Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

A novel nutritional management regimen for very long-chain acyl-CoA dehydrogenase deficiency

Hiroyo Haruki, M.D., Motoharu Kawai, M.D., Ph.D., Jun-ichi Ogasawara, M.D., Ph.D., Michiaki Koga, M.D., Ph.D., Kiyoshi Negoro, M.D., Ph.D. and Takashi Kanda, M.D., Ph.D.

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine

We report a novel regimen of nutritional management in 22-year-old woman with myopathic form of very-long-chain acyl-CoA dehydrogenase deficiency. This regimen is based on avoidance of fasting by frequent intake of carbohydrates and substitution of medium chain triglyceride for long- and very long-chain fatty acids. Oral intake of medium amount of long-chain fatty acid (300 kcal daily) was allowed, to facilitate compliance and to escape pigmentary retinopathy. After this nutritional management and lifestyle guidance about prevention of fatigue and starvation, the patient was free from severe rhabdomyolysis for more than three years, which had forced her to hospital management nine times in seven years.
Full Text of this Article in Japanese PDF (326K)

(CLINICA NEUROL, 50: 172|174, 2010)
key words: very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, nutritional management, medium chain triglyceride (MCT) oil, recurrent rhabdomyolysis

(Received: 10-Sep-09)