Jean-Philippe Camdessanché

Lyon Neuroscience Research Center, Lyons, Rhône-Alpes, France

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Publications (40)130 Total impact

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    ABSTRACT: Background: Age-related white matter hyperintensities are frequent incidental findings on T2-weighted brain MRI, and they are evaluated in clinical practice using a visual rating scale. Objective: To evaluate inter- and intra-rater agreement in MRI visual evaluations of age-related white matter hyperintensities made by two radiologists with different levels of experience using a visual rating scale. Methods: Two radiologists of two different levels of experience separately rated age-related white matter hyperintensities in 40 consecutive 3-tesla brain MRI scans using the Fazekas and Schmidt visual rating scale. Ratings were made on axial FLAIR (fluid-attenuated inversion recovery) sequences. Two readings were made by each radiologist. Intra- and inter-rater agreement was statistically determined by using Cohen's weighted kappa analysis. Results: Forty patients (21 females, 19 males; mean age = 57 ± 18.43 years) were included between September and October 2011. Mean values ± SD for visual scores were as follows: periventricular hyperintensities, between 1.175 ± 0.9 and 1.375 ± 0.89; number of deep white matter hyperintensity lesions, between 1.325 ± 1.18 and 1.575 ± 1.15, and extent of deep white matter hyperintensity lesions, between 0.925 ± 0.78 and 1.1 ± 0.74. Intra- and inter-rater agreement was very good (x03BA; values, 0.85-0.91 and 0.80-0.97, respectively) for each of the three visual scale criteria, with significant correlations between ratings (r = 0.95; p < 0.0001) and readings (r = 0.91; p < 0.0001). Conclusion: Visual assessment of age-related white matter hyperintensities by radiologists using a visual scale on FLAIR sequence is reproducible. Differences in experience level do not influence readings. Visual scale use is thus justified in common practice.
    No preview · Article · Dec 2015 · Neurodegenerative Diseases
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    ABSTRACT: Patients with inflammatory sensory neuronopathy (SNN) may benefit from immunomodulatory or immunosuppressant treatments if administered timely. Knowing the temporal profile of neuronal loss in dorsal root ganglia will help to ascertain whether a final diagnosis may be reached before the occurrence of irreversible neuronal injuries. Thus, we addressed the evolution of neuronal loss in SNN by using sensory nerve action potentials (SNAPs) as a surrogate marker of neuron degeneration.
    No preview · Article · Dec 2015

  • No preview · Article · Sep 2015 · Neurology
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    ABSTRACT: Recent studies in multiple sclerosis (MS) showed longer survival times from clinical onset than older hospital-based series. However estimated median time ranges widely, from 24 to 45 years, which makes huge difference for patients as this neurological disease mainly starts around age 20 to 40. Precise and up-to-date reference data about mortality in MS are crucial for patients and neurologists, but unavailable yet in France. Estimate survival in MS patients and compare mortality with that of the French general population. We conducted a multicenter observational study involving clinical longitudinal data from 30,413 eligible patients, linked to the national deaths register. Inclusion criteria were definite MS diagnosis and clinical onset prior to January, 1st 2009 in order to get a minimum of 1-year disease duration. After removing between-center duplicates and applying inclusion criteria, the final population comprised 27,603 MS patients (F/M sex ratio 2.5, mean age at onset 33.0 years, 85.5% relapsing onset). During the follow-up period (mean 15.2 +/- 10.3 years), 1569 deaths (5.7%) were identified; half related to MS. Death rates were significantly higher in men, patients with later clinical onset, and in progressive MS. Overall excess mortality compared with the general population was moderate (Standardized Mortality Ratio 1.48, 95% confidence interval [1.41-1.55]), but increased considerably after 20 years of disease (2.20 [2.10-2.31]). This study revealed a moderate decrease in life expectancy in MS patients, and showed that the risk of dying is strongly correlated to disease duration and disability, highlighting the need for early actions that can slow disability progression.
    Full-text · Article · Jul 2015 · PLoS ONE
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    ABSTRACT: Auteur correspondant. CHU de Saint-Étienne, Neurologie, avenue Albert-Raymond, 42270 Saint-Priest-en-Jarez, France.
    No preview · Article · Apr 2015 · Revue Neurologique
  • Sophie Jeannin Mayer · Claire Boutet · Yanis Medniek · Marie-France Lucht · Jean-Philippe Camdessanche

    No preview · Article · Apr 2015 · Revue Neurologique
  • Emilie Sala · Geneviève Demarquay · Jean-Philippe Camdessanché · Laure Mazzola · Philippe Convers · Marc Guenot
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    ABSTRACT: Auteur correspondant. Hôpital Nord, CHU de Saint-Étienne, Neurologie, avenue Albert-Raymond, 42270 Saint-Priest-en-Jarez, France.
    No preview · Article · Apr 2015 · Revue Neurologique
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    ABSTRACT: Background Immunological mechanisms are suspected in sensory neuropathy (SN) occurring with systemic autoimmune diseases and in some idiopathic cases, but so far there are no antibodies (Abs) identifying these neuropathies. Methods In the search for such specific antibodies, serum samples were collected from 106 patients with SN of these 72 fulfilled the diagnosis criteria of sensory neuronopathy (SNN) and 211 control subjects including patients with sensorimotor neuropathies, other neurological diseases (ONDs), systemic autoimmune diseases and healthy blood donors. Results In the first step, a protein array with 8000 human proteins allowed identification of the intracellular domain of the fibroblast growth factor receptor 3 (FGFR3) as a target of Abs in 7/16 SNN and 0/30 controls. In the second step, an ELISA method was used to test the 317 patients and controls for anti-FGFR3 Abs. Abs were detected in 16/106 patients with SN and 1/211 controls (p<0.001). Among the 106 patients with SN, anti-FGFR3 Abs were found in 11/38 patients with autoimmune context, 5/46 with idiopathic neuropathy and 0/22 with neuropathy of other aetiology (p=0.006). The only control patient with anti-FGFR3 Abs had lupus and no recorded neuropathy. Sensitivity, specificity, and positive and negative predictive values of anti-FGFR3 Abs for a diagnosis of idiopathic or dysimmune SN were 19%, 99.6%, 94.1% and 77.3%, respectively. A cell-based assay confirmed serum reactivity against the intracellular domain of FGFR3. The neuropathy in patients with anti-FGF3 Abs was non-length dependent in 87% of patients and fulfilled the criteria of probable SNN in 82%. Trigeminal nerve involvement and pain were frequent features. Conclusions A anti-FGFR3 Abs identify a subgroup of patients with SN in whom an underlying autoimmune disorder affecting sensory neurons in the dorsal root and trigeminal nerve ganglia is suspected.
    No preview · Article · Jan 2015 · Journal of Neurology Neurosurgery & Psychiatry
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    ABSTRACT: Inherited white matter diseases are rare and heterogeneous disorders usually encountered in infancy. Adult-onset forms are increasingly recognized. Our objectives were to determine relative frequencies of genetic leukoencephalopathies in a cohort of adult-onset patients and to evaluate the effectiveness of a systematic diagnostic approach. Inclusion criteria of this retrospective study were: (i) symmetrical involvement of white matter on the first available brain MRI; (ii) age of onset above 16 years. Patients with acquired diseases were excluded. Magnetic resonance imaging analysis identified three groups (vascular, cavitary and non-vascular/non-cavitary) in which distinct genetic and/or biochemical testing were realized. One hundred and fifty-four patients (male/female = 60/94) with adult-onset leukoencephalopathies were identified. Mean age of onset was 38.6 years. In the vascular group, 41/55 patients (75%) finally had a diagnosis [including CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy, n = 32) and COL4A1 mutation, n = 7]. In the cavitary group, 13/17 (76%) patients had a diagnosis of EIF2B-related disorder. In the third group (n = 82), a systematic biological screening allowed a diagnosis in 23 patients (28%) and oriented direct genetic screening identified 21 additional diseases (25.6%). Adult-onset genetic leukoencephalopathies are a rare but probably underestimated entity. Our study confirms the use of a magnetic resonance imaging-based classification with a final diagnosis rate of 64% (98/154) cases. © The Author (2014). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.
    Full-text · Article · Dec 2014 · Brain
  • Jean-Philippe Camdessanché · Jean-Christophe Antoine
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    ABSTRACT: Sensory neuronopathy (SNN) depends on a pathophysiological process that targets the sensory neuron in the posterior root ganglia. These rare diseases are sometimes difficult to diagnose because the site of impairment is not directly assessable by conventional neurophysiological techniques. After recalling the general data concerning SNN, we propose an easy to use clinical and electrophysiological diagnosis criteria and guidelines for clinicians in their search for an etiology in a patient with NNS.
    No preview · Article · Nov 2014 · La Presse Médicale
  • Philippe Corcia · Paul H. Gordon · Jean-Philippe Camdessanche
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    ABSTRACT: Our objective was to examine the strength of evidence in support of the paraneoplastic syndrome (PNS) as one cause of ALS and, if the association appears more likely than chance, determine which features of ALS imply concurrent malignancy. We reviewed the literature on concurrent ALS and neoplasia assessing the strength of evidence for the association. Most accounts of ALS and neoplasm are case reports or small uncontrolled series. In order of strength of evidence, three clinical situations that support a paraneoplastic aetiology for ALS are: 1) laboratory evidence of well-characterized onconeuronal antibodies, most often anti-Hu, anti-Yo or anti-Ri; 2) co-occurrence of ALS and a neoplasm known to cause PNS, usually lymphoma or cancer of the breast; and 3) combined ALS and a neoplasm not classically associated with PNS, without detectable onconeuronal antibodies. Clinical features that warrant evaluation of neoplasm include upper motor neuron disease in elderly females, rapid progression, non-motor signs, and young onset. In conclusion, most examples of ALS and neoplasm do not constitute a classically established PNS. Rare instances of elevated onconeuronal antibody titres or typical neoplasm, implies that, albeit rare, the PNS is one of a multitude of causes of ALS.
    No preview · Article · Oct 2014 · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
  • Jean-Philippe Camdessanché · Jean-Christophe Antoine
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    ABSTRACT: Sensory neuronopathy (SNN) depends on a pathophysiological process that targets the sensory neuron in the posterior root ganglia. These rare diseases are sometimes difficult to diagnose because the site of impairment is not directly assessable by conventional neurophysiological techniques. After recalling the general data concerning SNN, we propose an easy to use clinical and electrophysiological diagnosis criteria and guidelines for clinicians in their search for an etiology in a patient with NNS.
    No preview · Article · Sep 2014
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    ABSTRACT: There are no validated criteria for the diagnosis of sensory neuronopathy (SNN) yet. In a preliminary monocenter study a set of criteria relying on clinical and electrophysiological data showed good sensitivity and specificity for a diagnosis of probable SNN. The aim of this study was to test these criteria on a French multicenter study. 210 patients with sensory neuropathies from 15 francophone reference centers for neuromuscular diseases were included in the study with an expert diagnosis of non-SNN, SNN or suspected SNN according to the investigations performed in these centers. Diagnosis was obtained independently from the set of criteria to be tested. The expert diagnosis was taken as the reference against which the proposed SNN criteria were tested. The set relied on clinical and electrophysiological data easily obtainable with routine investigations. 9/61 (16.4 %) of non-SNN patients, 23/36 (63.9 %) of suspected SNN, and 102/113 (90.3 %) of SNN patients according to the expert diagnosis were classified as SNN by the criteria. The SNN criteria tested against the expert diagnosis in the SNN and non-SNN groups had 90.3 % (102/113) sensitivity, 85.2 % (52/61) specificity, 91.9 % (102/111) positive predictive value, and 82.5 % (52/63) negative predictive value. Discordance between the expert diagnosis and the SNN criteria occurred in 20 cases. After analysis of these cases, 11 could be reallocated to a correct diagnosis in accordance with the SNN criteria. The proposed criteria may be useful for the diagnosis of probable SNN in patients with sensory neuropathy. They can be reached with simple clinical and paraclinical investigations.
    No preview · Article · Aug 2014 · Journal of Neurology
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    ABSTRACT: Background: Patients with peripheral neuropathy and anti-MAG monoclonal IgM may respond to Rituximab, a humanized monoclonal anti-CD20 antibody. Methods: We report on three patients with peripheral neuropathy and anti-MAG monoclonal IgM who deteriorated under Rituximab and reviewed seven previously published cases. Results: Worsening was acute and severe, and occurred during the treatment period. All the patients improved after deterioration but at final evaluation only one was improved comparatively to baseline, five were worsened and four were stabilized. Deterioration was not clearly associated with an increase of the anti-MAG antibody titer. Two patients received Rituximab prior or after the course which induced worsening without adverse reaction. Conclusion: Although rare, acute worsening of the neuropathy can occur after Rituximab. The deterioration is however reversible within some weeks to several months.
    No preview · Article · Aug 2014 · Journal of the Neurological Sciences
  • Jean-Philippe Camdessanché · Timothée Lenglet
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    ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a degenerative disease which prognosis is poor. Early diagnosis permits to set up immediately adapted treatment and cares. Available diagnostic criteria are based on the detection of both the central and peripheral motor neuron injury in bulbar, cervical, thoracic and lumbar regions. Electromyographic study is the key tool to identify peripheral motor neuron involvement. Conduction velocities are systematically performed to rule out differential diagnosis. Needle examination records abnormal activities at rest and looks for neurogenic pattern during muscle contraction. Motor unit potentials morphology is modified primary to recruitment. Motor evoked potentials remain the test of choice to identify impairment of central motor neurons. For the monitoring of ALS patients, the MUNE technique (motor unit number estimation) seems the most interesting.
    No preview · Article · May 2014 · La Presse Médicale
  • Jean-Philippe Camdessanché · Timothée Lenglet
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    ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a degenerative disease which prognosis is poor. Early diagnosis permits to set up immediately adapted treatment and cares. Available diagnostic criteria are based on the detection of both the central and peripheral motor neuron injury in bulbar, cervical, thoracic and lumbar regions. Electromyographic study is the key tool to identify peripheral motor neuron involvement. Conduction velocities are systematically performed to rule out differential diagnosis. Needle examination records abnormal activities at rest and looks for neurogenic pattern during muscle contraction. Motor unit potentials morphology is modified primary to recruitment. Motor evoked potentials remain the test of choice to identify impairment of central motor neurons. For the monitoring of ALS patients, the MUNE technique (motor unit number estimation) seems the most interesting.
    No preview · Article · Apr 2014 · La Presse Médicale
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    ABSTRACT: Objective: To test the influence of functional cerebral reorganization in amyotrophic lateral sclerosis (ALS) on disease progression. Methods: Nineteen predominantly right-handed ALS patients and 21 controls underwent clinical evaluation, functional Magnetic Resonance Imaging (fMRI), and diffusion tensor imaging. Patients were clinically re-evaluated 1 year later and followed until death. For fMRI, subjects executed and imagined a simple hand-motor task. Between-group comparisons were performed, and correlations were searched with motor deficit arm Medical Research Council (MRC) score, disease progression ALS Functional Rating Scale (ALSFRS), and survival time. Results: By the MRC score, the hand strength was lowered by 12% in the ALS group predominating on the right side in accordance with an abnormal fractional anisotropy (FA) limited to the left corticospinal tract (37.3% reduction vs. controls P < 0.01). Compared to controls, patients displayed overactivations in the controlateral parietal (P < 0.004) and somatosensory (P < 0.004) cortex and in the ipsilateral parietal (P < 0.01) and somatosensory (P < 0.01) cortex to right-hand movement. Movement imagination gave similar results while no difference occurred with left-hand tasks. Stepwise regression analysis corrected for multiple comparisons showed that controlateral parietal activity was inversely correlated with disease progression (R(2) = 0.43, P = 0.001) and ipsilateral somatosensory activations with the severity of the right-arm deficit (R(2) = 0.48, P = 0.001). Conclusions: Cortical Blood Oxygen Level Dependent (BOLD) signal changes occur in the brain of ALS patients during a simple hand-motor task when the motor deficit is still moderate. It is correlated with the rate of disease progression suggesting that brain functional rearrangement in ALS may have prognostic implications. Hum Brain Mapp, 2012. © 2012 Wiley Periodicals, Inc.
    No preview · Article · Oct 2013 · Human Brain Mapping
  • Jean-Christophe Antoine · Jean-Philippe Camdessanché
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    ABSTRACT: Paraneoplastic neurological syndromes are rare but can affect any part of the peripheral nervous system (PNS) including motor neurons, sensory ganglia, nerve roots, plexuses, cranial and peripheral nerves, and neuromuscular junctions. The type of cancer, lymphoma or solid tumour, is a determinant factor of the underlying mechanism. With solid tumour, antibodies directed to intracellular (anti-Hu or anti-CV2/CRMP5 antibodies) or surface antigens (anti-VGCC,or LGI1 and Caspr2 antibodies) have been identified while with lymphoma, the neuropathy is usually linked to a monoclonal gammopathy. This review discusses the different etiologies and mechanisms of paraneoplastic disorders of the PNS in patients emphasising their evaluation, diagnosis and treatment.
    No preview · Article · Apr 2013 · La Presse Médicale
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    ABSTRACT: Objective: To assess factors that predict good tolerance of noninvasive ventilation (NIV), in order to improve survival and quality of life in subjects with amyotrophic lateral sclerosis. Methods: We conducted a prospective study in subjects with amyotrophic lateral sclerosis and requiring NIV. The primary end point was NIV tolerance at 1 month. Subjects, several of whom failed to complete the study, were classified as "tolerant" or "poorly tolerant," according to the number of hours of NIV use (more or less than 4 h per night, respectively). Results: Eighty-one subjects, 73 of whom also attended the 1-month follow-up visit, participated over 34 months. NIV tolerance after the first day of utilization predicted tolerance at 1 month (77.6% and 75.3% of subjects, respectively). Multivariate analysis disclosed 3 factors predicting good NIV tolerance: absence of airway secretions accumulation prior to NIV onset (odds ratio 11.5); normal bulbar function at initiation of NIV (odds ratio 8.5); and older age (weakly significant, odds ratio 1.1). Conclusion: Our study reveals 3 factors that are predictive of good NIV tolerance, in particular the absence of airway secretion accumulation, which should prompt NIV initiation before its appearance.
    Full-text · Article · Jan 2013 · Respiratory care
  • Jean-Christophe Antoine · Jean-Philippe Camdessanché
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    ABSTRACT: Opinion statement: Paraneoplastic disorders of the peripheral nervous system (PNS) are the most frequent manifestation of paraneoplasia. As with the central nervous system, two categories of immune mechanisms are distinguished. On one side, antibodies toward intracellular antigens (HuD and CV2-CRMP5) occur with subacute sensory neuronopathy or sensorimotor neuropathy probably depending on a T cell mediated disorder (group 1). On the other side, the Lambert-Eaton myasthenic syndrome (LEMS) and peripheral nerve hyperexcitability (PNH) occur with antibodies to cell membrane antigens, respectively, the voltage gated calcium channel and CASPR2 proteins, which are responsible for the disease (group 2). Treatment recommendation mostly depends on class IV studies. Three lines of therapeutics can be proposed, namely tumor, immunomodulatory and symptomatic treatments. Cancer treatment is crucial since an early tumor cure is the best way to stabilize patients in group 1 and improve those in group 2. This implies the use of an efficient strategy for cancer diagnosis. With group 2 symptomatic treatment including 3,4 diaminopyridine for LEMS and carbamazepine for PNH may suffice to obtain good quality remission. Immunomodulatory treatments like IVIg and plasma exchange, which have a well-established efficacy in antibody dependent diseases, may be used as second line treatments. Rituximab, for which there is only little evidence in this context, may be kept in a third line for severe refractory patients. With group 1 patients, who frequently develop an evolving and disabling disorder, bolus of methylprednisolone and or IVIg may be recommended while searching for and treating the tumor. If the tumor is not found and the patient deteriorates, monthly pulses of cyclophosphamide may stabilize the patients. Antidepressants and antiepileptic drugs efficacious in the treatment of neuropathic pain are to be used as symptomatic treatment when necessary. The choice is then based on the cost effectiveness and tolerance of these drugs.
    No preview · Article · Jan 2013 · Current Treatment Options in Neurology

Publication Stats

375 Citations
130.00 Total Impact Points

Institutions

  • 2014
    • Lyon Neuroscience Research Center
      Lyons, Rhône-Alpes, France
  • 2006-2014
    • Centre Hospitalier Universitaire de Saint-Étienne
      • Department of Neurology
      Saint-Étienne, Rhône-Alpes, France
    • Université Jean Monnet
      Saint-Étienne, Rhône-Alpes, France
  • 2011-2012
    • University of Lyon
      Lyons, Rhône-Alpes, France
    • Claude Bernard University Lyon 1
      Villeurbanne, Rhône-Alpes, France
  • 2009
    • Unité Inserm U1077
      Caen, Lower Normandy, France