Walsh & Hoyt: Neuronal Ceroid Lipofuscinoses

Neuronal ceroid lipofuscinosis is characterized by brain and retinal atrophy with selective necrosis of neurons, leading to progressive mental and motor deterioration. Visual loss is an important symptom that is prominent among children and less so among adults. These disorders are characterized by the accumulation in the body of two lipopigments: lipofuscin and ceroid. Distinctive membranebound cytoplasmic inclusions have granular, curvilinear, or fingerprint patterns. The infantile Hagberg-Santovouri type, late-infantile Jansky-Bielschowsky type, juvenile Spielmeyer-Vogt form, and adult Kufs type were delineated on clinical grounds