Ketogenic Diet In Inclusion Body Myositis

None listed

Sporadic inclusion body myositis is the most common myopathy in patients over 50 years of age.1 The prevalence of the condition in Australia is 9.3 per 1 million inhabitants; it is relatively rare in non-Caucasians.2,3 Clinically, the most involved muscles are usually the long finger flexors and quadriceps muscles, with the biceps brachii and foot dorsiflexors also commonly afflicted.4 The mean decline in muscle strength is about 5% per year.4,5 Further symptoms of the disease include dysphagia, which occurs in 40 to 80% of patients, as well as global weakness and fatigue.4 In its later stages, inclusion body myositis can result in severe disability, leading to the eventual use of a wheelchair in virtually all patients, and severely reduced quality of life, such that a significant minority opt for euthanasia.5,6 The pathophysiology of inclusion body myositis is incompletely understood, but it appears to involve the coexistence of muscle inflammation and degeneration.3,4 On one hand, inclusion body myositis is characterized by upregulated inflammatory mediators and an aggressive cytotoxic T cell invasion of nonnecrotic muscle fibers.4 On the other hand, it is also characterized by the accumulation of abnormal protein aggregates, many of which have been described in neurodegeneration,6 and mitochondrial structural changes suggestive of mitochondrial dysfunction.4 Moreover, it has been suggested that autophagy, a process responsible for the degradation and recycling of damaged cell components, may be impaired, or even overloaded, in inclusion body myositis.3,4 To date, inclusion body myositis has proven refractory to all known pharmacological treatments; current management focuses on exercise, physical therapy, orthotic devices, and occupational therapy.3 A well-balanced diet has also been suggested as potentially beneficial,4 but to our knowledge, no prospective diet studies in inclusion body myositis have been attempted. In theory, a high-fat, low-carbohydrate “ketogenic” diet may have beneficial effects in inclusion body myositis. Preliminary evidence from animal studies suggests that such a diet exerts anti-inflammatory effects,7,8 possibly by producing fewer reactive oxygen species and/or by elevating levels of the anti-inflammatory neuromodulator adenosine.7 With respect to muscle fiber degeneration, evidence from animal studies also shows that ketogenic diets may result in increased ATP levels and enhanced mitochondrial biogenesis,8 both of which may alleviate the potential energy shortage produced in the context of mitochondrial dysfunction. On this background, our objective is to observe the effects of a ketogenic diet in a patient with inclusion body myositis for a period of 1 year, and to determine whether this approach is plausible, safe, and efficacious with respect to strength, function, and quality of life. References: (1) Needham M, Corbett A, Day T, et al. Prevalence of sporadic inclusion body myositis and factors contributing to delayed diagnosis. J Clin Neurosci 2008;15:1350-1353. (2) Phillips BA, Zilko PJ, Mastaglia FL. Prevalence of sporadic inclusion body myositis in Western Australia. Muscle Nerve 2000;23(6):970-972. (3) Mazen M, Dimachkie MD, Barohn RJ. Inclusion Body Myositis. Semin Neurol 2012;32(3):237-245. (4) Schmidt K, Schmidt J. Inclusion body myositis: advancements in diagnosis, pathomechanisms, and treatment. Curr Opin Rheumatol 2017;29(6): 632-638. (5) Cox FM, Titulaer MJ, Sont JK, et al. A 12-year follow-up in sporadic inclusion body myositis: an end stage with major disabilities. Brain 2011;134(Pt 11):3167-3175. (6) Macado P, Brady S, Hanna MG. Update in inclusion body myositis. Curr Opin Rheumatol 2013;25:763-771. (7) Masino AS, Ruskin DN. Ketogenic Diets and Pain. J Child Neurol 2013;28(8):993-1001. (8) Storoni M, Plant GT. The Therapeutic Potential of the Ketogenic Diet in Treating Progressive Multiple Sclerosis. Mult Scler Int 2015; 2015: 681289.

No related papers found yet.