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Mitchondrial disease

J Finsterer
The central nervous system (CNS) is the second most frequently affected organ in mitochondrial disorders (MD). In the majority of the cases, clinical CNS manifestations correlate with the CNS lesions on imaging and vice versa. The most important method to demonstrate CNS abnormalities in MD is the MRI. This imaging technique can demonstrate structural lesions, such as strokelike lesions, focal or diffuse atrophy, cysts or lacunas, focal or diffuse white matter lesions, grey matter lesions of the cortex or deep grey matter, and increased iron deposition...
June 2009: Der Nervenarzt
M Gassen, Y Glinka, B Pinchasi, M B Youdim
Ergoline-derived dopamine receptor agonists, like pergolide or bromocryptine, have recently attracted attention as potential neuroprotective drugs. The classical mixed type dopamine D1 and D2 receptor agonist apomorphine, although used clinically in the therapy of Parkinson's disease, has never been examined for any properties related to neuroprotection. In this paper, we examine the effects of 0.1-100 microM apomorphine on ascorbate/iron-stimulated free radical processes in rat brain mitchondrial fraction...
July 18, 1996: European Journal of Pharmacology
K Kurahashi, T Andoh, K Sato, S Yamazaki, F Okumura
A patient with carnitine deficiency syndrome for tracheostomy and resection of tracheal granuloma was anesthetized with nitrous oxide and sevoflurane and vecuronium bromide. Potentiation of effect of vecuronium was minimal, and anesthetic course was uneventful. Carnitine is essential for transportation of long chain fatty acids into mitchondrial matrix. Its deficiency causes metabolic failure in skeletal muscle and accumulation of acyl-CoA in central nervous system. Carnitine deficiency syndrome is associated with a number of metabolic disorders of fatty acid...
August 1993: Masui. the Japanese Journal of Anesthesiology
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