From the Research
The management of paraneoplastic sensitive neuropathies involves a dual approach targeting both the underlying malignancy and the immune response, with first-line treatment focusing on identifying and treating the primary tumor, and immunotherapy as the cornerstone of symptomatic management, typically beginning with intravenous immunoglobulin (IVIG) or plasma exchange, as supported by the most recent study 1. The goal of treatment is to improve or stabilize neurological symptoms, and to manage pain and other symptoms.
- Identifying and treating the primary tumor is crucial, as this can lead to improvement or stabilization of neurological symptoms.
- Immunotherapy is the cornerstone of symptomatic management, and may include:
- Intravenous immunoglobulin (IVIG) at 2g/kg divided over 2-5 days, followed by monthly maintenance doses of 1g/kg.
- Plasma exchange with 5-6 exchanges over 10-14 days.
- Corticosteroids such as prednisone (starting at 1mg/kg/day with gradual taper) or methylprednisolone (1g IV daily for 3-5 days).
- For refractory cases, second-line immunosuppressants may be necessary, including:
- Rituximab (375mg/m² weekly for 4 weeks).
- Cyclophosphamide (750-1000mg/m² monthly).
- Mycophenolate mofetil (1-2g daily).
- Pain management often requires neuropathic pain medications like gabapentin (300-1200mg three times daily), pregabalin (75-300mg twice daily), or duloxetine (30-60mg daily).
- Regular neurological assessments every 3-6 months with electrophysiological studies help monitor disease progression and treatment response, as noted in 1. This comprehensive approach is necessary because these neuropathies result from immune-mediated damage to dorsal root ganglia neurons triggered by tumor antigens, causing sensory deficits, neuropathic pain, and sensory ataxia, and is supported by the latest research 1.