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.
Studieoversigt
Status
Betingelser
Undersøgelsestype
Tilmelding (Anslået)
Fase
- Ikke anvendelig
Kontakter og lokationer
Studiekontakt
- Navn: Jean philippe PHD CAMDESSANCHE
- Telefonnummer: +33 4 77 12 05 59
- E-mail: j.philippe.camdessanche@chu-st-etienne.fr
Undersøgelse Kontakt Backup
- Navn: Clara PFENNINGER
- Telefonnummer: +33 4 77 12 02 87
- E-mail: clara.pfenninger@chu-st-etienne.fr
Studiesteder
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Angers, Frankrig
- Chu D Angers
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Kontakt:
- CASSEREAU PH JULIEN
- Telefonnummer: +33 2 41 35 62 64
- E-mail: jucassereau@chu-angers.fr
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Clermont-Ferrand, Frankrig
- Chu de Clermont Ferrand
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Kontakt:
- PONCET- MAGEMONT DR Louis
- Telefonnummer: +33 4 73 752 202
- E-mail: lponcet-megemnt@chu-clermontferrand.fr
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Grenoble, Frankrig
- CHU de Grenoble
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Kontakt:
- MALLARET DR MARTIAL
- Telefonnummer: +33 4 76 76 54 49
- E-mail: mmallaret1@chu-grenoble.fr
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Lille, Frankrig
- CHU de Lille
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Kontakt:
- TARD DR CELINE
- Telefonnummer: +33 3 20 44 41 45
- E-mail: celine.tard@chu-lillle.fr
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Limoges, Frankrig
- CHU de Limoges
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Kontakt:
- MAGY PHD Laurent
- Telefonnummer: +33 5 55 05 65 61
- E-mail: laurent.magy@unilim.fr
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Lyon, Frankrig
- Hospices Civils de Lyon
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Kontakt:
- BOUHOUR DR Francoise
- Telefonnummer: +33 4 72 11 80 63
- E-mail: francoise.bouhour@chu-lyon.fr
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Marseille, Frankrig
- Hopitaux Universitaire de Marseille
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Kontakt:
- DELMONT DR Emilien
- Telefonnummer: +33 4 91 38 65 79
- E-mail: emilien.delmont@ap-hm.fr
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Nancy, Frankrig
- Chu de Nancy
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Kontakt:
- MICHAUD DR MAUD
- Telefonnummer: +33 3 83 85 94 88
- E-mail: m.michaud@chru-nancy.fr
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Nantes, Frankrig
- CHU de Nantes
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Kontakt:
- PEREON PHD Yann
- Telefonnummer: +33 240 083 704
- E-mail: Yann.Pereon@univ-nantes.fr
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Nice, Frankrig
- CHU de Nice
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Kontakt:
- PUMA DR ANGELA
- Telefonnummer: +33 4 92 03 90 73
- E-mail: puma.ar@chu-nice.fr
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Paris, Frankrig
- Hopital, Bicetre
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Kontakt:
- ECHANIZ-LAGUNA PHD ANDONI
- Telefonnummer: +33 1 45 21 31 59
- E-mail: andoni.echaniz-laguna@aphp.fr
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Saint-Etienne, Frankrig, 42055
- Chu de Saint-Etienne
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Saint-Etienne, Frankrig
- CHU St Etienne
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Kontakt:
- LOSER Dr Valentin
- Telefonnummer: 0477127805
- E-mail: valentin.loser@chu-st-etienne.fr
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Saint-Pierre, Frankrig
- Chu de La Reunion
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Kontakt:
- CHOUMERT DR ARIANE
- Telefonnummer: +262 262 35 91 66
- E-mail: ariane.choumert@chu-reunion.fr
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Strasbourg, Frankrig
- Hopitaux Universitaire de Strasbourg
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Kontakt:
- CHANSON DR JEAN BAPTISTE
- Telefonnummer: +33 3 88 12 85 60
- E-mail: jean-baptiste.chanson@chru-strasbourg.fr
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Toulouse, Frankrig
- CHU de Toulouse
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Kontakt:
- CINTAS DR PASCAL
- Telefonnummer: +33 5 61 77 94 40
- E-mail: cintas.p@chu-toulouse.fr
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Geneva, Schweiz
- Hopitaux Universitaire de Geneve
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Kontakt:
- LASCANO DR AGUSTINE
- Telefonnummer: +41 22 372 83 58
- E-mail: Agustina.Lascano@hug.ch
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Lausanne, Schweiz
- Chuv Centre Hospitalier Vaudois
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Kontakt:
- THEAUDIN PHD MARINE
- Telefonnummer: 0041(0)21 314 81 26
- E-mail: marie.theaudin@chuv.ch
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Sion, Schweiz
- Hopital de Sion
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Kontakt:
- ALVAREZ DR VINCENT
- Telefonnummer: +41 27 603 40 00
- E-mail: vincent.alvarez@hopitalvs.ch
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Deltagelseskriterier
Berettigelseskriterier
Aldre berettiget til at studere
- Voksen
- Ældre voksen
Tager imod sunde frivillige
Beskrivelse
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
Hvordan er undersøgelsen tilrettelagt?
Design detaljer
- Primært formål: Andet
- Tildeling: N/A
- Interventionel model: Enkelt gruppeopgave
- Maskning: Ingen (Åben etiket)
Våben og indgreb
Deltagergruppe / Arm |
Intervention / Behandling |
|---|---|
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Eksperimentel: 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 |
Hvad måler undersøgelsen?
Primære resultatmål
Resultatmål |
Foranstaltningsbeskrivelse |
Tidsramme |
|---|---|---|
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The Clinical Global Impression of Change (CGI-C) and The Patient Global Impression of Change (PGI-C).
Tidsramme: 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
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Sekundære resultatmål
Resultatmål |
Foranstaltningsbeskrivelse |
Tidsramme |
|---|---|---|
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miSS Score change
Tidsramme: 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
Tidsramme: 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
Tidsramme: 6 months and 12 months
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Change in CADT score from baseline.
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6 months and 12 months
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SARA change
Tidsramme: 6 months and 12 months
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Change in SARA score from baseline.
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6 months and 12 months
|
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ONLS change
Tidsramme: 6 months and 12 months
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Change in ONLS score from baseline.
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6 months and 12 months
|
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I-RODS change
Tidsramme: 6 months and 12 months
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Change in I-RODS score from baseline.
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6 months and 12 months
|
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9-Hole Peg Test change
Tidsramme: 6 months and 12 months
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Change in 9-Hole Peg Test score from baseline.
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6 months and 12 months
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Timed Up and Go test change
Tidsramme: 6 months and 12 months
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Change in Timed Up and Go test from baseline
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6 months and 12 months
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Modified Rankin Scale change
Tidsramme: 6 months and 12 months
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Change in Modified Rankin Scale from baseline
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6 months and 12 months
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Quantified Rydel tuning fork test change
Tidsramme: 6 months and 12 months
|
Change in Quantified Rydel tuning fork test from baseline
|
6 months and 12 months
|
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Visual Analog Scale change
Tidsramme: 6 months and 12 months
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Change in Visual Analog scale frome baseline
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6 months and 12 months
|
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Electroneuromyography
Tidsramme: 6 months and 12 months
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Change in Electroneuromyography from baseline
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6 months and 12 months
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Samarbejdspartnere og efterforskere
Datoer for undersøgelser
Studer store datoer
Studiestart (Anslået)
Primær færdiggørelse (Anslået)
Studieafslutning (Anslået)
Datoer for studieregistrering
Først indsendt
Først indsendt, der opfyldte QC-kriterier
Først opslået (Faktiske)
Opdateringer af undersøgelsesjournaler
Sidste opdatering sendt (Faktiske)
Sidste opdatering indsendt, der opfyldte kvalitetskontrolkriterier
Sidst verificeret
Mere information
Begreber relateret til denne undersøgelse
Nøgleord
Andre undersøgelses-id-numre
- 26CH090
- 2026-A00695-46 (Anden identifikator: ANSM)
Plan for individuelle deltagerdata (IPD)
Planlægger du at dele individuelle deltagerdata (IPD)?
Lægemiddel- og udstyrsoplysninger, undersøgelsesdokumenter
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