

A 14-month-old child with Dravet syndrome presented with uncontrolled partial and hemiclonic seizures. Despite multiple drug treatments, seizures became serial and status, until Nesdonal combined with Phenobarbital proved effective. Genetic mutations in Dravet identified include SCN1A, SCN1B, PCDH19, GBRG2.

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A14 month old child with Seizure

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  1. A14 month old child with Seizure Dravet syndrome

  2. History • A 14 m lod child referred to our hospital with uncontrolled seizure.It was Partial and hemiclonic and duration was 3-4 minutes.He had fever(lowgrade)and symptoms of a viral infection.(Cough-Coriza) • PMH: • Product of C/S with no history of Asphyxia –Icter or truama.At 2month of age he had a seizure after vaccination.He discharged without any medication.At 6 month of age an dafter vaccination he had a seizure again and Pb prescribed for him.He was seizure free for 9 month.

  3. FH: Parents aren't relative. Positive history of seizure is seen in his uncle. • Development: Normal till 6 m.

  4. DRUGS & Treatments • Phenobarbital wasn't effective. • Pb +PHTnot effective • Seizures became serial and to status • Midazolam(drip) was not effective • Sodium valproate+topiramate(+Carnitine) adminstered.Not effective • Propofole used for him.(Not effective) • Nesdonal Controlled

  5. We start tapering of Nesdonal and add Phenobarbital to ourdrugs (PB+TOPIRAMATE+CLOBAZAM)

  6. Mutations in Dravet • SCN1A • SCN1B • PCDH19(protocadherin 19) • GBRG2

  7. Treatment

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