Republic of the Philippines

Benguet State University

COLLEGE OF VETERINARY MEDICINE
La Trinidad, Benguet
DEPARTMENT OF CLINICAL SCIENCES

VM174 Ruminant Medicine

MAPLE SYRUP
URINE DISEASE
Sales, Dylan Joy A.
 ETIOLOGY
• Caused by an accumulation of branched chain
amino acids including valine, leucine and
isoleucine due to absence of branched chain
ketoacid decarboxylase (Radostits, et al., 2006)
• Lethal genetic abnormality and is simple
autosomal recessive gene. (Bedwell, 2019)
• This genetic abnormality leads to metabolic
disorders brought by amino acid deficiency
EPIDEMIOLOGY
This aminoacidopathy has been diagnosed
in polled Shorthorn, polled Hereford, and
Hereford cattle in Australia, Uruguay,
Argentina and Canada and is result of
mutation of branched chain alpha-ketoacid
dehydrogenase complex (Santos, 2015)
TRANSMISSION
• Not contagious and non-infectious
• Genetic defect which is hereditary
 PATHOGENESIS
A deficiency in branched-chain α-ketoacid dehydrogenase complex results in an inability to
oxidize the branched-chain ketoacids of the branched-chain amino acids leucine, isoleucine,
and valine

Loss of this activity results in the accumulation of the branched-chain amino acids along with
their respective ketoacids: ketoisocaproic, keto-β-methylvaleric, and ketoisovaleric acids in
cerebrospinal fluid, blood, and tissues.

These metabolites would then cause nervous system dysfunction.

 CLINICAL SIGNS & SYMPTOMS
(Radostits, 2006)

• Dullness, recumbency, tremor, tetanic spasms and

opisthotonos, a scruffy coat, blindness, and severe
hyperthermia
• When held in a standing position some calves have
tetanic paralysis, others have flaccid paralysis
• When held in a standing position some calves have
tetanic paralysis, others have flaccid paralysis
• Burnt sugar smell of urine
Figure 1. Calve showing tetanic paralysis
CLINICAL PATHOLOGY
(Robarge et. al., 2015)

• Status spongiosus
• Histologic hallmark of MSUD
• Straight chain amino acids in nervous
tissue
DIAGNOSIS
DNA testing is the only method of
screening and diagnosis at this point in
time

PREVENTION AND CONTROL

At the present time there is no research
being done on this disease and no urgency
due to its perceived rarity
TREATMENT
There are no medications or therapies
that can reverse this process available at
this time

ZOONOTIC RISK
Maple syrup urine disease is not zoonotic
 REFERENCES:
• Bedwell, S. (2019). MSUD Explained. American HerefordAmerican
Hereford Association.
https://hereford.org/wp-content/uploads/2019/04/0419_PerformanceMatters
.pdf Maple syrup urine disease (MSUD). (2019, April 26). H and R Ranch.
https://www.handrminiherefords.com/page-116546.html
• Radostits, O. M., Gay, C. C., Hinchcliff, K. W., & Constable, P. D. (2006).
Veterinary medicine: A textbook of the diseases of cattle, horses, sheep,
pigs and goats. Elsevier Health Sciences.
• Robarge, M. E., Beever, J. E., Lenz, S. D., Lynch, C. J., & Wigle, W. L.
(2015). Maple syrup urine disease in a Central Indiana Hereford herd. Case
Reports in Veterinary Medicine, 2015, 1-4.
https://doi.org/10.1155/2015/204037