Diagnosis of late infantile neuronal "ceroid-lipofuscinosis" from histochemical and ultrastructural changes in dental pulp.
Sections of dental pulp were removed from a tooth of a boy 3 years 10 months of age with myoclonic seizures. When stained with hematoxylin and eosin, these sections showed eosinophilic inclusions. Ultrastructurally, these inclusions were large cytosomes filled with whorls of electron-opaque substance. The opaque material consisted of bilaminate strands, approximately 15 nm in diameter, arranged in a curvilinear pattern compatible with that seen in the late infantile form of "ceroid-lipofuscinosis." Most histochemical staining reactions were compatible with ceroid or lipofuscin, with the exception of the diamine silver reaction. Late infantile ceroid-lipofuscinosis represents another disease in which asymptomatic accumulation of storage material occurs in the dental pulp.
D002675:Child, Preschool; D003782:Dental Pulp / Q000378:metabolism* / Q000648:ultrastructure; D006651:Histocytochemistry; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D009207:Myoclonus / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D009472:Neuronal Ceroid-Lipofuscinoses / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D013194:Staining and Labeling / Q000379:methods