Maple syrup urine disease in calves: a clinical, pathological and biochemical study

Abstract

The clinical, pathological and biochemical findings of a study of 30 Poll Hereford, Hereford, Poll Hereford cross or Hereford cross calves affected with branched chain ketoacid dehydrogenase (BCKAD) complex deficiency or maple syrup urine disease (MSUD) are presented. In breeding studies, 6 of 21 calves from obligate heterozygote matings were affected with MSUD, suggesting the disease is inherited in an autosomal recessive manner. Calves were clinically affected from birth, but there were variations in the subsequent course of progressive deterioration of central nervous system function. Concentrations of the branched chain amino acids and keto acids were elevated in pre-suckle plasma and cerebellar water content was higher in affected calves. Activity of BCKAD complex was minimal in fibroblasts cultured from an affected calf. Spongiform encephalopathy and elevated ratios of the branched to straight chain amino acids in formalin fixed cerebral tissue were found in a stillborn foetus and a 3-month-old Hereford calf. These findings suggest the disease occurs prenatally and that a delayed form may exist.

Myelin Vacuolation, Optic Neuropathy and Retinal Degeneration after Closantel Overdosage in Sheep and in a Goat

“…In domestic animals, intoxications characterized by separation of myelin lamellae at the intraperiod line include those caused by overdosage with ammonia (Cho and Leipold, 1977) or copper (Morgan, 1973), ingestion of plants such as Stypandra imbricata (Huxtable et al, (1992) and Huxtable et al, 1980;Main et al, 1981) and Helichrysum argyrosphaerum (van der Lugt et al, 1996) or the fungus Stenocarpella maydis (= Diplodia maydis) (Kellerman et al, 1991 andKellerman et al, 1985;Prozesky et al, 1994). Maple syrup urine disease, a metabolic disorder causing intramyelinic vacuole formation in the brain, has been described in calves (Harper et al, 1989 andHarper et al, 1986).…”

Congenital tremor in Holstein Friesian‐cattle

“…In Limousin and Ayrshire calves encephalopathies characterised by myelinolysis have been reported (14,23,25). In polled Hereford cattle myelinic oedema associated with the socalled maple syrup disease has been described (13,15).…”

Status Spongiosus of White Matter in Newborn Gelbvieh-Cross Calves

“…Chemical analysis of blood and cerebrospinal fluid from an affected calf did not reveal abnormal concentrations of branched chain keto-acids, which are invariably elevated in MSUD. 3,8,18 Neither of the 2 recognized mutations of MSUD was identified in affected calves nor in presumed carrier dams, a grand-dam, or the sire of 1 affected calf. Clinical onset at birth and survival to 10 days are atypical of MSUD, as affected MSUD calves are born bright, alert, and active.…”

“…Clinical onset at birth and survival to 10 days are atypical of MSUD, as affected MSUD calves are born bright, alert, and active. 3,18,19 Several hours to days elapse before neurological signs of bovine MSUD occur.…”

“…8,43 MSUD has been reported in polled Hereford, Hereford, and Hereford cross-bred calves in Australia and in Hereford calves in Canada. 3,18,20,24 MSUD is likely to represent a subset of the hereditary neuraxial edema disease complex in Hereford calves originally described by Cordy et al, 10 and so MSUD probably occurred in the United States.…”