Progressive myoclonus epilepsy of the Unverricht-Lundborg type (EPM1) is an autosomal recessive neurodegenerative disorder that has the highest incidence among the progressive myoclonus epilepsies worldwide. The primary therapeutic approaches for EPM1 patients include rehabilitation and symptomatic pharmacologic management. Pharmacologic intervention includes: valproic acid, clonazepam, high doses of piracetam, levetiracetam and topiramate and zonisamide. Several case reports suggest that treatment of EPM1 patients with the antioxidant N-acetylcysteine (NAC) alleviates key features of the disorder including dysarthria, ataxia, and seizures. Although the role of oxidative stress in EPM1-linked neuronal degeneration is not completely understood, a specific decrease in cerebellar defenses against oxidative stress and a concomitant increase in lipid peroxidation occurs in the mouse model for EPM1. Myoclonic seizures can also be easily misdiagnosed as tonic-clonic seizures or even pseudoepileptic seizures, especially as the majority of the myoclonic movements are not time-locked to electroencephalogram (EEG) discharges.

Lafora disease typically starts between ages 12 and 17 years, after a period of apparently normal development. Lafora disease-associated mutations are scattered all along the coding regions of the EPM2A and NHLRC1 genes, but also accumulate in discrete spots of high recurrence. The main seizure types in Lafora disease include myoclonic seizures and occipital seizures, although generalized tonic-clonic seizures, atypical absence seizures, and atonic and complex partial seizures may occur. Studies of the combined mutation detection frequency of sequence analysis in EPM2A and NHLRC1 reveal that between 88% and 97% of mutations in these two genes can be detected using sequence analysis alone. Antiepileptic drugs have a major effect against generalized seizures, sometimes controlling seizures for many months. Valproic acid is the traditional antiepileptic treatment for Lafora disease because it is a broad-spectrum drug that controls both the generalized tonic-clonic seizures and myoclonic jerks.