Síndrome de polirradiculopatía desmielinizante crónica variante asimétrica paraneoplásica
.
Palabras clave:
polirradiculopatía, seminoma, plasmaféresis, inmunoglobulinas
Resumen
El síndrome de polirradiculopatía desmielinizante crónica de presentación atípica, es una neuropatía inmunomediada caracterizada por debilidad progresiva o recurrente, alteraciones sensitivas y arreflexia, con evolución mayor a ocho semanas. Sus formas atípicas pueden simular entidades agudas como el síndrome de Guillain-Barré, dificultando el diagnóstico. En raras ocasiones, puede presentarse una forma asimétrica (1-2% de los casos), más aún la forma paraneoplásica (1% de la formas asimétricas).
Este caso destaca la importancia del seguimiento clínico en neuropatías desmielinizantes, ya que la evolución con recaídas permite diferenciar formas crónicas de formas agudas. La asociación con seminoma sugiere un posible mecanismo inmunológico paraneoplásico. La respuesta al tratamiento combinado refuerza la necesidad de un abordaje integral.
Debe considerarse en pacientes con evolución atípica de neuropatías agudas, especialmente en presencia de neoplasias, para optimizar el diagnóstico y mejorar el pronóstico mediante tratamiento oportuno.
Citas
1. Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force—Second revision. Eur J Neurol [Internet]. 2021 [cited 2025 Nov 8];28(11):3556-83. Available from: https://onlinelibrary.wiley.com/doi/full/10.1111/ene.14959
2. Allen JA, Lewis RA. CIDP diagnostic pitfalls and perception of treatment benefit. Neurology [Internet]. 2015 [cited 2025 Nov 8];85(6): 498-504. Available from: https://pubmed.ncbi.nlm.nih.gov/26180143/ Subscription required
3. Mathey EK, Park SB, Hughes RAC, Pollard JD, Armati PJ, Barnett MH, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry [Internet]. 2015 [cited 2025 Nov 8];86(9):973-85. Available from: https://pubmed.ncbi.nlm.nih.gov/25677463/
4. Doneddu PE, Cocito D, Manganelli F, Fazio R, Briani Ch, Filosto M, et al. Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry [Internet]. 2019 [cited 2025 Nov 8];90(2):125-32. Available from: https://pubmed.ncbi.nlm.nih.gov/30297520/
5. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet [Internet]. 2016 [cited 2025 Nov 8];388(10045):717-27. Available from: https://www.thelancet.com/article/S0140-6736(16)00339-1/fulltext
6. Shahrizaila N, Lehmann HC, Kuwabara S. Guillain-Barré syndrome. Lancet [Internet]. 2021[cited 2025 Nov 8];397(10280):1214-28. Available from: https://pubmed.ncbi.nlm.nih.gov/33647239/ Subscription required
7. Lehmann HCh, Burke D, Kuwabara S. Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment. J Neurol Neurosurg Psychiatry [Internet]. 2019 [cited 2025 Nov 8];90(9):981-7. Available from: https://pubmed.ncbi.nlm.nih.gov/30992333/
8. Querol L, Illa I. Paranodal and other autoantibodies in chronic inflammatory neuropathies. Curr Opin Neurol [Internet]. 2015 [cited 2025 Nov 8];28(5):474-9. Available from: https://pubmed.ncbi.nlm.nih.gov/26263474/
9. Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JChG, Desestret V, Dubey D, et al. Updated diagnostic criteria for paraneoplastic neurologic syndromes. Neurol Neuroimmunol Neuroinflamm [Internet]. 2021 [cited 2025 Nov 8];8(4): e1014. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC8237398/
10. Vogrig A, Gigli GL, Segatti S, Corazza E, Marini A, Bernardini A, et al. Paraneoplastic neuropathies: clinical presentations and management. Curr Treat Options Neurol [Internet]. 2020 [cited 2025 Nov 8];22(12):43. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC8517070/ (Referencia no localizada)
11. Oaklander AL, Lunn MP, Hughes RA, van Schaik IN, Frost Ch, Chalk CH. Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst Rev [Internet]. 2017 [cited 2025 Nov 8];1(1):CD010369. Available from: https://pubmed.ncbi.nlm.nih.gov/28084646/
12. Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Engl J Med [Internet]. 2003 [cited 2025 Nov 8];349(16):1543-54. Available from: https://pubmed.ncbi.nlm.nih.gov/14561798/
13. Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol [Internet]. 2019 [cited 2025 Nov 8];15(11):671-83. Available from: https://pubmed.ncbi.nlm.nih.gov/31541214/
14. Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology [Internet]. 2011 [cited 2025 Nov 8];36(2):123-33. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC5703046/
15. Fokke Ch, van den Berg B, Drenthen J, Walgaard Ch, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain [Internet]. 2014 [cited 2025 Nov 8];137(Pt 1):33-43. Available from: https://www.gbs-cidp.org/wp-content/uploads/2014/01/2014-Fokke-GBS-Brighton-Brain-2014.pdf
16. Hughes RA, Brassington R, Gunn AA, van Doorn PA. Corticosteroids for Guillain-Barré syndrome. Cochrane Database Syst Rev [Internet]. 2016 [cited 2025 Nov 8];10(10):CD001446. Available from: https://pubmed.ncbi.nlm.nih.gov/27775812/
17. Rajabally YA, Nicolas G, Piéret F, Bouche P, Van den Bergh PYK. Validity of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy: a multicentre European study. J Neurol Neurosurg Psychiatry [Internet]. 2009 [cited 2025 Nov 8];80(12):1364-8. Available from: https://www.researchgate.net/publication/26686112_Validity_of_diagnostic_criteria_for_chronic_inflammatory_demyelinating_polyneuropathy_A_multicentre_European_study
18. Kuwabara S, Misawa S. Chronic inflammatory demyelinating polyneuropathy: clinical subtypes and their correlation with electrophysiology. Clin Exp Neuroimmunol [Internet]. 2011 [cited 2025 Nov 8];2(2):41-8. Available from: https://onlinelibrary.wiley.com/doi/full/10.1111/j.1759-1961.2011.00020.x
19. Zekeridou A, Lennon VA. Neurologic autoimmunity in the era of checkpoint inhibitor cancer immunotherapy. Mayo Clin Proc [Internet]. 2019 [cited 2025 Nov 8];94(9):1865-78. Available from: https://pubmed.ncbi.nlm.nih.gov/31358366/
20. Vogrig A, Fouret M, Joubert B, Picard G, Rogemond V, Pinto AL, et al. Increased frequency of anti-Ma2 encephalitis associated with immune checkpoint inhibitors. Neurol Neuroimmunol Neuroinflamm [Internet]. 2019 [cited 2025 Nov 8];6(6): e604. Available from: https://www.neurology.org/doi/10.1212/NXI.0000000000000604
21. Cortese I, Chaudhry V, So YT, Cantor F, Cornblath DR, Rae-Grant A. Evidence-based guideline update: plasmapheresis in neurologic disorders. Neurology [Internet]. 2011 [cited 2025 Nov 8];76(3):294-300. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC3034395/
22. Graus F, Dalmau J. Paraneoplastic neurological syndromes in the era of immune-checkpoint inhibitors. Nat Rev Clin Oncol [Internet]. 2019 [cited 2025 Nov 8];16(9):535-48. Available from: https://pubmed.ncbi.nlm.nih.gov/30867573/ Subscription required
23. Lehmann HC, Wunderlich G, Fink GR, Sommer C. Diagnosis of peripheral neuropathy. Neurol Res Pract. 2020 Jul 15;2:20. doi: 10.1186/s42466-020-00064-2. Available from: https://pubmed.ncbi.nlm.nih.gov/33324924/
24. Dimachkie MM, Barohn RJ. Chronic inflammatory demyelinating polyneuropathy. Curr Treat Options Neurol [Internet]. 2013 [cited 2025 Nov 8];15(3):350-66. Available from: https://pubmed.ncbi.nlm.nih.gov/23564314/
25. Maudes E, Landa J, Muñoz-Lopetegi A, Armangue T, Alba M, Saiz A, et al. Clinical significance of Kelch-like protein 11 antibodies. Neurol Neuroimmunol Neuroinflamm [Internet]. 2020 [cited 2025 Nov 8];7(3):e666. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC7051195/
26. Devine MF, Kothapalli N, Elkhooly M, Dubey D. Paraneoplastic neurological syndromes: clinical presentations and management. Ther Adv Neurol Disord [Internet]. 2021 [cited 2025 Nov 8];14:1756286420985323. Available from: https://pubmed.ncbi.nlm.nih.gov/33796141/
27. Harahsheh E, Hammami MB, Gupta P, Costello BA, Leibovich B, Cheville JC, et al. Testicular cancer–associated paraneoplastic neurologic syndromes. JAMA Netw Open [Internet]. 2025 [cited 2025 Dec 8];8(10):e e2538584. Available from: https://pubmed.ncbi.nlm.nih.gov/41118166/
28. Dalmau J, Rosenfeld MR. Paraneoplastic syndromes of the CNS. Lancet Neurol [Internet]. 2008 [cited 2025 Nov 8];7(4):327-40. Available from: https://pubmed.ncbi.nlm.nih.gov/18339348/
29. Dimitrov S, Tsalta-Mladenov M, Kabakchieva P, Georgiev T, Andonova S. Paraneoplastic Neurological Syndromes: Advances and Future Perspectives in Immunopathogenesis and Management. Antibodies (Basel). 2026 Jan 14;15(1):8. doi: 10.3390/antib15010008. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC12821609/
30. Gilligan M, McGuigan C, McKeon A. Paraneoplastic Neurologic Disorders. Curr Neurol Neurosci Rep. 2023 Mar;23(3):67-82. doi: 10.1007/s11910-023-01250-w. Available from: https://pubmed.ncbi.nlm.nih.gov/36781586/
31. Graus F, Dalmau J. Paraneoplastic neurological syndromes: diagnosis and treatment. Curr Opin Neurol [Internet]. 2007 [cited 2025 Nov 8];20(6):732-7. Available from: https://pubmed.ncbi.nlm.nih.gov/17992098/ Subscription required
32. Titulaer MJ, Soffietti R, Dalmau J, Gilhus NE, Giometto B, Graus F, et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force. Eur J Neurol [Internet]. 2011 [cited 2025 Nov 8];18(1):19-e3. Available from: https://pubmed.ncbi.nlm.nih.gov/20880069/
33. Thakolwiboon S, Zekeridou A. Paraneoplastic neurologic disorders and neurologic complications of immune checkpoint inhibitors. Handb Clin Neurol. 2026;214:85-103. doi: 10.1016/B978-0-323-90887-0.00018-3. Available from: https://pubmed.ncbi.nlm.nih.gov/41526153/
2. Allen JA, Lewis RA. CIDP diagnostic pitfalls and perception of treatment benefit. Neurology [Internet]. 2015 [cited 2025 Nov 8];85(6): 498-504. Available from: https://pubmed.ncbi.nlm.nih.gov/26180143/ Subscription required
3. Mathey EK, Park SB, Hughes RAC, Pollard JD, Armati PJ, Barnett MH, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry [Internet]. 2015 [cited 2025 Nov 8];86(9):973-85. Available from: https://pubmed.ncbi.nlm.nih.gov/25677463/
4. Doneddu PE, Cocito D, Manganelli F, Fazio R, Briani Ch, Filosto M, et al. Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry [Internet]. 2019 [cited 2025 Nov 8];90(2):125-32. Available from: https://pubmed.ncbi.nlm.nih.gov/30297520/
5. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet [Internet]. 2016 [cited 2025 Nov 8];388(10045):717-27. Available from: https://www.thelancet.com/article/S0140-6736(16)00339-1/fulltext
6. Shahrizaila N, Lehmann HC, Kuwabara S. Guillain-Barré syndrome. Lancet [Internet]. 2021[cited 2025 Nov 8];397(10280):1214-28. Available from: https://pubmed.ncbi.nlm.nih.gov/33647239/ Subscription required
7. Lehmann HCh, Burke D, Kuwabara S. Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment. J Neurol Neurosurg Psychiatry [Internet]. 2019 [cited 2025 Nov 8];90(9):981-7. Available from: https://pubmed.ncbi.nlm.nih.gov/30992333/
8. Querol L, Illa I. Paranodal and other autoantibodies in chronic inflammatory neuropathies. Curr Opin Neurol [Internet]. 2015 [cited 2025 Nov 8];28(5):474-9. Available from: https://pubmed.ncbi.nlm.nih.gov/26263474/
9. Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JChG, Desestret V, Dubey D, et al. Updated diagnostic criteria for paraneoplastic neurologic syndromes. Neurol Neuroimmunol Neuroinflamm [Internet]. 2021 [cited 2025 Nov 8];8(4): e1014. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC8237398/
10. Vogrig A, Gigli GL, Segatti S, Corazza E, Marini A, Bernardini A, et al. Paraneoplastic neuropathies: clinical presentations and management. Curr Treat Options Neurol [Internet]. 2020 [cited 2025 Nov 8];22(12):43. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC8517070/ (Referencia no localizada)
11. Oaklander AL, Lunn MP, Hughes RA, van Schaik IN, Frost Ch, Chalk CH. Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst Rev [Internet]. 2017 [cited 2025 Nov 8];1(1):CD010369. Available from: https://pubmed.ncbi.nlm.nih.gov/28084646/
12. Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Engl J Med [Internet]. 2003 [cited 2025 Nov 8];349(16):1543-54. Available from: https://pubmed.ncbi.nlm.nih.gov/14561798/
13. Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol [Internet]. 2019 [cited 2025 Nov 8];15(11):671-83. Available from: https://pubmed.ncbi.nlm.nih.gov/31541214/
14. Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology [Internet]. 2011 [cited 2025 Nov 8];36(2):123-33. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC5703046/
15. Fokke Ch, van den Berg B, Drenthen J, Walgaard Ch, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain [Internet]. 2014 [cited 2025 Nov 8];137(Pt 1):33-43. Available from: https://www.gbs-cidp.org/wp-content/uploads/2014/01/2014-Fokke-GBS-Brighton-Brain-2014.pdf
16. Hughes RA, Brassington R, Gunn AA, van Doorn PA. Corticosteroids for Guillain-Barré syndrome. Cochrane Database Syst Rev [Internet]. 2016 [cited 2025 Nov 8];10(10):CD001446. Available from: https://pubmed.ncbi.nlm.nih.gov/27775812/
17. Rajabally YA, Nicolas G, Piéret F, Bouche P, Van den Bergh PYK. Validity of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy: a multicentre European study. J Neurol Neurosurg Psychiatry [Internet]. 2009 [cited 2025 Nov 8];80(12):1364-8. Available from: https://www.researchgate.net/publication/26686112_Validity_of_diagnostic_criteria_for_chronic_inflammatory_demyelinating_polyneuropathy_A_multicentre_European_study
18. Kuwabara S, Misawa S. Chronic inflammatory demyelinating polyneuropathy: clinical subtypes and their correlation with electrophysiology. Clin Exp Neuroimmunol [Internet]. 2011 [cited 2025 Nov 8];2(2):41-8. Available from: https://onlinelibrary.wiley.com/doi/full/10.1111/j.1759-1961.2011.00020.x
19. Zekeridou A, Lennon VA. Neurologic autoimmunity in the era of checkpoint inhibitor cancer immunotherapy. Mayo Clin Proc [Internet]. 2019 [cited 2025 Nov 8];94(9):1865-78. Available from: https://pubmed.ncbi.nlm.nih.gov/31358366/
20. Vogrig A, Fouret M, Joubert B, Picard G, Rogemond V, Pinto AL, et al. Increased frequency of anti-Ma2 encephalitis associated with immune checkpoint inhibitors. Neurol Neuroimmunol Neuroinflamm [Internet]. 2019 [cited 2025 Nov 8];6(6): e604. Available from: https://www.neurology.org/doi/10.1212/NXI.0000000000000604
21. Cortese I, Chaudhry V, So YT, Cantor F, Cornblath DR, Rae-Grant A. Evidence-based guideline update: plasmapheresis in neurologic disorders. Neurology [Internet]. 2011 [cited 2025 Nov 8];76(3):294-300. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC3034395/
22. Graus F, Dalmau J. Paraneoplastic neurological syndromes in the era of immune-checkpoint inhibitors. Nat Rev Clin Oncol [Internet]. 2019 [cited 2025 Nov 8];16(9):535-48. Available from: https://pubmed.ncbi.nlm.nih.gov/30867573/ Subscription required
23. Lehmann HC, Wunderlich G, Fink GR, Sommer C. Diagnosis of peripheral neuropathy. Neurol Res Pract. 2020 Jul 15;2:20. doi: 10.1186/s42466-020-00064-2. Available from: https://pubmed.ncbi.nlm.nih.gov/33324924/
24. Dimachkie MM, Barohn RJ. Chronic inflammatory demyelinating polyneuropathy. Curr Treat Options Neurol [Internet]. 2013 [cited 2025 Nov 8];15(3):350-66. Available from: https://pubmed.ncbi.nlm.nih.gov/23564314/
25. Maudes E, Landa J, Muñoz-Lopetegi A, Armangue T, Alba M, Saiz A, et al. Clinical significance of Kelch-like protein 11 antibodies. Neurol Neuroimmunol Neuroinflamm [Internet]. 2020 [cited 2025 Nov 8];7(3):e666. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC7051195/
26. Devine MF, Kothapalli N, Elkhooly M, Dubey D. Paraneoplastic neurological syndromes: clinical presentations and management. Ther Adv Neurol Disord [Internet]. 2021 [cited 2025 Nov 8];14:1756286420985323. Available from: https://pubmed.ncbi.nlm.nih.gov/33796141/
27. Harahsheh E, Hammami MB, Gupta P, Costello BA, Leibovich B, Cheville JC, et al. Testicular cancer–associated paraneoplastic neurologic syndromes. JAMA Netw Open [Internet]. 2025 [cited 2025 Dec 8];8(10):e e2538584. Available from: https://pubmed.ncbi.nlm.nih.gov/41118166/
28. Dalmau J, Rosenfeld MR. Paraneoplastic syndromes of the CNS. Lancet Neurol [Internet]. 2008 [cited 2025 Nov 8];7(4):327-40. Available from: https://pubmed.ncbi.nlm.nih.gov/18339348/
29. Dimitrov S, Tsalta-Mladenov M, Kabakchieva P, Georgiev T, Andonova S. Paraneoplastic Neurological Syndromes: Advances and Future Perspectives in Immunopathogenesis and Management. Antibodies (Basel). 2026 Jan 14;15(1):8. doi: 10.3390/antib15010008. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC12821609/
30. Gilligan M, McGuigan C, McKeon A. Paraneoplastic Neurologic Disorders. Curr Neurol Neurosci Rep. 2023 Mar;23(3):67-82. doi: 10.1007/s11910-023-01250-w. Available from: https://pubmed.ncbi.nlm.nih.gov/36781586/
31. Graus F, Dalmau J. Paraneoplastic neurological syndromes: diagnosis and treatment. Curr Opin Neurol [Internet]. 2007 [cited 2025 Nov 8];20(6):732-7. Available from: https://pubmed.ncbi.nlm.nih.gov/17992098/ Subscription required
32. Titulaer MJ, Soffietti R, Dalmau J, Gilhus NE, Giometto B, Graus F, et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force. Eur J Neurol [Internet]. 2011 [cited 2025 Nov 8];18(1):19-e3. Available from: https://pubmed.ncbi.nlm.nih.gov/20880069/
33. Thakolwiboon S, Zekeridou A. Paraneoplastic neurologic disorders and neurologic complications of immune checkpoint inhibitors. Handb Clin Neurol. 2026;214:85-103. doi: 10.1016/B978-0-323-90887-0.00018-3. Available from: https://pubmed.ncbi.nlm.nih.gov/41526153/
