Clinical Evaluation of Sensory Neuronopathies: the Neuronoscore Study (NEURONOSCORE)
Sensory neuronopathies (SN) are a group of rare neuropathies characterized by selective destruction of sensory neurons located in the dorsal root ganglia. SN may result from a wide range of etiologies, particularly paraneoplastic, autoimmune, toxic, and genetic causes. The functional prognosis of patients with SN is generally poor: in a recent study, two-thirds of patients had a modified Rankin Scale (mRS) score ≥3 and nearly half had an mRS ≥4.
The absence of reliable biomarkers in neuropathies justifies the use of clinical scales as indicators of disease severity, disability, and treatment response. However, none of the currently available "general neuropathy" scales have been specifically designed or validated for SN. The only scale developed specifically for SN is the SEARS (Sensory Ataxia Rating Scale), proposed in 2019, but it has not been widely used nor validated in large populations.
As a result, the absence of a clinical scale specifically designed for patients with SN makes longitudinal follow-up more challenging, particularly when assessing the response to immunomodulatory or immunosuppressive treatments when these therapies are indicated.
Studieöversikt
Status
Betingelser
Studietyp
Inskrivning (Beräknad)
Fas
- Inte tillämpbar
Kontakter och platser
Studiekontakt
- Namn: Jean philippe PHD CAMDESSANCHE
- Telefonnummer: +33 4 77 12 05 59
- E-post: j.philippe.camdessanche@chu-st-etienne.fr
Studera Kontakt Backup
- Namn: Clara PFENNINGER
- Telefonnummer: +33 4 77 12 02 87
- E-post: clara.pfenninger@chu-st-etienne.fr
Studieorter
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Angers, Frankrike
- Har inte rekryterat ännu
- CHU d Angers
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Kontakt:
- CASSEREAU PH JULIEN
- Telefonnummer: +33 2 41 35 62 64
- E-post: jucassereau@chu-angers.fr
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Clermont-Ferrand, Frankrike
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- Chu de Clermont Ferrand
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Kontakt:
- PONCET- MAGEMONT DR Louis
- Telefonnummer: +33 4 73 752 202
- E-post: lponcet-megemnt@chu-clermontferrand.fr
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Grenoble, Frankrike
- Har inte rekryterat ännu
- CHU de GRENOBLE
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Kontakt:
- MALLARET DR MARTIAL
- Telefonnummer: +33 4 76 76 54 49
- E-post: mmallaret1@chu-grenoble.fr
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Lille, Frankrike
- Har inte rekryterat ännu
- CHU de Lille
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Kontakt:
- TARD DR CELINE
- Telefonnummer: +33 3 20 44 41 45
- E-post: celine.tard@chu-lillle.fr
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Limoges, Frankrike
- Har inte rekryterat ännu
- CHU de Limoges
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Kontakt:
- MAGY PHD Laurent
- Telefonnummer: +33 5 55 05 65 61
- E-post: laurent.magy@unilim.fr
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Lyon, Frankrike
- Har inte rekryterat ännu
- Hospices Civils de Lyon
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Kontakt:
- BOUHOUR DR Francoise
- Telefonnummer: +33 4 72 11 80 63
- E-post: francoise.bouhour@chu-lyon.fr
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Marseille, Frankrike
- Har inte rekryterat ännu
- Hopitaux Universitaire de Marseille
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Kontakt:
- DELMONT DR Emilien
- Telefonnummer: +33 4 91 38 65 79
- E-post: emilien.delmont@ap-hm.fr
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Nancy, Frankrike
- Har inte rekryterat ännu
- CHU de Nancy
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Kontakt:
- MICHAUD DR MAUD
- Telefonnummer: +33 3 83 85 94 88
- E-post: m.michaud@chru-nancy.fr
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Nantes, Frankrike
- Har inte rekryterat ännu
- CHU de Nantes
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Kontakt:
- PEREON PHD Yann
- Telefonnummer: +33 240 083 704
- E-post: Yann.Pereon@univ-nantes.fr
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Nice, Frankrike
- Har inte rekryterat ännu
- Chu De Nice
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Kontakt:
- PUMA DR ANGELA
- Telefonnummer: +33 4 92 03 90 73
- E-post: puma.ar@chu-nice.fr
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Paris, Frankrike
- Har inte rekryterat ännu
- Hopital, Bicetre
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Kontakt:
- ECHANIZ-LAGUNA PHD ANDONI
- Telefonnummer: +33 1 45 21 31 59
- E-post: andoni.echaniz-laguna@aphp.fr
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Saint-Etienne, Frankrike, 42055
- Rekrytering
- CHU de Saint-Etienne
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Kontakt:
- JEAN-PHILIPPE CAMDESSANCHE, PHD
- Telefonnummer: (4)77120559
- E-post: j.philippe.camdessanche@chu-st-etienne.fr
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Saint-Etienne, Frankrike
- Har inte rekryterat ännu
- CHU St Etienne
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Kontakt:
- LOSER Dr Valentin
- Telefonnummer: 0477127805
- E-post: valentin.loser@chu-st-etienne.fr
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Saint-Pierre, Frankrike
- Har inte rekryterat ännu
- CHU de la Réunion
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Kontakt:
- CHOUMERT DR ARIANE
- Telefonnummer: +262 262 35 91 66
- E-post: ariane.choumert@chu-reunion.fr
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Strasbourg, Frankrike
- Har inte rekryterat ännu
- Hopitaux Universitaire de Strasbourg
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Kontakt:
- CHANSON DR JEAN BAPTISTE
- Telefonnummer: +33 3 88 12 85 60
- E-post: jean-baptiste.chanson@chru-strasbourg.fr
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Toulouse, Frankrike
- Har inte rekryterat ännu
- CHU de Toulouse
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Kontakt:
- CINTAS DR PASCAL
- Telefonnummer: +33 5 61 77 94 40
- E-post: cintas.p@chu-toulouse.fr
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Geneva, Schweiz
- Har inte rekryterat ännu
- Hopitaux Universitaire de Geneve
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Kontakt:
- LASCANO DR AGUSTINE
- Telefonnummer: +41 22 372 83 58
- E-post: Agustina.Lascano@hug.ch
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Lausanne, Schweiz
- Har inte rekryterat ännu
- Chuv Centre Hospitalier Vaudois
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Kontakt:
- THEAUDIN PHD MARINE
- Telefonnummer: 0041(0)21 314 81 26
- E-post: marie.theaudin@chuv.ch
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Sion, Schweiz
- Har inte rekryterat ännu
- Hopital de Sion
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Kontakt:
- ALVAREZ DR VINCENT
- Telefonnummer: +41 27 603 40 00
- E-post: vincent.alvarez@hopitalvs.ch
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Deltagandekriterier
Urvalskriterier
Åldrar som är berättigade till studier
- Vuxen
- Äldre vuxen
Tar emot friska volontärer
Beskrivning
Inclusion Criteria:
- Patient affiliated with or beneficiary of a social security system
- Patient having received appropriate study information
- Adult patient ≥18 years old, male or female
- Patient diagnosed with probable SN according to Camdessanché et al. diagnostic criteria
- SN with one of the following etiologies:
Paraneoplastic SN with anti-Hu or anti-CV2/CRMP5 antibodies SN associated with Sjögren's syndrome, systemic lupus erythematosus, or primary biliary cholangitis Platinum-salt-induced SN SN caused by CANVAS syndrome
Exclusion Criteria:
- Patient unable to understand or read French
- Patient refusal to participate
Patient known to have another neuropathy phenotype and/or etiology that could significantly influence clinical scales and electrophysiological parameters, including:
- Diabetes mellitus
- Significant alcohol consumption
- Severe chronic kidney disease (GFR <30 ml/min)
- Vitamin B12 and/or vitamin E deficiency
- Vitamin B6 excess
- Chemotherapy other than platinum salts
- HIV infection
Studieplan
Hur är studien utformad?
Designdetaljer
- Primärt syfte: Övrig
- Tilldelning: N/A
- Interventionsmodell: Enskild gruppuppgift
- Maskning: Ingen (Open Label)
Vapen och interventioner
Deltagargrupp / Arm |
Intervention / Behandling |
|---|---|
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Experimentell: Group experimental
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After patient consent, three follow-up visits (T0, T6, T12) will be scheduled. Some information will already be collected at baseline (demographics, medical history, comorbidities). Three visits will be conducted: (T0), Follow-up at 6 months (T6), Follow-up at 12 months (T12) At each visit the following will be assessed: Clinical scales: mISS, SEARS, CADT, SARA, ONLS, I-RODS, 9-Hole Peg Test, Timed Up and Go test, mRS, Quantified Rydel tuning fork test, Visual Analog Scale (VAS) ENMG including sensory nerve action potentials of the radial and sural nerves (antidromic recording |
Vad mäter studien?
Primära resultatmått
Resultatmått |
Åtgärdsbeskrivning |
Tidsram |
|---|---|---|
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The Clinical Global Impression of Change (CGI-C) and The Patient Global Impression of Change (PGI-C).
Tidsram: 6 months and 12 months
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Global Impression of change measure evaluating overall change in clinical status compared with baseline.
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6 months and 12 months
|
Sekundära resultatmått
Resultatmått |
Åtgärdsbeskrivning |
Tidsram |
|---|---|---|
|
miSS Score change
Tidsram: 6 months and 12 months
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Change in mISS score from baseline.
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6 months and 12 months
|
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SEARS change
Tidsram: 6 months, 12 months
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Change in SEARS score from baseline.
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6 months, 12 months
|
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CADT change
Tidsram: 6 months and 12 months
|
Change in CADT score from baseline.
|
6 months and 12 months
|
|
SARA change
Tidsram: 6 months and 12 months
|
Change in SARA score from baseline.
|
6 months and 12 months
|
|
ONLS change
Tidsram: 6 months and 12 months
|
Change in ONLS score from baseline.
|
6 months and 12 months
|
|
I-RODS change
Tidsram: 6 months and 12 months
|
Change in I-RODS score from baseline.
|
6 months and 12 months
|
|
9-Hole Peg Test change
Tidsram: 6 months and 12 months
|
Change in 9-Hole Peg Test score from baseline.
|
6 months and 12 months
|
|
Timed Up and Go test change
Tidsram: 6 months and 12 months
|
Change in Timed Up and Go test from baseline
|
6 months and 12 months
|
|
Modified Rankin Scale change
Tidsram: 6 months and 12 months
|
Change in Modified Rankin Scale from baseline
|
6 months and 12 months
|
|
Quantified Rydel tuning fork test change
Tidsram: 6 months and 12 months
|
Change in Quantified Rydel tuning fork test from baseline
|
6 months and 12 months
|
|
Visual Analog Scale change
Tidsram: 6 months and 12 months
|
Change in Visual Analog scale frome baseline
|
6 months and 12 months
|
|
Electroneuromyography
Tidsram: 6 months and 12 months
|
Change in Electroneuromyography from baseline
|
6 months and 12 months
|
Samarbetspartners och utredare
Studieavstämningsdatum
Studera stora datum
Studiestart (Faktisk)
Primärt slutförande (Beräknad)
Avslutad studie (Beräknad)
Studieregistreringsdatum
Först inskickad
Först inskickad som uppfyllde QC-kriterierna
Första postat (Faktisk)
Uppdateringar av studier
Senaste uppdatering publicerad (Faktisk)
Senaste inskickade uppdateringen som uppfyllde QC-kriterierna
Senast verifierad
Mer information
Termer relaterade till denna studie
Nyckelord
Andra studie-ID-nummer
- 26CH090
- 2026-A00695-46 (Annan identifierare: ANSM)
Plan för individuella deltagardata (IPD)
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