Progression despite replacement of a myopathic form of coenzyme Q10 defect
K Aure, JF Benoist, HO De Baulny, NB Romero… - Neurology, 2004 - AAN Enterprises
K Aure, JF Benoist, HO De Baulny, NB Romero, O Rigal, A Lombes
Neurology, 2004•AAN EnterprisesThe authors report 7 years of follow-up evaluation of a patient with coenzyme Q10 (CoQ10)
deficiency. Initial symptoms of exercise intolerance and hyperlactatemia improved markedly
with substitutive treatment. However, CoQ10 supplementation did not prevent the onset of a
cerebellar syndrome. A switch to idebenone treatment resulted in clinical and metabolic
worsening, which disappeared with subsequent CoQ10 treatment. CoQ10 defects may
cause progressive neurologic disease despite supplementation.
deficiency. Initial symptoms of exercise intolerance and hyperlactatemia improved markedly
with substitutive treatment. However, CoQ10 supplementation did not prevent the onset of a
cerebellar syndrome. A switch to idebenone treatment resulted in clinical and metabolic
worsening, which disappeared with subsequent CoQ10 treatment. CoQ10 defects may
cause progressive neurologic disease despite supplementation.
The authors report 7 years of follow-up evaluation of a patient with coenzyme Q10 (CoQ10) deficiency. Initial symptoms of exercise intolerance and hyperlactatemia improved markedly with substitutive treatment. However, CoQ10 supplementation did not prevent the onset of a cerebellar syndrome. A switch to idebenone treatment resulted in clinical and metabolic worsening, which disappeared with subsequent CoQ10 treatment. CoQ10 defects may cause progressive neurologic disease despite supplementation.
American Academy of Neurology