TY - CHAP M1 - Book, Section TI - Lafora Syndrome or Disease A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - It is a very rare congenital neurodegenerative and progressive myoclonic syndrome associated with seizures and severe mental deterioration. It is characterized by the presence of intracellular inclusions bodies, Lafora bodies, in the cell of the heart, liver, muscle, and skin. Patients develop the first clinical signs during adolescence and they consist of severe seizures, syncope, myoclonus, ataxia, and, rapid development of dementia. The life expectancy of all individuals affected with Lafora disease (LD) is the age of 25, but death usually happen within 10 years of the onset of symptoms. At present, there is no cure or treatment for this disease. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/07 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164076199 ER -