Evaluating Clinical Tools to Monitor Sensory Neuronopathies Over Time in Adults

What this Trial Is About

Sensory neuronopathies SN are rare neuropathies characterized by damage to sensory neurons in the dorsal root ganglia, caused by various conditions such as paraneoplastic, autoimmune, toxic, and genetic factors. The functional outlook for patients is generally poor, with many experiencing significant disability. Because there are no reliable biomarkers for SN, clinical scales are used to assess disease severity and treatment response, but currently available scales are not specifically designed or validated for SN. This study is evaluating the use of multiple clinical assessment tools over time to identify the most appropriate scale for tracking disease progression in sensory neuronopathies. Patients will be followed longitudinally with three visits at baseline, 6 months, and 12 months. At each visit, a variety of clinical scales and tests such as 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, and electroneuromyography ENMG will be conducted. Participants will provide demographic and medical history information at baseline and undergo assessments at each scheduled visit. Researchers will measure changes in clinical scores and neurological function to evaluate these tools effectiveness in monitoring SN. The primary outcomes include the Clinical Global Impression of Change and Patient Global Impression of Change at 6 and 12 months. The study lasts 12 months with comprehensive follow-up and safety monitoring to better understand disease progression.

Clinical Evaluation of Sensory Neuronopathies: the Neuronoscore Study