Clinical Evaluation of Sensory Neuronopathies: the Neuronoscore Study (NEURONOSCORE)

June 11, 2026 updated by: Centre Hospitalier Universitaire de Saint Etienne

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.

Study Overview

Status

Not yet recruiting

Conditions

Intervention / Treatment

Study Type

Interventional

Enrollment (Estimated)

70

Phase

  • Not Applicable

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Adult
  • Older Adult

Accepts Healthy Volunteers

No

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Primary Purpose: Other
  • Allocation: N/A
  • Interventional Model: Single Group Assignment
  • Masking: None (Open Label)

Arms and Interventions

Participant Group / Arm
Intervention / Treatment
Experimental: Group experimental
Other: Longitudinal monitoring of clinical assessment scores to identify the most appropriate tool for tracking disease progression in sensory neuronopathies.

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

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
The Clinical Global Impression of Change (CGI-C) and The Patient Global Impression of Change (PGI-C).
Time Frame: 6 months and 12 months
Global Impression of change measure evaluating overall change in clinical status compared with baseline.
6 months and 12 months

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
miSS Score change
Time Frame: 6 months and 12 months
Change in mISS score from baseline.
6 months and 12 months
SEARS change
Time Frame: 6 months, 12 months
Change in SEARS score from baseline.
6 months, 12 months
CADT change
Time Frame: 6 months and 12 months
Change in CADT score from baseline.
6 months and 12 months
SARA change
Time Frame: 6 months and 12 months
Change in SARA score from baseline.
6 months and 12 months
ONLS change
Time Frame: 6 months and 12 months
Change in ONLS score from baseline.
6 months and 12 months
I-RODS change
Time Frame: 6 months and 12 months
Change in I-RODS score from baseline.
6 months and 12 months
9-Hole Peg Test change
Time Frame: 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
Time Frame: 6 months and 12 months
Change in Timed Up and Go test from baseline
6 months and 12 months
Modified Rankin Scale change
Time Frame: 6 months and 12 months
Change in Modified Rankin Scale from baseline
6 months and 12 months
Quantified Rydel tuning fork test change
Time Frame: 6 months and 12 months
Change in Quantified Rydel tuning fork test from baseline
6 months and 12 months
Visual Analog Scale change
Time Frame: 6 months and 12 months
Change in Visual Analog scale frome baseline
6 months and 12 months
Electroneuromyography
Time Frame: 6 months and 12 months
Change in Electroneuromyography from baseline
6 months and 12 months

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Centre Hospitalier Universitaire de Saint Etienne

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Estimated)

June 25, 2026

Primary Completion (Estimated)

June 25, 2028

Study Completion (Estimated)

June 25, 2028

Study Registration Dates

First Submitted

June 8, 2026

First Submitted That Met QC Criteria

June 11, 2026

First Posted (Actual)

June 16, 2026

Study Record Updates

Last Update Posted (Actual)

June 16, 2026

Last Update Submitted That Met QC Criteria

June 11, 2026

Last Verified

June 1, 2026

More Information

Terms related to this study

Keywords

Other Study ID Numbers

  • 26CH090
  • 2026-A00695-46 (Other Identifier: ANSM)

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

UNDECIDED

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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