Journal ArticleDOI
Treatment of central nervous system manifestations in mitochondrial disorders
Reads0
Chats0
TLDR
Effective treatment acting on the pathogenic cascade may increase the quality of life and outcome in patients with MID and may prevent a therapeutic nihilism occasionally upcoming with MIDs.Abstract:
Central nervous system (CNS) manifestations of mitochondrial disorders (MIDs) are accessible to therapy. Therapy of CNS abnormalities may be categorized as acting on the pathogenic cascade or on the genetic level, which is experimental. Treatment acting on the pathogenic cascade may be classified as non-specific, including antioxidants, electron donors/acceptors, lactate-lowering agents, alternative energy providers, cofactors, avoidance of mitochondrion-toxic drugs, and physiotherapy, or as specific, including drugs against epilepsy, movement disorders, migraine, spasticity, psychiatric abnormalities, hypopituitarism, or bulbar manifestations, ketogenic diet, deep brain stimulation, or artificial ventilation. Stroke-like episodes need to be delineated from ischaemic stroke and require special management. Potentially, mitochondrion-toxic drugs and drug cocktails need to be avoided, seizures should be consequently treated even with mitochondrion-toxic drugs if necessary, and as few drugs as possible should be given. Effective treatment acting on the pathogenic cascade may increase the quality of life and outcome in patients with MID and may prevent a therapeutic nihilism occasionally upcoming with MIDs.read more
Citations
More filters
Journal Article
Resistance training in patients with single, large-scale deletions of mitochondrial DNA. Commentary
Massimo Zeviani,J.L. Murphy,Emma L. Blakely,Andrew M. Schaefer,Langping He,Phil Wyrick,Ronald G. Haller,Robert W. Taylor,Douglass M. Turnbull,Tanja Taivassalo +9 more
TL;DR: The hypothesis of resistance exercise-induced mitochondrial gene-shifting in muscle containing satellite cells which have low or absent levels of deleted mtDNA is supported.
Journal ArticleDOI
Mitochondrial DNA shifting in older adults following resistance exercise training.
TL;DR: Preliminary data strongly suggest that mitochondrial DNA shifting occurs in skeletal muscle from older adults following resistance-exercise training.
Journal ArticleDOI
Melatonin and neurotrophins NT-3, BDNF, NGF in patients with varying levels of depression severity.
TL;DR: Melatonin and NF in depressed patients show neuroprotective effects andMelatonin may be a significant marker of depression severity.
Journal ArticleDOI
Mitochondrial diseases caused by mtDNA mutations: a mini-review.
A.I. Ryzhkova,Margarita A. Sazonova,Vasily V. Sinyov,Elena V. Galitsyna,Mariуa M Chicheva,Alexandra A. Melnichenko,Andrey V. Grechko,Anton Y. Postnov,Alexander N. Orekhov,Tatiana P. Shkurat +9 more
TL;DR: For every individual, more effective therapeutic approach could be developed after wide-range mutant background analysis of mitochondrial genome, and data about mutations linked with cytopathies that facilitate diagnosis of different syndromes by using genetic analysis methods are contained.
Journal ArticleDOI
Drugs and mitochondrial diseases: 40 queries and answers
Michelangelo Mancuso,Daniele Orsucci,Massimiliano Filosto,Costanza Simoncini,Gabriele Siciliano +4 more
TL;DR: To critically review this still unclear field, this paper attempts to answer the ‘frequently asked questions', such as: Is valproic acid safe in mitochondrial patients?
References
More filters
Journal ArticleDOI
Mitochondrial Disorders in the Nervous System
Salvatore DiMauro,Eric A. Schon +1 more
TL;DR: Altered mitochondrial dynamics in the etiology of specific neurological diseases and in the physiopathology of more common neurodegenerative disorders are reviewed.
Journal ArticleDOI
Deleterious Mutation in the Mitochondrial Arginyl-Transfer RNA Synthetase Gene Is Associated with Pontocerebellar Hypoplasia
Simon Edvardson,Avraham Shaag,Olga Kolesnikova,John M. Gomori,Ivan Tarassov,Tom Einbinder,Ann Saada,Orly Elpeleg +7 more
TL;DR: It is speculated that missplicing mutations in mitochondrial aminoacyl-tRNA synthethase genes preferentially affect the brain because of a tissue-specific vulnerability of the splicing machinery.
Journal ArticleDOI
Dichloroacetate causes toxic neuropathy in MELAS: a randomized, controlled clinical trial.
Petra Kaufmann,Kristin Engelstad,Ying Wei,Sarah Jhung,Mary Sano,Dikoma C. Shungu,W. S. Millar,X. Hong,Clifton L. Gooch,Xiangling Mao,Juan M. Pascual,Michio Hirano,Peter W. Stacpoole,Salvatore DiMauro,Darryl C. De Vivo +14 more
TL;DR: The findings show that DCA-associated neuropathy overshadows the assessment of any potential benefit in MELAS, and DCA at 25 mg/kg/day is associated with peripheral nerve toxicity resulting in a high rate of medication discontinuation and early study termination.
Journal ArticleDOI
l-Arginine improves the symptoms of strokelike episodes in MELAS
Yasutoshi Koga,Yukihiro Akita,Junko Nishioka,Shuichi Yatsuga,Nataliya Povalko,Yuzo Tanabe,S. Fujimoto,Toyojiro Matsuishi +7 more
TL;DR: L-Arginine infusions significantly improved all strokelike symptoms, suggesting that oral administration within 30 minutes of a stroke significantly decreased frequency and severity of strokelike episodes.
Journal ArticleDOI
Beneficial effects of creatine, CoQ10, and lipoic acid in mitochondrial disorders.
M. Christine Rodriguez,J. R. MacDonald,Douglas J. Mahoney,Gianni Parise,M. Flint Beal,Mark A. Tarnopolsky +5 more
TL;DR: The results suggest that combination therapies targeting multiple final common pathways of mitochondrial dysfunction favorably influence surrogate markers of cellular energy dysfunction, and future studies with larger sample sizes in relatively homogeneous groups will be required.
Related Papers (5)
Anesthetic considerations in patients with mitochondrial defects.
Julie Niezgoda,Phil G. Morgan +1 more