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Neuronal ceroid‐lipofuscinosis and hydrocephalus in a chihuahua
Author(s) -
Kuwamura M.,
Hattori R.,
Yamate J.,
Kotani T.,
Sasai K.
Publication year - 2003
Publication title -
journal of small animal practice
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.7
H-Index - 67
eISSN - 1748-5827
pISSN - 0022-4510
DOI - 10.1111/j.1748-5827.2003.tb00148.x
Subject(s) - lipofuscin , medicine , neuronal ceroid lipofuscinosis , hydrocephalus , fontanelle , pathology , lamellar granule , anatomy , lamella (surface anatomy) , ultrastructure , radiology , disease
A two‐year‐old, female chihuahua presented with a six‐month history of visual dysfunction. Computed tomography revealed dilation of the lateral ventricles in the central nervous system (CNS). The dog was tentatively diagnosed as having hydrocephalus and a month later was euthanased at the owner's request. The skull was expanded and dome‐like in shape and an open fontanelle was observed on postmortem examination. Histologically, swollen neurons possessing yellowish pigment granules in the cytoplasm were observed throughout the CNS. These storage materials stained positively with periodic acid Schiff, Schmorl method for lipofuscin and oil red O for lipid, and showed autofluorescence under fluorescence microscopy. Ultrastructurally, the storage materials consisted of dense lamellar structures. This case was unique in having ceroid‐lipofuscinosis in association with hydrocephalus.