Lafora’s disease presents with –
Correct Answer: Myoclonic epilepsy
Description: Ans. is 'b' i.e., Myoclonic epilepsy Lafora's disease (6q 24) Lafora Bodies (a myloid bodies, myoclonus bodies, polyglucosan bodies)o Rounded periodic acid schiff-positive, argyophillic intracytoplasmic inclusion bodies with a dense central basophilic core and a rim that takes up less stain found in the larger neurons of the thalamus, dentate nuclei and substantia nigra in progressive myoclonic epilepsy (lafora body disease) and less abundantly in normal aged brain.Lafora body disease (progressive myoclonic epilepsy with lafora bodies)o Recessively inherited poliencephalomyopathy of late childhood or early adult life.o It is mapped on 6q 24 but there is more than one locus.o It is clinically characterized by# Progressive dementia# Dysarthria# Visual loss# Pyramidal and cerebellar signs# Photoconvulsive progressive myoclonic and other seizure types.o The diagnostic pathological finding is la fora bodies.Intraneuronal inclusion bodiesConditionPicks bodiesPicks diseaseLewy bodiesParkinson s disease and Lewy body diseaseHirano bodiesAlzheimer s diseaseBunina bodiesAmyotrohic lateral sclerosisLafora bodiesProgressive myoclonic epilepsyMarinesco bodiesNormal Brain tissue
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