Effect of vitamin B6 supplementation in McArdle's disease: a strategic case study

Phoenix, Joanne; Hopkins, Pam; Bartram, Clare; Beynon, Robert J.; Quinlivan, R.; Edwards, R. H. T.

doi:10.1016/s0960-8966(98)00004-2

Cited by 53 publications

(19 citation statements)

References 6 publications

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“…Therefore, supplementation of vitamin B6 has been hypothesized to activate the residual phosphorylase activity. The effect of vitamin B6 (oral 50 mg/day supplementation for two years) in a McArdle disease patient was reported by Phoenix et al in 1998 in a patient-blind study (10). But thereafter there have been few reports suggesting the efficacy of vitamin B6.…”

Section: Discussionmentioning

confidence: 90%

A Case of McArdle Disease: Efficacy of Vitamin B6 on Fatigability and Impaired Glycogenolysis

et al. 2010

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McArdle disease is a glycogenetic myopathy caused by a deficit of myophosphorylase inherited in an autosomal recessive pattern. Here, we report a case of McArdle disease in which fatigability was the only subjective complaint. Objective neurological findings were normal except for very mild muscle weakness in limbs and an elevated serum creatine kinase level. Ischemic forearm exercise test showed deficient glycogenolysis. In the muscle biopsy specimen, periodic acid Schiff (PAS) stained subsarcolemmal glycogen was increased and the muscle phosphorylase A activity was decreased. After administration of vitamin B6, fatigability was diminished and ischemic forearm exercise test showed improved glycogenolysis. Vitamin B6 may be beneficial for McArdle disease, especially for its easy fatigability.

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Section: Discussionmentioning

confidence: 90%

A Case of McArdle Disease: Efficacy of Vitamin B6 on Fatigability and Impaired Glycogenolysis

et al. 2010

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“…So Vit B 6 is considered to be associated with energy metabolism. It has been reported that Vit B 6 plays an active part in promoting amino acid metabolism and recovery of peripheral nerve 23) . In other paper, administration of Vit B6 can enhance the utilization of selenium, increase glutathione peroxidase (GSH-Px) activity in erythrocyte, skeletal muscle, myocardium and testis 24) , and decrease lipid peroxides levels 25) .…”

Section: Discussionmentioning

confidence: 99%

Effect of Energy Compound on Skeletal Muscle Strain Injury and Regeneration in Rats

Feng

et al. 2008

INDUSTRIAL HEALTH

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This study was designed to determine whether the supplement of energy compound could attenuate strain-induced damage to skeletal muscle in rats. Energy compound is a saline mixture of the following ingredients: ATP (10 mg), Coenzyme-A (50 units), Coenzyme-Q 10 (50 mg), Cytochrome C (30 mg) and Vitamin B 6 (50 mg). Experimental animals were injured in right gastrocnemius muscles by a strain injury model. Energy compound groups were given energy compound 10 ml/kg body weight per day since injured, while saline groups were given saline at the same dose. And a sham operation was performed on the right hindlimb of control group. Plasma was centrifuged to measure lactate dehydrogenase (LDH), lactic acid (La) and creatine kinase (CK) on 3, 7 and 14 d post injury. Muscles were removed and fixed for histology observation and immunohistochemistry assay of desmin and vimentin. The results showed a similar tendency of plasma CK, La and LDH in saline and energy compound groups, while the lower level was found in the energy-compound group. The histological examination of muscle sections revealed a lower degree of damage in the energy compound group in which the expression levels of desmin and vimentin were higher than in the saline group. It is suggested that energy compound supplement may attenuate strain-induced muscle damage and facilitate its regeneration.

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“…Vitamin B 6 has also been used because overall body stores of pirydoxal phosphate are depleted in McArdle's disease patients. A beneficial effect has been documented in one patient but this requires confirmation [50]. A placebocontrolled, crossover trial of creatine monohydrate in nine patients showed improvement in symptoms and increases in their capacity for ischemic, isometric forearm exercise [51].…”

Section: Managementmentioning

confidence: 97%

Molecular genetics of McArdle’s disease

Nogales‐Gadea¹,

Arenas²,

Andreu

2007

Curr Neurol Neurosci Rep

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This review highlights recent advances in our understanding of McArdle's disease, including the mechanisms involved in the regulation of the clinical phenotype. The latest molecular genetic studies have demonstrated the genetic heterogeneity of the disorder, with more than 65 mutations identified to date. There is not a specific treatment for McArdle's disease, but some nutritional treatments in combination with aerobic conditioning could improve the quality of life in most patients.

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Effect of vitamin B6 supplementation in McArdle's disease: a strategic case study

Cited by 53 publications

References 6 publications

A Case of McArdle Disease: Efficacy of Vitamin B6 on Fatigability and Impaired Glycogenolysis

A Case of McArdle Disease: Efficacy of Vitamin B6 on Fatigability and Impaired Glycogenolysis

Effect of Energy Compound on Skeletal Muscle Strain Injury and Regeneration in Rats

Molecular genetics of McArdle’s disease

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