Atypical Chronic Inflammatory Demyelinating Polyneuropathy With Relapsing Sensory-Predominant Sensorimotor Neuropathy
DOI:
https://doi.org/10.19166/lijn.v1i3.10338Keywords:
Chronic inflammatory demyelinating polyneuropathy, electrodiagnostic studies, immunotherapy, relapse, sensory-predominantAbstract
Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, acquired autoimmune neuropathy with progressive symptoms. Intravenous immunoglobulin (IVIg) and corticosteroids are effective first-line therapies; however, many patients experience residual symptoms or relapse within the first year.
Case Presentation: A 39-year-old male with an initial diagnosis of carpal tunnel syndrome presented with a 13-month history of progressive sensory disturbances, evolving from lower limb paresthesia with gait instability. EMG confirmed demyelinating and axonal sensorimotor polyneuropathy consistent with CIDP. He was hospitalized and responded well to intravenous methylprednisolone and IVIg, but relapsed nine months later after a respiratory infection, presenting with distal sensory symptoms and preserved strength. Cerebrospinal fluid analysis showed albuminocytologic dissociation, while autoimmune nodopathy markers were negative. Retreatment with IVIg and methylprednisolone led to clinical improvement, with only mild exertion-related symptoms at two-month follow-up.
Discussion: This case highlights a rare sensory-predominant variant of CIDP with relapse triggered by infection. Relapses often require renewed immunotherapy, with corticosteroids and IVIg as first-line treatments; long-term management may include maintenance IVIg and gradual steroid tapering.
Conclusions: Early recognition, appropriate immunotherapy, and long-term
monitoring are essential to sustain remission and prevent disability in sensory
predominant CIDP.
Keywords: Chronic inflammatory demyelinating polyneuropathy; electrodiagnostic studies; immunotherapy; relapse; sensory-predominant
References
1. Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy. Available online: https://practicalneurology.com/diseases diagnoses/neuromuscular/diagnosis-of chronic-inflammatory-demyelinating polyneuropathy/32112/ (accessed on 10 August 2025)
2. Caballero-Ávila M, Martin-Aguilar L, Collet-Vidiella R, pathophysiological et al. and mechanistic review of chronic inflammatory demyelinating polyradiculoneuropathy therapy. Frontiers in Immunology. 2025;16.https://doi.org/10.3389/fimmu.2025.1575464
3. Gogia B, Cabrero FR, Suheb MZK, et al. Chronic Inflammatory Demyelinating Polyradiculoneuropathy. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024. Available from: https://www.ncbi.nlm.nih.gov/books/NBK563249/
4. Van den Bergh PY, van Doorn PA, Hadden RD, et al. European Academy of Neurology/PeripheralNerveSociety guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force—second revision. Journal of the Peripheral Nervous System. 2021;26(3):242–68. https://doi.org/10.1111/jns.12455
5. Mathey EK, Park SB, Hughes RAC, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry. 2015;86:973-985. https://doi.org/10.1136/jnnp-2014309697
6. Bus SR, Broers MC, Lucke IM, et al. Clinical outcome of CIDP one year after start of treatment: A prospective cohort study. Journal of Neurology. 2021;269(2):945 55. https://doi.org/10.1007/s00415-02110677-5
7. De Lorenzo A, Liberatore G, Doneddu PE, et al. Impact of 2021 European Academy of Neurology/Peripheral Nerve Society diagnostic criteria on diagnosis and therapy of chronic inflammatory demyelinating polyradiculoneuropathy variants. European Journal of Neurology. 2024;31(4). https://doi.org/10.1111/ene.16190
8. Niu J, Zhang L, Hu N, et al. Long-term follow-up of relapse and remission of CIDP in a Chinese cohort. BMJ Neurology Open. A 2024;6(1). https://doi.org/10.1136/bmjno-2024000651
9. Oh SJ, King P. Sensory chronic inflammatory demyelinating polyradiculoneuropathy: immunotherapy responsive Neglected sensory neuropathy. Journal of Clinical Neurology. 2024;20(3):276. https://doi.org/10.3988/jcn.2023.0469
10. Shelly S, Shouman K, Paul P, et al. Expanding the spectrum of chronic immune sensory polyradiculopathy. Neurology. 2021;96(16). https://doi.org/10.1212/wnl.0000000000011792
11. Menon D, Katzberg HD, Bril V. Treatment approaches for atypical CIDP. Frontiers in Neurology. 2021;12. https://doi.org/10.3389/fneur.2021.653734
12. Lewis RA, van Doorn PA, Sommer C. Tips in navigating the diagnostic complexities of chronic inflammatory demyelinating polyradiculoneuropathy. Journal of the Neurological Sciences. 2022;443:120478. https://doi.org/10.1016/j.jns.2022.120478
13. Cornblath DR, van Doorn PA, Hartung HP, et al. ProCID Investigators. Randomized trial of three IVIg doses for treating chronic inflammatory demyelinating polyneuropathy. Brain. 2022;145(3):887896.
https://doi.org/10.1093/brain/awab422
14. López-Hernández JC, Mercado-Pompa A, Pérez-Torres T, et al. Efficacy of long-term prednisone therapy in patients with chronic inflammatory demyelinating polyneuropathy (CIDP): a retrospective cohort study. Rev Neurol. 2021;73(8): 275-81. https://doi.org/10.33588/rn.7308.2021261
