Abstract
Type 1 polysaccharide storage myopathy (PSSM1) is a glycogen storage disorder of known cause whereas the basis for type 2 PSSM (PSSM2) is unknown. The same diet and exercise regime prescribed for PSSM1 is recommended for PSSM2; however, the benefit of these recommendations for PSSM2 is undocumented. The objectives of this study were to determine traits of PSSM2 Warmblood horses (WB), determine the changes in exercise responses that occur with a recommended low-starch/fat-supplemented diet and exercise regime, and determine if glycogen concentrations correspond to the severity of signs. Owners of PSSM2 WB (2008–2016), completed a retrospective questionnaire regarding their horse. Glycogen concentrations were analyzed in skeletal muscle of PSSM2 WB (n = 36) obtained prior to recommendations and in control WB with no evident myopathy (n = 23). Chi-square, Fisher’s exact, McNemar’s tests with Bonferroni correction and Mann Whitney testing were utilized. Abnormal exercise responses reported by owners, began at approximately 6 years of age and included a decline in performance, a reluctance to collect and reluctance to go forward in over 50% of horses. With the recommended diet and exercise regime, 80% of PSSM2 WB owners reported an overall improvement with significant decreases in the proportion of horses showing a decline in performance and rhabdomyolysis. However, 53% of PSSM2 WB were still not advancing as expected with reluctance to go forward and collect persisting in approximately one third of horses. Median muscle glycogen concentrations did not differ between PSSM2 WB and WB with no evident myopathy. PSSM2 WB with the highest glycogen concentrations were significantly more likely to show a decline in performance than those with lower glycogen concentrations. In conclusion, diet and exercise recommendations ideal for PSSM1 improve but do not eliminate the decline in performance and reluctance to go forward under saddle characteristic of PSSM2.
Highlights
In 1992, Polysaccharide Storage Myopathy (PSSM) associated with chronic ER was discovered in Quarter Horse-related breeds based on the hallmark feature of amylase-resistant polysaccharide in skeletal muscle biopsies [1]
The results of the present study provide a comprehensive owner reported description of the characteristics of PSSM2 in Warmblood horses (WB) and for the first time provide valuable prognostic information with regard to response of PSSM2 WB to current diet and exercise management as perceived by Performance Decline in performance Reluctance to collect Reluctance to go forward Neuromuscular Generalized atrophy Rhabdomyolysis Muscle fasciculations Behavior Overall change in behavior
The onset of abnormal exercise responses and behaviors in PSSM2 WB was insidious with an average age of onset of 6 yrs, an age when WB horses are expected to be advancing in their training [23]
Summary
In 1992, Polysaccharide Storage Myopathy (PSSM) associated with chronic ER was discovered in Quarter Horse-related breeds based on the hallmark feature of amylase-resistant polysaccharide in skeletal muscle biopsies [1]. Aggregates of amylase-sensitive glycogen and subsarcolemmal lakes of muscle glycogen were later added as potential diagnostic features which expanded the range of breeds affected by PSSM [2]. Analysis of over 830 horses of a variety of breeds in the US determined that 72% of Quarter Horses and 18% of WB diagnosed with PSSM by the presence of aggregates of amylase- sensitive or resistant glycogen in muscle samples possessed the GYS1 mutation [4].
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