| Literature DB >> 31932179 |
Matthew S Gentry1, Zaid Afawi2, Dustin D Armstrong3, Antonio Delgado-Escueta4, Y Paul Goldberg5, Tamar R Grossman5, Joan J Guinovart6, Frank Harris7, Thomas D Hurley8, Roberto Michelucci9, Berge A Minassian10, Pascual Sanz11, Carolyn A Worby12, Jose M Serratosa13.
Abstract
Lafora disease (LD) is both a fatal childhood epilepsy and a glycogen storage disease caused by recessive mutations in either the Epilepsy progressive myoclonus 2A (EPM2A) or EPM2B genes. Hallmarks of LD are aberrant, cytoplasmic carbohydrate aggregates called Lafora bodies (LBs) that are a disease driver. The 5th International Lafora Epilepsy Workshop was recently held in Alcala de Henares, Spain. The workshop brought together nearly 100 clinicians, academic and industry scientists, trainees, National Institutes of Health (NIH) representation, and friends and family members of patients with LD. The workshop covered aspects of LD ranging from defining basic scientific mechanisms to elucidating a LD therapy or cure and a recently launched LD natural history study.Entities:
Keywords: Glycogen; Glycogen storage disease; Lafora disease; Neurodegeneration; Progressive myoclonus epilepsy
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Year: 2020 PMID: 31932179 PMCID: PMC7024738 DOI: 10.1016/j.yebeh.2019.106839
Source DB: PubMed Journal: Epilepsy Behav ISSN: 1525-5050 Impact factor: 2.937