Hugues Chevassus - Academia.edu (original) (raw)
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Papers by Hugues Chevassus
Journal of the Neurological Sciences, 2015
OPA1 mutations are responsible for more than half of autosomal dominant optic atrophy (ADOA), a b... more OPA1 mutations are responsible for more than half of autosomal dominant optic atrophy (ADOA), a blinding disease affecting the retinal ganglion neurons. In most patients the clinical presentation is restricted to the optic nerve degeneration, albeit in 20% of them, additional neuro-sensorial symptoms might be associated to the loss of vision, as frequently encountered in mitochondrial diseases. This study describes clinical and neuroradiological features of OPA1 patients. Twenty two patients from 17 families with decreased visual acuity related to optic atrophy and carrying an OPA1 mutation were enrolled. Patients underwent neuro-ophthalmological examinations. Brain magnetic resonance imaging (T1, T2 and flair sequences) was performed on a 1.5-Tesla MR Unit. Twenty patients underwent 2-D proton spectroscopic imaging. Brain imaging disclosed abnormalities in 12 patients. Cerebellar atrophy mainly involving the vermis was observed in almost a quarter of the patients; other abnormalities included unspecific white matter hypersignal, hemispheric cortical atrophy, and lactate peak. Neurological examination disclosed one patient with a transient right hand motor deficit and ENT examination revealed hearing impairment in 6 patients. Patients with abnormal MRI were characterized by: (i) an older age (ii) more severe visual impairment with chronic visual acuity deterioration, and (iii) more frequent associated deafness. Our results demonstrate that brain imaging abnormalities are common in OPA1 patients, even in those with normal neurological examination. Lactate peak, cerebellar and cortical atrophies are consistent with the mitochondrial dysfunction related to OPA1 mutations and might result from widespread neuronal degeneration.
Therapie, Jan 3, 2015
Clinical studies involve an increasing amount of data collection and management. However, there i... more Clinical studies involve an increasing amount of data collection and management. However, there is no specific quality standard sufficiently practical, in free access, and open for data management and the underlying IT-infrastructure in academic units. ECRIN (European Clinical Research Infrastructures Network) published Standard requirements for certified data management units. We present a French version of these standards. A group of experts produced the standards, by consensus. The first version was revised after two pilot audits for data centre certification were performed. The revised version includes 21 lists of five to ten standards, in three groups: information technologies, data management (DM) and "general". These standards offer a clear description of DM and IT requirements for clinical studies. Initially created for ECRIN certification purposes, they offer a very useful reference for academic DM structures.
Journal of the Neurological Sciences, 2015
OPA1 mutations are responsible for more than half of autosomal dominant optic atrophy (ADOA), a b... more OPA1 mutations are responsible for more than half of autosomal dominant optic atrophy (ADOA), a blinding disease affecting the retinal ganglion neurons. In most patients the clinical presentation is restricted to the optic nerve degeneration, albeit in 20% of them, additional neuro-sensorial symptoms might be associated to the loss of vision, as frequently encountered in mitochondrial diseases. This study describes clinical and neuroradiological features of OPA1 patients. Twenty two patients from 17 families with decreased visual acuity related to optic atrophy and carrying an OPA1 mutation were enrolled. Patients underwent neuro-ophthalmological examinations. Brain magnetic resonance imaging (T1, T2 and flair sequences) was performed on a 1.5-Tesla MR Unit. Twenty patients underwent 2-D proton spectroscopic imaging. Brain imaging disclosed abnormalities in 12 patients. Cerebellar atrophy mainly involving the vermis was observed in almost a quarter of the patients; other abnormalities included unspecific white matter hypersignal, hemispheric cortical atrophy, and lactate peak. Neurological examination disclosed one patient with a transient right hand motor deficit and ENT examination revealed hearing impairment in 6 patients. Patients with abnormal MRI were characterized by: (i) an older age (ii) more severe visual impairment with chronic visual acuity deterioration, and (iii) more frequent associated deafness. Our results demonstrate that brain imaging abnormalities are common in OPA1 patients, even in those with normal neurological examination. Lactate peak, cerebellar and cortical atrophies are consistent with the mitochondrial dysfunction related to OPA1 mutations and might result from widespread neuronal degeneration.
![Research paper thumbnail of Assessment of single-dose benzodiazepines on insulin secretion, insulin sensitivity and glucose effectiveness in healthy volunteers: a double-blind, placebo-controlled, randomized cross-over trial [ISRCTN08745124]](https://attachments.academia-assets.com/53067243/thumbnails/1.jpg)
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Naunyn-Schmiedeberg's Archives of Pharmacology, 2002
Fundamental and Clinical Pharmacology, 2004
European Neuropsychopharmacology, 2013
European Journal of Clinical Pharmacology, 2009
Fenugreek seeds (Trigonella foenum-graecum L.) are an old herbal remedy used to treat metabolic a... more Fenugreek seeds (Trigonella foenum-graecum L.) are an old herbal remedy used to treat metabolic and nutritive dysfunctions. They have been shown to modulate feeding behaviour in animals, but strong clinical data are lacking. The aim of this study was to investigate the effects of a repeated administration of a fenugreek seed extract on energy intake and eating behaviour in healthy human volunteers. Twelve healthy male volunteers completed a double-blind randomized placebo-controlled three-period cross-over trial of two different doses of a fenugreek seed extract (588 and 1176 mg). The three 14-day treatment periods were separated by a 14-day washout period. The main endpoints were energy intake, assessed in volunteers under normal ambulatory and free-living conditions by a 3-day detailed dietary record and during a meal test, weight, fasting glucose level, insulin and lipid profile, visual analogue scale scores of appetite/satiety and blood glucose and insulin levels measured repeatedly after a standardized breakfast. Daily fat consumption was significantly decreased by the higher dose of fenugreek seed extract [3.73 vs. 4.51 MJ day(-1), -17.3% vs. placebo, 95% confidence interval (CI) -1.51 to -0.05, n = 12, P = 0.038]. This specific reduction tended to lower the total energy intake (9.97 vs. 11.29 MJ day(-1), -11.7% vs. placebo, 95% CI -2.91 to 0.26, n = 12, P = 0.094). No significant effect was observed on the other nutrients or other endpoints. The repeated administration of a fenugreek seed extract specifically decreases dietary fat consumption in humans which, given the traditional use of the plant, constitutes a novel result.
Diabetes Research and Clinical Practice, 2006
Diabetes & Metabolism, 2006
British Journal of Clinical Pharmacology, 2002
Journal of the Neurological Sciences, 2015
OPA1 mutations are responsible for more than half of autosomal dominant optic atrophy (ADOA), a b... more OPA1 mutations are responsible for more than half of autosomal dominant optic atrophy (ADOA), a blinding disease affecting the retinal ganglion neurons. In most patients the clinical presentation is restricted to the optic nerve degeneration, albeit in 20% of them, additional neuro-sensorial symptoms might be associated to the loss of vision, as frequently encountered in mitochondrial diseases. This study describes clinical and neuroradiological features of OPA1 patients. Twenty two patients from 17 families with decreased visual acuity related to optic atrophy and carrying an OPA1 mutation were enrolled. Patients underwent neuro-ophthalmological examinations. Brain magnetic resonance imaging (T1, T2 and flair sequences) was performed on a 1.5-Tesla MR Unit. Twenty patients underwent 2-D proton spectroscopic imaging. Brain imaging disclosed abnormalities in 12 patients. Cerebellar atrophy mainly involving the vermis was observed in almost a quarter of the patients; other abnormalities included unspecific white matter hypersignal, hemispheric cortical atrophy, and lactate peak. Neurological examination disclosed one patient with a transient right hand motor deficit and ENT examination revealed hearing impairment in 6 patients. Patients with abnormal MRI were characterized by: (i) an older age (ii) more severe visual impairment with chronic visual acuity deterioration, and (iii) more frequent associated deafness. Our results demonstrate that brain imaging abnormalities are common in OPA1 patients, even in those with normal neurological examination. Lactate peak, cerebellar and cortical atrophies are consistent with the mitochondrial dysfunction related to OPA1 mutations and might result from widespread neuronal degeneration.
Therapie, Jan 3, 2015
Clinical studies involve an increasing amount of data collection and management. However, there i... more Clinical studies involve an increasing amount of data collection and management. However, there is no specific quality standard sufficiently practical, in free access, and open for data management and the underlying IT-infrastructure in academic units. ECRIN (European Clinical Research Infrastructures Network) published Standard requirements for certified data management units. We present a French version of these standards. A group of experts produced the standards, by consensus. The first version was revised after two pilot audits for data centre certification were performed. The revised version includes 21 lists of five to ten standards, in three groups: information technologies, data management (DM) and "general". These standards offer a clear description of DM and IT requirements for clinical studies. Initially created for ECRIN certification purposes, they offer a very useful reference for academic DM structures.
Journal of the Neurological Sciences, 2015
OPA1 mutations are responsible for more than half of autosomal dominant optic atrophy (ADOA), a b... more OPA1 mutations are responsible for more than half of autosomal dominant optic atrophy (ADOA), a blinding disease affecting the retinal ganglion neurons. In most patients the clinical presentation is restricted to the optic nerve degeneration, albeit in 20% of them, additional neuro-sensorial symptoms might be associated to the loss of vision, as frequently encountered in mitochondrial diseases. This study describes clinical and neuroradiological features of OPA1 patients. Twenty two patients from 17 families with decreased visual acuity related to optic atrophy and carrying an OPA1 mutation were enrolled. Patients underwent neuro-ophthalmological examinations. Brain magnetic resonance imaging (T1, T2 and flair sequences) was performed on a 1.5-Tesla MR Unit. Twenty patients underwent 2-D proton spectroscopic imaging. Brain imaging disclosed abnormalities in 12 patients. Cerebellar atrophy mainly involving the vermis was observed in almost a quarter of the patients; other abnormalities included unspecific white matter hypersignal, hemispheric cortical atrophy, and lactate peak. Neurological examination disclosed one patient with a transient right hand motor deficit and ENT examination revealed hearing impairment in 6 patients. Patients with abnormal MRI were characterized by: (i) an older age (ii) more severe visual impairment with chronic visual acuity deterioration, and (iii) more frequent associated deafness. Our results demonstrate that brain imaging abnormalities are common in OPA1 patients, even in those with normal neurological examination. Lactate peak, cerebellar and cortical atrophies are consistent with the mitochondrial dysfunction related to OPA1 mutations and might result from widespread neuronal degeneration.
Naunyn-Schmiedeberg's Archives of Pharmacology, 2002
Fundamental and Clinical Pharmacology, 2004
European Neuropsychopharmacology, 2013
European Journal of Clinical Pharmacology, 2009
Fenugreek seeds (Trigonella foenum-graecum L.) are an old herbal remedy used to treat metabolic a... more Fenugreek seeds (Trigonella foenum-graecum L.) are an old herbal remedy used to treat metabolic and nutritive dysfunctions. They have been shown to modulate feeding behaviour in animals, but strong clinical data are lacking. The aim of this study was to investigate the effects of a repeated administration of a fenugreek seed extract on energy intake and eating behaviour in healthy human volunteers. Twelve healthy male volunteers completed a double-blind randomized placebo-controlled three-period cross-over trial of two different doses of a fenugreek seed extract (588 and 1176 mg). The three 14-day treatment periods were separated by a 14-day washout period. The main endpoints were energy intake, assessed in volunteers under normal ambulatory and free-living conditions by a 3-day detailed dietary record and during a meal test, weight, fasting glucose level, insulin and lipid profile, visual analogue scale scores of appetite/satiety and blood glucose and insulin levels measured repeatedly after a standardized breakfast. Daily fat consumption was significantly decreased by the higher dose of fenugreek seed extract [3.73 vs. 4.51 MJ day(-1), -17.3% vs. placebo, 95% confidence interval (CI) -1.51 to -0.05, n = 12, P = 0.038]. This specific reduction tended to lower the total energy intake (9.97 vs. 11.29 MJ day(-1), -11.7% vs. placebo, 95% CI -2.91 to 0.26, n = 12, P = 0.094). No significant effect was observed on the other nutrients or other endpoints. The repeated administration of a fenugreek seed extract specifically decreases dietary fat consumption in humans which, given the traditional use of the plant, constitutes a novel result.
Diabetes Research and Clinical Practice, 2006
Diabetes & Metabolism, 2006
British Journal of Clinical Pharmacology, 2002