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Sensory Neuronopathies: Clinical Presentation, Management, and Outcome
Aamna Maniyar1, Pawan T Ojha, Akash Chheda
1Department of Neurology, Grant Government Medical College and JJ Hospital, Mumbai, Maharashtra, India.
Annals of Indian Academy of Neurology
|September 30, 2024
Summary
Early and aggressive treatment with rituximab (RTX) shows promising results for sensory neuronopathies (SNNs), particularly Sjogren's syndrome-associated SNN and idiopathic SNN. Prompt immunotherapy initiation is key for patient improvement.
Area of Science:
- Neurology
- Immunology
- Clinical Medicine
Background:
- Sensory neuronopathies (SNNs) are rare disorders characterized by asymmetrical sensory loss.
- These conditions present unique clinical and electrophysiological features.
Purpose of the Study:
- To investigate the clinical characteristics and etiology of SNNs.
- To share treatment experiences and outcomes for SNN patients.
Main Methods:
- A 3-year prospective observational study evaluated 14 patients with SNN.
- Diagnosis followed Camdessanch´e criteria; etiology workup and treatment were individualized.
- Outcomes were assessed using the modified Rankin Scale at 12 months.
Main Results:
- Idiopathic SNN (I-SNN) comprised 64.3% of cases; Sjogren's syndrome-associated SNN (SS-SNN) and paraneoplastic syndrome were also identified.
- Rituximab (RTX) treatment correlated positively with improvement in SS-SNN and I-SNN (P=0.0256).
- Early immunotherapy initiation in I-SNN patients strongly correlated with improved outcomes (P=0.0119).
Conclusions:
- Intensive treatment with RTX yielded promising outcomes for SS-SNN and I-SNN.
- An 'hit hard and early' treatment strategy is crucial for managing SNNs effectively.
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