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.
Studieoversikt
Status
Forhold
Studietype
Registrering (Antatt)
Fase
- Ikke aktuelt
Kontakter og plasseringer
Studiekontakt
- Navn: Jean philippe PHD CAMDESSANCHE
- Telefonnummer: +33 4 77 12 05 59
- E-post: j.philippe.camdessanche@chu-st-etienne.fr
Studer Kontakt Backup
- Navn: Clara PFENNINGER
- Telefonnummer: +33 4 77 12 02 87
- E-post: clara.pfenninger@chu-st-etienne.fr
Studiesteder
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Angers, Frankrike
- Har ikke rekruttert ennå
- CHU d Angers
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Ta kontakt med:
- 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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Ta kontakt med:
- PONCET- MAGEMONT DR Louis
- Telefonnummer: +33 4 73 752 202
- E-post: lponcet-megemnt@chu-clermontferrand.fr
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Grenoble, Frankrike
- Har ikke rekruttert ennå
- CHU de GRENOBLE
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Ta kontakt med:
- MALLARET DR MARTIAL
- Telefonnummer: +33 4 76 76 54 49
- E-post: mmallaret1@chu-grenoble.fr
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Lille, Frankrike
- Har ikke rekruttert ennå
- CHU de Lille
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Ta kontakt med:
- TARD DR CELINE
- Telefonnummer: +33 3 20 44 41 45
- E-post: celine.tard@chu-lillle.fr
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Limoges, Frankrike
- Har ikke rekruttert ennå
- CHU de Limoges
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Ta kontakt med:
- MAGY PHD Laurent
- Telefonnummer: +33 5 55 05 65 61
- E-post: laurent.magy@unilim.fr
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Lyon, Frankrike
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- Hospices Civils de Lyon
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Ta kontakt med:
- BOUHOUR DR Francoise
- Telefonnummer: +33 4 72 11 80 63
- E-post: francoise.bouhour@chu-lyon.fr
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Marseille, Frankrike
- Har ikke rekruttert ennå
- Hopitaux Universitaire de Marseille
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Ta kontakt med:
- DELMONT DR Emilien
- Telefonnummer: +33 4 91 38 65 79
- E-post: emilien.delmont@ap-hm.fr
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Nancy, Frankrike
- Har ikke rekruttert ennå
- CHU de Nancy
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Ta kontakt med:
- MICHAUD DR MAUD
- Telefonnummer: +33 3 83 85 94 88
- E-post: m.michaud@chru-nancy.fr
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Nantes, Frankrike
- Har ikke rekruttert ennå
- CHU de Nantes
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Ta kontakt med:
- PEREON PHD Yann
- Telefonnummer: +33 240 083 704
- E-post: Yann.Pereon@univ-nantes.fr
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Nice, Frankrike
- Har ikke rekruttert ennå
- Chu De Nice
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Ta kontakt med:
- PUMA DR ANGELA
- Telefonnummer: +33 4 92 03 90 73
- E-post: puma.ar@chu-nice.fr
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Paris, Frankrike
- Har ikke rekruttert ennå
- Hopital, Bicetre
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Ta kontakt med:
- 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
- Rekruttering
- CHU de Saint-Etienne
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Ta kontakt med:
- JEAN-PHILIPPE CAMDESSANCHE, PHD
- Telefonnummer: (4)77120559
- E-post: j.philippe.camdessanche@chu-st-etienne.fr
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Saint-Etienne, Frankrike
- Har ikke rekruttert ennå
- CHU St Etienne
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Ta kontakt med:
- LOSER Dr Valentin
- Telefonnummer: 0477127805
- E-post: valentin.loser@chu-st-etienne.fr
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Saint-Pierre, Frankrike
- Har ikke rekruttert ennå
- CHU de la Réunion
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Ta kontakt med:
- CHOUMERT DR ARIANE
- Telefonnummer: +262 262 35 91 66
- E-post: ariane.choumert@chu-reunion.fr
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Strasbourg, Frankrike
- Har ikke rekruttert ennå
- Hopitaux Universitaire de Strasbourg
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Ta kontakt med:
- 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 ikke rekruttert ennå
- CHU de Toulouse
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Ta kontakt med:
- CINTAS DR PASCAL
- Telefonnummer: +33 5 61 77 94 40
- E-post: cintas.p@chu-toulouse.fr
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Geneva, Sveits
- Har ikke rekruttert ennå
- Hopitaux Universitaire de Geneve
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Ta kontakt med:
- LASCANO DR AGUSTINE
- Telefonnummer: +41 22 372 83 58
- E-post: Agustina.Lascano@hug.ch
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Lausanne, Sveits
- Har ikke rekruttert ennå
- Chuv Centre Hospitalier Vaudois
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Ta kontakt med:
- THEAUDIN PHD MARINE
- Telefonnummer: 0041(0)21 314 81 26
- E-post: marie.theaudin@chuv.ch
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Sion, Sveits
- Har ikke rekruttert ennå
- Hopital de Sion
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Ta kontakt med:
- ALVAREZ DR VINCENT
- Telefonnummer: +41 27 603 40 00
- E-post: vincent.alvarez@hopitalvs.ch
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Deltakelseskriterier
Kvalifikasjonskriterier
Alder som er kvalifisert for studier
- Voksen
- Eldre voksen
Tar imot friske 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 studiet utformet?
Designdetaljer
- Primært formål: Annen
- Tildeling: N/A
- Intervensjonsmodell: Enkeltgruppeoppdrag
- Masking: Ingen (Open Label)
Våpen og intervensjoner
Deltakergruppe / Arm |
Intervensjon / Behandling |
|---|---|
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Eksperimentell: 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 |
Hva måler studien?
Primære resultatmål
Resultatmål |
Tiltaksbeskrivelse |
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 |
Tiltaksbeskrivelse |
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
|
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
|
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
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Change in Quantified Rydel tuning fork test from baseline
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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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Samarbeidspartnere og etterforskere
Studierekorddatoer
Studer hoveddatoer
Studiestart (Faktiske)
Primær fullføring (Antatt)
Studiet fullført (Antatt)
Datoer for studieregistrering
Først innsendt
Først innsendt som oppfylte QC-kriteriene
Først lagt ut (Faktiske)
Oppdateringer av studieposter
Sist oppdatering lagt ut (Faktiske)
Siste oppdatering sendt inn som oppfylte QC-kriteriene
Sist bekreftet
Mer informasjon
Begreper knyttet til denne studien
Nøkkelord
Andre studie-ID-numre
- 26CH090
- 2026-A00695-46 (Annen identifikator: ANSM)
Plan for individuelle deltakerdata (IPD)
Planlegger du å dele individuelle deltakerdata (IPD)?
Legemiddel- og utstyrsinformasjon, studiedokumenter
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