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Review ArticleReview Article
Open Access

Lennox-Gastaut syndrome

Management update

Muradi H. Al-Banji, Doaa K. Zahr and Mohammed M. Jan
Neurosciences Journal July 2015, 20 (3) 207-212; DOI: https://doi.org/10.17712/nsj.2015.3.20140677
Muradi H. Al-Banji
From the Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia
Pharm. D
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Doaa K. Zahr
From the Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia
Pharm. D
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Mohammed M. Jan
From the Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia
MBChB, FRCP(C)
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    Table 1

    Differential diagnoses of drug resistant epilepsy.

    DiagnosisExamples
    None epileptic eventsShuddering attacks, self-gratification, panic attacks, tics, pseudo seizures, dystonic dyspepsia
    Improper managementIncorrect diagnosis or classification, poor compliance, under treatment
    Intractable epilepsy syndromeLGS, infantile spasms, dravet syndrome, Doose syndrome
    Lesional epilepsyCortical dysplasia, mesial temporal sclerosis, cortical tumors, hamartomas
    Treatable inherited metabolic epilepsiesPlease refer to Table 2
    Neuro-metabolic disorderAmino acid or organic acid disorders, urea cycle disorders.
    Neurodegenerative disorderProgressive myoclonic epilepsy, neuronal ceroid lipofuscinosis, mitochondrial cytopathy
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      Table 2

      Treatable inherited metabolic epilepsies.

      DisorderSpecific treatment
      B6 dependent epilepsyPyridoxine
      Pyridoxal phosphate responsive epilepsyPyridoxal phosphate
      Folinic acid responsive seizuresFolinic acid
      Glucose transporter 1 deficiencyKetogenic diet
      Biopterin synthesis disordersL-dopa, 5-hydroxytryptophan, biopterin
      Cerebral folate deficiencyFolinic acid
      Biotinidase deficiencyBiotin
      Serine biosynthesis disordersSerine, glycine
      Creatine synthesis defectCreatine monohydrate
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      Table 3

      Doses of important antiepileptic drugs used for LGS.34

      DrugDose
      Valproic acid (Depakene)15-40 mg/kg/day
      Clonazepam (Rivotril)0.02-0.2 mg/kg/day
      Clobazam (Frisium)0.5-2 mg/kg/day
      Lamotrogine (Lamictal)5-10 mg/kg/day
      1-5 mg/kg/day with valproic acid
      10-15 mg/kg/day with enzyme inducers
      Topiramate (Topamax)2-10 mg/kg/day
      Levetiracetam (Keppra)15-60 mg/kg/day
      Rufinamide (Banzel)25-45 mg/kg/day
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      Neurosciences Journal: 20 (3)
      Neurosciences Journal
      Vol. 20, Issue 3
      1 Jul 2015
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      Lennox-Gastaut syndrome
      Muradi H. Al-Banji, Doaa K. Zahr, Mohammed M. Jan
      Neurosciences Journal Jul 2015, 20 (3) 207-212; DOI: 10.17712/nsj.2015.3.20140677

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      Lennox-Gastaut syndrome
      Muradi H. Al-Banji, Doaa K. Zahr, Mohammed M. Jan
      Neurosciences Journal Jul 2015, 20 (3) 207-212; DOI: 10.17712/nsj.2015.3.20140677
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