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Health News Archive 238 - Muscle Strength
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CoQ10 Improves Muscle Strength

In the January 2006 edition of the prestigious journal Neurology, physicians in Germany reported on three unrelated patients with muscle CoenzymeQ10 deficiency, ages 6, 29 and 32 years old, who presented with muscle weakness and elevation of muscle enzymes in the blood. Muscle biopsy showed lipid storage myopathy, combined deficiency of respiratory chain complexes I and III, and CoQ10 levels below 50% of normal. Supplementation with oral high dose CoQ10 improved muscle strength dramatically and normalized the muscle enzyme in the blood.

Source: R. Horvath, P. Schneiderat, B.G.H. Schoser, K. Gempel, E. Neuen-Jacob, H. Plöger, J. Müller-Höcker, D. E. Pongratz, A. Naini, S. DiMauro, and H. Lochmüller. Coenzyme Q10 deficiency and isolated myopathy. Neurology 2006 66: 253-255.

CoQ10 Deficiency Causes Muscle Weakness

Background Primary coenzyme Q10 (CoQ10) deficiency is rare. The encephalomyopathic form, described in few families, is characterized by exercise intolerance, recurrent myoglobinuria, developmental delay, ataxia, and seizures.

Objective To report a rare manifestation of CoQ10 deficiency with isolated mitochondrial myopathy without central nervous system involvement.

Methods The patient was evaluated for progressive muscle weakness. Comprehensive clinical evaluation and muscle biopsy were performed for histopathologic analysis and mitochondrial DNA and respiratory chain enzyme studies. The patient began taking 150 mg/d of a CoQ10 supplement.

Results The elevated creatine kinase and lactate levels with abnormal urine organic acid and acylcarnitine profiles in this patient suggested a mitochondrial disorder. Skeletal muscle histochemical evaluation revealed ragged red fibers, and respiratory chain enzyme analyses showed partial reductions in complex I, I + III, and II + III activities with greater than 200% of normal citrate synthase activity. The CoQ10 concentration in skeletal muscle was 46% of the normal reference mean. The in vitro addition of 50 µmol/L of coenzyme Q1 to the succinate cytochrome-c reductase assay of the patient’s skeletal muscle whole homogenate increased the succinate cytochrome-c reductase activity 8-fold compared with 2.8-fold in the normal control homogenates. Follow-up of the patient in 6 months demonstrated significant clinical improvement with normalization of creatine kinase and lactate levels.

Conclusions The absence of central nervous system involvement and recurrent myoglobinuria expands the clinical phenotype of this treatable mitochondrial disorder. The complete recovery of myopathy with exogenous CoQ10 supplementation observed in this patient highlights the importance of early identification and treatment of this genetic disorder.

Source: Isolated Mitochondrial Myopathy Associated With Muscle Coenzyme Q10 Deficiency
Seema R. Lalani, MD; Georgirene D. Vladutiu, PhD; Katie Plunkett, MS; Timothy E. Lotze, MD; Adekunle M. Adesina, MD, PhD; Fernando Scaglia, MD. Arch Neurol. 2005;62:317-320.

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