Sensory Neuronopathy
Conditions
Keywords
neurology, specific scale, rare neuropathy
Brief summary
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.
Interventions
OTHERLongitudinal 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
Sponsors
Centre Hospitalier Universitaire de Saint Etienne
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| The Clinical Global Impression of Change (CGI-C) and The Patient Global Impression of Change (PGI-C). | 6 months and 12 months | Global Impression of change measure evaluating overall change in clinical status compared with baseline. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| miSS Score change | 6 months and 12 months | Change in mISS score from baseline. |
| SEARS change | 6 months, 12 months | Change in SEARS score from baseline. |
| CADT change | 6 months and 12 months | Change in CADT score from baseline. |
| SARA change | 6 months and 12 months | Change in SARA score from baseline. |
| ONLS change | 6 months and 12 months | Change in ONLS score from baseline. |
| I-RODS change | 6 months and 12 months | Change in I-RODS score from baseline. |
| 9-Hole Peg Test change | 6 months and 12 months | Change in 9-Hole Peg Test score from baseline. |
| Timed Up and Go test change | 6 months and 12 months | Change in Timed Up and Go test from baseline |
| Modified Rankin Scale change | 6 months and 12 months | Change in Modified Rankin Scale from baseline |
| Quantified Rydel tuning fork test change | 6 months and 12 months | Change in Quantified Rydel tuning fork test from baseline |
| Visual Analog Scale change | 6 months and 12 months | Change in Visual Analog scale frome baseline |
| Electroneuromyography | 6 months and 12 months | Change in Electroneuromyography from baseline |
Countries
France, Switzerland
Contacts
CONTACTJean philippe PHD CAMDESSANCHE
CONTACTClara PFENNINGER
Outcome results
None listed