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Carbamezapine
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Imiglucerase
Phenytoin
Valproate
Vigabatrin
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This patient with a generalized tonic-clonic seizure (GTCS), absence seizures (episodes of inattention), myoclonic jerks (jerking of the upper extremities with preserved consciousness), and characteristic EEG findings (4 to 6 Hz bilateral polyspikes) most likely has juvenile myoclonic epilepsy (JME). Valproate is recommended as the first-line treatment for patients with JME. JME is an idiopathic generalized epilepsy. JME usually presents in adolescence with 3 common seizure types: myoclonic jerks, GTCS, and absence seizures. Myoclonic jerks are isolated jerks usually involving the upper limbs during which consciousness is preserved. GTCS occurs in almost all patients with JME and is the most common seizure that leads to diagnosis. Absence seizures can precede other seizure types by several years, and are characterized by brief periods of inattention. Diagnosis can be secured by overnight interictal electroencephalography, which has a nearly 100% sensitivity for JME. Patients with JME are usually very responsive to treatment with antiepileptic drugs (AED). The first-line treatment is valproate; however, this medication should be used with caution in postpubertal female patients. Other AEDs that are effective in JME include levetiracetam, lamotrigine, and topiramate. In addition, patients should avoid seizure triggers such as sleep deprivation, alcohol use, and medication nonadherence. Chowdhury et al. study the use of valproate in the treatment of JME. The authors found that 92% of the 186 patients studied had remission of JME after antiepileptic drug treatment. The majority of these patients were treated with sodium valproate. The authors recommend the use of valproate to treat JME. Incorrect Answers: Answer 1: Carbamazepine can be used in the treatment of both partial and generalized seizures. However, carbamazepine should be avoided in JME. Although it can control GTCS in refractory cases, it can worsen both absence seizures and myoclonic jerks. Answer 2: Imiglucerase is an enzyme replacement therapy that can be used to treat nonneuronopathic Gaucher disease. Gaucher disease has a wide variety of presentations, with Gaucher disease type 3a characterized by progressive dementia, ataxia, and myoclonus. However, Gaucher disease type 3a usually presents earlier in childhood. In addition, imiglucerase does not cross the blood-brain barrier and is ineffective in patients with neuronopathic Gaucher disease. Answer 3: Phenytoin is an antiepileptic that can be used in the treatment of both partial and generalized seizures. Like carbamazepine, phenytoin can worsen both absence seizures and myotonic jerks. Although it can be used to control GTCS in refractory cases, phenytoin should generally be avoided in patients with JME. Answer 5: Vigabatrin is an antiepileptic that can be used in the treatment of partial seizures. It should be avoided in patients with JME as it can worsen all seizure types. There are also reports that vigabatrin can lead to myoclonic or absence status epilepticus in patients with JME. Bullet Summary: Juvenile myoclonic epilepsy (JME) presents in adolescence with the triad of generalized tonic-clonic seizures, absence seizures, and myotonic jerks; valproate is the first-line treatment for JME.
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