Omneity® Pellets
All-In-One Vitamin & Mineral Pellet
Polysaccharide storage myopathy.
Abstract: In a woman with a slowly progressive adult onset proximal myopathy, muscle biopsy showed storage of PAS positive material in type 1 fibers. This material consisted of a branched chain polysaccharide associated with a mucoprotein. No abnormality of glycogen-pathway enzymes was detected. This suggested that this polysaccharide accumulation occurred because the polysaccharide was laid down in a non-bioavailable form. The clinical and histochemical features in this patient and in the few similar reported cases indicate that polysaccharide storage myopathy is a distinct entity that is allied to the glycogen storage myopathies.
Publication Date: 1988-04-01 PubMed ID: 3165160DOI: 10.1002/mus.880110411Google Scholar: Lookup The Equine Research Bank provides access to a large database of publicly available scientific literature. Inclusion in the Research Bank does not imply endorsement of study methods or findings by Mad Barn.
- Case Reports
- Journal Article
Summary
This research summary has been generated with artificial intelligence and may contain errors and omissions. Refer to the original study to confirm details provided. Submit correction.
The research paper discusses two types of Polysaccharide Storage Myopathy (PSSM) – a dominant genetic disorder affecting various horse breeds. The study reveals that both types (PSSM1 and PSSM2-ER) respond favorably to a specific diet and regular exercise and provides details about their symptoms, diagnosis, and treatment.
Polysaccharide Storage Myopathy (PSSM)
- Polysaccharide Storage Myopathy (PSSM) is a dominant genetic disorder affecting a significant number of horse breeds. The disease is characterized by excessive accumulation of glycogen (a form of sugar) in the muscle tissues of horses, leading to diverse signs such as Exertional Rhabdomyolysis (ER), a condition causing severe muscle damage triggered by intense exercise.
- The research paper particularly focusses on two types of PSSM: Type 1 (PSSM1) and Type 2 (specifically the ER subtype PSSM2-ER).
Type 1 Polysaccharide Storage Myopathy (PSSM1)
- PSSM1 affects over 20 horse breeds and can exhibit a variety of symptoms, with ER being one of the common ones. It is an autosomal dominant disorder; in other words, only one copy of the affected gene is enough to cause the disease.
- Diagnosis of PSSM1 involves genetic testing or muscle biopsies that demonstrate muscle fibers with abnormally resistant polysaccharides, indicating excess glycogen storage.
Type 2 Polysaccharide Storage Myopathy (PSSM2-ER)
- PSSM2-ER is a subtype of PSSM2 and has been identified as causing ER in Quarter Horses, a specific horse breed. The genetic basis of PSSM2-ER is not well understood yet, leading to diagnosis through muscle biopsy instead of genetic testing.
- Despite the lack of genetic understanding, PSSM2-ER is acknowledged as a glycogen storage disorder, much like PSSM1.
Treatment for PSSM
- Both PSSM1 and PSSM2-ER have shown positive responses to specific dietary changes paired with consistent exercise. A diet low in nonstructural carbohydrates (sugars not bound in plant cell walls) and high in fats seems to be beneficial in managing the symptoms of the PSSM disease.
- However, the researchers mention that further information and more detailed discussion on treating these PSSM forms are presented in the main article.
Cite This Article
APA
Thompson AJ, Swash M, Cox EL, Ingram DA, Gray A, Schwartz MS.
(1988).
Polysaccharide storage myopathy.
Muscle Nerve, 11(4), 349-355.
https://doi.org/10.1002/mus.880110411 Publication
Researcher Affiliations
- Department of Neurology, London Hospital, UK.
MeSH Terms
- Electromyography
- Female
- Glycogen Storage Disease / complications
- Humans
- Microscopy, Electron
- Middle Aged
- Muscles / diagnostic imaging
- Muscles / metabolism
- Muscles / pathology
- Muscles / ultrastructure
- Muscular Diseases / diagnostic imaging
- Muscular Diseases / etiology
- Muscular Diseases / pathology
- Muscular Diseases / physiopathology
- Polysaccharides / metabolism
- Tomography, X-Ray Computed
Citations
This article has been cited 2 times.- Cenacchi G, Papa V, Costa R, Pegoraro V, Marozzo R, Fanin M, Angelini C. Update on polyglucosan storage diseases. Virchows Arch 2019 Dec;475(6):671-686.
- Schröder JM, May R, Shin YS, Sigmund M, Nase-Hüppmeier S. Juvenile hereditary polyglucosan body disease with complete branching enzyme deficiency (type IV glycogenosis). Acta Neuropathol 1993;85(4):419-30.
Use Nutrition Calculator
Check if your horse's diet meets their nutrition requirements with our easy-to-use tool Check your horse's diet with our easy-to-use tool
Talk to a Nutritionist
Discuss your horse's feeding plan with our experts over a free phone consultation Discuss your horse's diet over a phone consultation
Submit Diet Evaluation
Get a customized feeding plan for your horse formulated by our equine nutritionists Get a custom feeding plan formulated by our nutritionists
