title: Neuronal ceroid lipofuscinosis in a Vietnamese pot-bellied pig (Sus scrofa).
abstract: The neuronal ceroid ipofuscinoses (NCL) are a group of heritable, neurodegenerative, storage diseases, typically with an autosomal recessive mode of inheritance. Cytoplasmic accumulation of storage material in cells of the nervous system and, variably in other tissues, characterizes NCL. NCL has been reported in many animal species, but to the authors' knowledge, this is the first report of the disease in a pig. Blindness and seizures are common clinical signs of disease, neither of which was a feature in this pig. The lesions were restricted to the central nervous system, which was diffusely affected, with the most severe lesions in the hippocampus, cerebral cortex, and cerebellum. The histologic lesions included neuronal loss and gliosis, which contributed to mild cerebrocortical and cerebellar atrophy and accumulation of autofluorescent storage material in neurons and glial cells. The storage material had morphologic, histologic, and ultrastructural properties typical of NCL.
authors.affiliation: Integrated Laboratory Systems, Inc., PO Box 13501, Research Triangle Park, NC 27709 (USA). mcesta@ils-inc.com
authors.forename: M F
authors.initials: MF
authors.lastname: Cesta
authors.forename: K
authors.initials: K
authors.lastname: Mozzachio
authors.forename: P B
authors.initials: PB
authors.lastname: Little
authors.forename: N J
authors.initials: NJ
authors.lastname: Olby
authors.forename: R C
authors.initials: RC
authors.lastname: Sills
authors.forename: T T
authors.initials: TT
authors.lastname: Brown
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology; D002493:Central Nervous System Diseases / Q000473:pathology / Q000662:veterinary*; D017809:Fatal Outcome; D006651:Histocytochemistry / Q000662:veterinary; D009472:Neuronal Ceroid-Lipofuscinoses / Q000473:pathology / Q000662:veterinary*; D034421:Sus scrofa; D013553:Swine Diseases / Q000473:pathology*