Maple syrup urine disease as a cause of spongiform encephalopathy in calves

Abstract

Estimates of branched chain amino acid concentrations in plasma and, or, serum, urine, cerebrospinal fluid, formalin-fixed cerebral tissue and the associated formalin, provided evidence for a diagnosis of branched chain keto acid decarboxylase deficiency in five polled Hereford calves. The similarity of the clinical signs of dullness, recumbency and opisthotonos, and the observation of severe status spongiosus within the central nervous system, indicated that this condition had probably affected seven other newborn calves. It is suggested that this condition is analogous to branched chain keto acid decarboxylase deficiency or maple syrup urine disease of children.

Etude d’un nouveau syndrome du veau charolais nouveau-né : les gastro-entérites paralysantes. I. Etude clinique, premiers résultats épidémiologiques

“…Ces derniers résultats évoquent les caractéristiques biochimiques de la maladie des urines à odeur de sirop d'érable (leucinose de l'enfant) décrite dans la race Hereford, maladie héréditaire liée probablement à un gène autosomal récessif résultant d'un blocage de la décarboxylation oxy dative des acides cétoniques ramifiés (isocaproïque, 3 méthyl-valérique, isovalérique) issus respectivement de la leucine, de l'isoleucine et de la valine (1) (10). Si les troubles diarrhéiques peuvent être du ressort du pouvoir patho gène des virus en particulier des rotavirus, cette catégorie de contami nants ne semble pas avoir la capacité de rendre compte des symptômes nerveux.…”

Status Spongiosus of White Matter in Newborn Gelbvieh-Cross Calves

“…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.…”

“…13,14 Other, less well-characterized forms of congenital or perinatal status spongiosus occur in cattle. 19,25,26,33,40 These vary in time of onset, clinical presentation and progression, presence or absence of gray matter changes, presence and severity of hypomyelination or dysmyelination, and whether spongy change is diffuse or localized.…”

“…19 MSUD calves become dull, recumbent, and exhibit opisthotonos, with some developing convulsions in the terminal stages of the disease. Status spongiosus is present predominantly in white matter of the brain due to intramyelinic edema and splitting of lamellae.…”

“…19,20,36 The anatomical basis may be swelling of myelin sheaths, of cytoplasm of astrocytes and/or oligodentrocytes, or of processes in neuropil. 15 Status spongiosus may occur as a result of metabolic disorders in animals and humans or due to specific intoxications or infections.…”