Multiple drugs
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Lack of efficacy: case report A 5-month-old male infant exhibited lack of efficacy during treatment with pyridoxal phosphate and sodium valproate for West syndrome. Additionally, he exhibited lack of efficacy during treatment with aripiprazole, clonazepam, ethosuximide, levomepromazine, methylphenidate, risperidone, thioridazine and zonisamide [dosages and routes not stated]. The male infant, who had hyperbilirubinaemia and had undergone phototherapy, was diagnosed with West syndrome at the age of 5 months. His family history was unremarkable. He was treated with pyridoxal phosphate and valproic acid, which were ineffective. Then, he was treated with tetracosactide [Cortrosyn-Z] with suppression of seizures. However, at the age of 9 months, the seizures recurred. He was treated with zonisamide and clonazepam with inadequate effects. At the age of 11 months, he was treated with second synthetic tetracosactide therapy with suppression of seizures. However, the seizures recurred. Hence, he was treated with ethosuximide which was also ineffective. After the onset of epilepsy, he had developmental delay. He developed tonic attacks from the age of 2 years and 10 months. EEG revealed generalised paroxysmal fast activity during sleep and slow spike-wave complexes during seizures, so transformation to Lennox-Gastaut syndrome was diagnosed. Subsequently, he was treated with nitrazepam, clobazam, phenytoin, primidone, topiramate, lamotrigine, gabapentin, rufinamide and levetiracetam with poor efficacy and the seizures persisted. He developed behavioral abnormalities such as hyperactivity and aggression, and autistic behaviour such as kicking things, irritability and hitting people from 5 to 9 years of age. Then, he developed self-harm such as bitting and pinching from 9 to 10 years of age. He developed myoclonic seizures from the 11 years of age. At the age of 18 years, the seizures were intractable. He was treated with levomepromazine [levomepromazine maleate], thioridazine [thioridazine hydrochloride], methylphenidate [methylphenidate hydrochloride], aripiprazole and risperidone but were ineffective. Hence, he started receiving perampanel at 18 years and 8 months of age with decrease in tonic seizures. At last follow-up (at the age of 22-years), myoclonic seizures and focal seizures were also suppressed. He was receiving perampanel, sodium valproate, zonisamide, rufinamide and clobazam. Maura N, et al. A case of dramatic improvement of lennox-gastaut syndrome in both seizures and aggressive behaviors by perampanel. [Japanese]. Journal of the Japan 803507074 Epilepsy Society 38: 36-42, No. 1, Jun 2020. Available from: URL: http://doi.org/10.3805/JJES.38.36 [Japanese; Summarised from a translation]
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Reactions 17 Oct 2020 No. 1826
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