The Experts below are selected from a list of 60 Experts worldwide ranked by ideXlab platform
Jan O Aasly - One of the best experts on this subject based on the ideXlab platform.
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proton magnetic resonance spectroscopy of cerebrospinal fluid in neurodegenerative disease indication of glial energy impairment in Huntington Chorea but not parkinson disease
Journal of Neuroscience Research, 2000Co-Authors: Mari Garseth, Ursula Sonnewald, Linda R White, Marianne Rod, Johnanker Zwart, Oystein P Nygaard, Jan O AaslyAbstract:Metabolite levels in cerebrospinal fluid from patients with Parkinson disease or Huntington Chorea were compared with the levels in healthy controls using proton magnetic resonance spectroscopy. No significant differences were found for any metabolite measured in cerebrospinal fluid from patients with Parkinson disease compared to controls. Slight but significantly reduced levels of both lactate and citrate, however, were found in cerebrospinal fluid from patients with Huntington Chorea compared to controls. This suggests possible impairment of both glycolysis and tricarboxylic acid cycle function. The reduction in lactate found in the present study may reflect neuronal loss. The decrease in citrate supports the theory of mitochondrial dysfunction in the brain of patients with Huntington Chorea, but also suggests that there may be an important astrocytic component in this disease. If so, it would certainly have implications for neuronal function. J. Neurosci. Res. 60:779–782, 2000. © 2000 Wiley-Liss, Inc.
Marco Trabucchi - One of the best experts on this subject based on the ideXlab platform.
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Treatment of Huntington's Chorea with bromocriptine.
Acta neurologica Scandinavica, 2009Co-Authors: L. Frattola, Pierfranco Spano, Marco TrabucchiAbstract:The authors tested the effects of 2-Br-ergocriptine (bromocriptine, CB-154), a drug which exerts a mixed agonist-antagonist activity on the dopaminergic receptors, in 12 patients with Huntington Chorea in a double-blind crossover trial. This treatment significantly reduced the abnormal involuntary movements and the disease severity in most of the patients. Subjects who were slightly disabled showed a better response than the ones with more severe degrees of disability.
Mari Garseth - One of the best experts on this subject based on the ideXlab platform.
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proton magnetic resonance spectroscopy of cerebrospinal fluid in neurodegenerative disease indication of glial energy impairment in Huntington Chorea but not parkinson disease
Journal of Neuroscience Research, 2000Co-Authors: Mari Garseth, Ursula Sonnewald, Linda R White, Marianne Rod, Johnanker Zwart, Oystein P Nygaard, Jan O AaslyAbstract:Metabolite levels in cerebrospinal fluid from patients with Parkinson disease or Huntington Chorea were compared with the levels in healthy controls using proton magnetic resonance spectroscopy. No significant differences were found for any metabolite measured in cerebrospinal fluid from patients with Parkinson disease compared to controls. Slight but significantly reduced levels of both lactate and citrate, however, were found in cerebrospinal fluid from patients with Huntington Chorea compared to controls. This suggests possible impairment of both glycolysis and tricarboxylic acid cycle function. The reduction in lactate found in the present study may reflect neuronal loss. The decrease in citrate supports the theory of mitochondrial dysfunction in the brain of patients with Huntington Chorea, but also suggests that there may be an important astrocytic component in this disease. If so, it would certainly have implications for neuronal function. J. Neurosci. Res. 60:779–782, 2000. © 2000 Wiley-Liss, Inc.
L. Frattola - One of the best experts on this subject based on the ideXlab platform.
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Treatment of Huntington's Chorea with bromocriptine.
Acta neurologica Scandinavica, 2009Co-Authors: L. Frattola, Pierfranco Spano, Marco TrabucchiAbstract:The authors tested the effects of 2-Br-ergocriptine (bromocriptine, CB-154), a drug which exerts a mixed agonist-antagonist activity on the dopaminergic receptors, in 12 patients with Huntington Chorea in a double-blind crossover trial. This treatment significantly reduced the abnormal involuntary movements and the disease severity in most of the patients. Subjects who were slightly disabled showed a better response than the ones with more severe degrees of disability.
S Todorovic - One of the best experts on this subject based on the ideXlab platform.
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is the 31 cag repeat allele of the spinocerebellar ataxia 1 sca1 gene locus non specifically associated with trinucleotide expansion diseases
Psychiatric Genetics, 2001Co-Authors: D Savic, Ivan Topisirovic, Milica Keckarevic, Dusan Keckarevic, Tamara Major, Biljana Culjkovic, Oliver Stojkovic, Vidosava Rakocevicstojanovic, Jelena Mladenovic, S TodorovicAbstract:A number of human hereditary neuromuscular and neurodegenerative disorders are caused by the expansion of trinucleotide repeats within certain genes. The molecular mechanisms that underlie these expansions are not yet known. We have analyzed six trinucleotide repeat-containing loci [spinocerebellar ataxias (SCA1, SCA3, SCA8), dentatorubral-pallidoluysian atrophy (DRPLA), Huntington Chorea (HD) and fragile X syndrome (FRAXA)] in myotonic dystrophy type 1 (DM1) patients (n = 52). As controls, we analyzed two groups of subjects: healthy control subjects (n =133), and a group of patients with non-triplet neuromuscular diseases (n = 68) caused by point mutations, deletions or duplications (spinal muscular atrophy, Charcot-Marie-Tooth disease, type 1A, hereditary neuropathy with liability to pressure palsies, and Duchenne and Becker muscular dystrophy). Allele frequency distributions for all tested loci were similar in these three groups with the exception of the SCA1 locus. In DM1 patients, the SCA1 allele with 31 CAG repeats account for 40.4% of all chromosomes tested, which is significantly higher than in two other groups (11.3% in healthy controls and 6.6% in the group of non-triplet diseased patients; P < 0.001, Fisher's exact test). This is consistent with our previous findings in HD patients. The absence of this association in non-triplet diseases as well as in healthy controls could indicate a possible role of this SCA1 allele with 31 repeats in triplet diseases. Here we discuss a possible role of the SCA1 region in pathological trinucleotide repeat expansions.