Neuropathic Pain in CASPR2 Antibody Disease Spectrum: A Systematic Review
Researchers at the University of Florence have conducted a systematic review to better understand neuropathic pain associated with Contactin-associated protein-like 2 (CASPR2) antibodies. The study found that 7.4% of patients with CASPR2 antibody disease spectrum presented with isolated neuropathic pain, which was typically severe and presented as distal burning pain, widespread pain with myalgia and cramps, or severe back pain radiating to the legs. Immunotherapy, especially second-line treatments, improved pain in 85.4% of cases, with complete remission of symptoms in 38.2% of patients.
Key Takeaways:
- CASPR2 antibodies are mainly linked to acquired neuromyotonia, limbic encephalitis, and Morvan syndrome, but neuropathic pain is increasingly reported.
- The literature review identified 216 patients with neuropathic pain in the context of CASPR2 antibody disease spectrum, with isolated pain described in only 16/216 (7.4%) cases.
- Pain was typically severe and presented as distal burning pain (50.3%), widespread pain with myalgia and cramps (21.1%), or severe back pain radiating to the legs (8.8%).
- Peripheral nerve hyperexcitability was found in 65.8% of patients, while small nociceptive fiber dysfunction was identified in 84.2% of patients.
- Immunotherapy, especially second-line treatments, improved pain in 85.4% of cases, with complete remission of symptoms in 38.2% of patients.
- Symptomatic treatment helped 55.1% of patients, with complete response in 20.4% of patients.
- Long-term therapy was often needed, and relapses occurred in 5.2% of patients after discontinuation.
Statistics:
- 216 patients with neuropathic pain in the context of CASPR2 antibody disease spectrum were identified in the literature review.
- 16/216 (7.4%) of patients with CASPR2 antibody disease spectrum presented with isolated neuropathic pain.
- 50.3% of patients with neuropathic pain presented with distal burning pain.
- 21.1% of patients with neuropathic pain presented with widespread pain with myalgia and cramps.
- 8.8% of patients with neuropathic pain presented with severe back pain radiating to the legs.
- 65.8% of patients with neuropathic pain showed peripheral nerve hyperexcitability.
- 84.2% of patients with neuropathic pain showed small nociceptive fiber dysfunction.
- 85.4% of patients with neuropathic pain improved with immunotherapy.
- 38.2% of patients with neuropathic pain achieved complete remission of symptoms with immunotherapy.
- 55.1% of patients with neuropathic pain improved with symptomatic treatment.
- 20.4% of patients with neuropathic pain achieved complete response with symptomatic treatment.
- 5.2% of patients with neuropathic pain relapsed after discontinuation of treatment.
Sources:
- Journal of Neuroimmunology
- University of Florence
- NewsRx LLC
- Drug Week (NewsRx LLC, 2025)