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.
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
Patient unable to understand or read French
Patient refusal to participate
Diabetes mellitus
Significant alcohol consumption
Single group
Longitudinal monitoring of clinical assessment scores to identify the most appropriate tool for tracking disease progression in sensory neuronopathies.
Other interventionAfter 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
The Clinical Global Impression of Change (CGI-C) and The Patient Global Impression of Change (PGI-C).
Global Impression of change measure evaluating overall change in clinical status compared with baseline.
miSS Score change
Change in mISS score from baseline.
SEARS change
Change in SEARS score from baseline.
CADT change
Change in CADT score from baseline.
SARA change
Change in SARA score from baseline.
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