A Neurological Disease with Spongy Degeneration in a Newborn Japanese Black Calf

Abstract

ABSTRACT. A Japanese Black calf, 3 day-old male, showed severe ataxia, lateral recumbency, and opisthotonos at the birth. Histopathological examinations revealed severe status spongiosis throughout the central nervous system. Numerous vacuoles within the neuropile varying in size and shape were observed in both formalin-fixed paraffin and cryostat sections. In the lesions, a limited number of spheroids and macrophages were observed within the myelin sheaths with very mild astrogliosis. These vacuoles were negative for both periodic acid Schiff and Sudan black stains. The clinical and histopathological features were almost in conformity with those of bovine maple syrup urine disease (MSUD). Although we could not confirm completely the etiology, congenital hereditary neurological diseases including MSUD are considerable as the possible disease entry in the present case. KEY WORDS: bovine, maple syrup urine disease, spongy degeneration.J. Vet. Med. Sci. 67(10): 1067-1070, 2005 Spongy degeneration in the central nervous system (CNS) is the most characteristic morphological feature of prion diseases in several animal species. Among them, the diagnosis and disclose of ovine or caprine scrapie and bovine spongiform encephalopathy (BSE) are very important especially in veterinary fields [13]. However, spongiform change is not specific feature for prion diseases, but other several metabolic or hereditary degenerative diseases also cause similar changes in the CNS. In humans, Canavan's disease, autosomal recessive disorder based on Nacetylaspartic acid metabolism alteration, has been known to cause spongy degeneration in the brain [2,9]. In addition, maple syrup urine disease (MSUD), autosomal recessive disorder due to hereditary metabolic disturbance of branched-chain amino acid (BCAA), results in status spongiosis in the CNS [1]. Even in calves, similar hereditary or metabolic CNS disorders including MSUD and congenital cerebral edema have been reported in the Hereford breed [3][4][5][6][7]12].In Japan, the first case of BSE was diagnosed in September 2001. Then, mass-screening system based on a detection of abnormal prion protein (PrP) by ELISA has been adopted at meat inspection and livestock hygiene centers since October 2001. The final diagnoses were based on the results of both Western blot examination and immunohistochemistry. Since the screening system for BSE has applied for all meat cattle and all ill or dead cattle over 24 month-old, hereditary degenerative disorders other than prion diseases might not be serious differential diagnosis to rule out. Besides, when very young calves developed spongy degeneration in the brain, these diseases could be very important. In addition, status spongiosis in the CNS may be induced by heavy metal poisoning such as triethyl tin [10]. It has been also known that spongiosis-like vacuolar changes are often produced by inadequate tissue processing for paraffin sections.The present study describes pathological features of a Japanese Black calf with severe congenital neurological ...

Carrier rate of the c.235G>C mutation in the bovine isoleucyl-tRNA synthetase (<i>IARS</i>) gene of Japanese Black cows at Kagoshima prefecture, Japan, and analysis of the metabolic profiling and reproductive performance of heterozygous cows

“…We found that all data relating to calves were very similar between the wild-type and carrier cows, whereas data relating to the treatment of cows indicated that the frequency and cost of treatments were slightly higher in the carrier cows than in the wild-type cows, although differences between the two groups were not significant. [8,9] and maple syrup urine disease (OMIA 000627) [6]. Collectively, these genetic disorders are assumed to have a substantial economic impact on the JB cattle industry; however, the details have yet to be evaluated.…”