disease 958 words KG: ent-dise-abdcb2c3 2026-03-21
kind:diseasesection:diseasesstate:published
Contents

Progressive Myoclonus Epilepsy

Disease Info
PrevalenceRare, estimated 1-2 per 100,000 for most forms
Age of onsetVaries by subtype; typically childhood or adolescence
Geographic clustersHigher incidence of Unverricht-Lundborg in Finland and Mediterranean regions
Lafora diseaseMutations in EPM2A (laforin) or NHLRC1 (malin) lead to abnormal glycogen metabolism[@singh2014]
Abnormal glycogen accumulationProgressive intracellular glycogen accumulation in neurons, astrocytes, and peripheral tissues
Lafora bodiesAbnormal glycogen inclusions that disrupt cellular function[@turnbull2011]
Protein aggregationSimilar mechanisms to other protein aggregation disorders (Alzheimer's, Parkinson's)
ER stressEndoplasmic reticulum stress response activation
Oxidative stressIncreased reactive oxygen species and mitochondrial dysfunction
Glutamate dysregulationAltered excitatory neurotransmission
Ion channel dysfunctionMutations affecting sodium and calcium channels
Impaired GABAergic inhibitionReduced inhibitory control contributing to seizures
DatabasesOMIMOrphanetClinicalTrialsPubMed

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