S.V. KOTOV, M.S. BUNAK, E.V. BORODATAYA, O.P. SIDOROVA

Moscow Regional Research and Scientific Clinical Institute named after M.F Vladimirsky, 61/2 Schepkin Str., buld. 10, Moscow, Russian Federation, 129110

  Kotov S.V. ― D. Sc. (medicine), Head of the Neurology Department, Head of the Department of Neurology at the Faculty of Advanced Medical Studies, e-mail: kotovsv@yandex.ru, ORCID ID: 0000-0002-8706-7317

Bunak M.S. ― Junior Researcher of the X-ray Department, tel. (495) 681-06-40, e-mail: mark-bunak@mail.ru, ORCID ID: 0000-0001-6436-024

Borodataya E.V. ― PhD (biology), researcher of the Pathology Department, e-mail: Elena.borodataya@gmail.com, ORCID ID: 0000-0002-0096-9140

Sidorova O.P. ― D. Sc. (medicine), Professor of the Neurology Department, e-mail: sidorovaop2008@rambler.ru, ORCID ID: 0000-0003-4113-5700

 Objective ― to evaluate the efficacy of energotropic therapy in Becker myopathy using magnetic resonance imaging of limbs muscles.

Material and methods. An adult patient with Becker myopathy was examined. MRI of the lower limbs muscles was performed on a device with magnetic field induction of 1.5 Tl (Optima Mr450w, GE Healthcare) in TH1 and TH2 mode. To assess tissue respiration (mitochondrial respiratory chain) and other types of metabolism in mitochondria, cytochemical analysis of lymphocytes in peripheral blood was performed.

Results. Becker myopathy was diagnosed in a 44-year-old male. The study of cytochemical activity of mitochondrial enzymes in peripheral blood lymphocytes revealed a decrease in the activity of succinate dehydrogenase, α-glycerophosphate dehydrogenase, glutamate dehydrogenase, lactate dehydrogenase. MRI of the lower extremities muscles revealed a significant lesion of m. Biceps femoris caput longum, m. Gastrocnemius caput laterale and mediale (4 points). Lesion of m. Laterlis vastus femoris, m. Vastus intermedius femoris, m. Femris vastus medialis, m. Biceps femoris caput breve, m. Adductor magus, longus, brevis was estimated as 3 points. Less pronounced lesions were noted in the examination of m. Sartorius, m. Semitendinosus, m. Semimembranosus. They were estimated as 2a points. Taking into account the changes in the cytochemical activity of mitochondrial enzymes in peripheral blood lymphocytes, energy-stimulating therapy was prescribed: carnitine and coenzyme Q10. Positive changes in m. Sartorius were observed 1 year after regular administration of energy-stimulating therapy. After 2 years, the changes in m Biceps femoris caput breve decreased from stage 3 to 1. In m. Biceps femoris caput longum degenerative changes decreased from stage 4 to 2a.

Conclusion. Muscle MRI method should be used in all patients with hereditary neuromuscular pathology for objective evaluation of the pathological process dynamics and for determining the treatment effectiveness.

Key words: Becker myopathy, muscle MRI, mitochondria, carnitine, coenzyme Q₁₀.

(For citation: Kotov S.V., Bunak M.S., Borodataya E.V., Sidorova O.P. Positive effect of energotropic therapy (L-carnitin, coenzim Q₁₀) in patient with Becker muscular dystrophy based on the magnetic resonance imaging of limb muscles. Practical Medicine. 2018)

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