Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1992 Feb;55(2):116–120. doi: 10.1136/jnnp.55.2.116

Peripheral neuropathy in essential mixed cryoglobulinaemia.

F Gemignani 1, G Pavesi 1, A Fiocchi 1, P Manganelli 1, G Ferraccioli 1, A Marbini 1
PMCID: PMC488973  PMID: 1311372

Abstract

The prevalence of various forms of peripheral neuropathy has not been previously assessed in large series of patients with essential mixed cryoglobulinaemia (EMC). Clinical and electrophysiological signs of peripheral neuropathy were observed in 21 of 37 EMC patients, consisting of polyneuropathy in 19, mononeuropathy or multiple mononeuropathy in eight, and both in six. The various forms of peripheral neuropathy occurred differently in the subgroups of EMC. Isolated polyneuropathy was more common with type II (eight of 10) than type III EMC (two of eight). Multifocal neuropathy, in association with polyneuropathy, was the most common form in type III EMC (five of eight). Patients with peripheral neuropathy and type II EMC were significantly older than type II EMC patients without neuropathy, regarding present age and age of onset of EMC. Patients with peripheral neuropathy and type III EMC tended to have higher values of ESR and IgM than type III EMC patients without neuropathy. Electrophysiological findings and sural nerve biopsy specimens (nine cases) showed prominent axonal changes. Vascular changes included vasculitis and alterations of the endoneurial microvessels in type II and type III EMC. Our findings suggest that distinct pathogenic factors are implicated in the subgroups of cryoglobulinaemic neuropathy, possibly inducing different types of vascular changes underlying polyneuropathy or, respectively, mononeuropathy and multiple mononeuropathy.

Full text

PDF

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Betourné C., Buge A., Dechy H., Dorra M., Dournon E., Rancurel G. Neuropathies périphériques au cours d'un myélome à IgA et d'une cryoglobulinémie mixte. Traitement par plasmaphérèses itératives. Nouv Presse Med. 1980 Apr 26;9(19):1369–1371. [PubMed] [Google Scholar]
  2. Brouet J. C., Clauvel J. P., Danon F., Klein M., Seligmann M. Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med. 1974 Nov;57(5):775–788. doi: 10.1016/0002-9343(74)90852-3. [DOI] [PubMed] [Google Scholar]
  3. Cavaletti G., Petruccioli M. G., Crespi V., Pioltelli P., Marmiroli P., Tredici G. A clinico-pathological and follow up study of 10 cases of essential type II cryoglobulinaemic neuropathy. J Neurol Neurosurg Psychiatry. 1990 Oct;53(10):886–889. doi: 10.1136/jnnp.53.10.886. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Chad D., Pariser K., Bradley W. G., Adelman L. S., Pinn V. W. The pathogenesis of cryoglobulinemic neuropathy. Neurology. 1982 Jul;32(7):725–729. doi: 10.1212/wnl.32.7.725. [DOI] [PubMed] [Google Scholar]
  5. Cream J. J., Hern J. E., Hughes R. A., MacKenzie I. C. Mixed or immune complex cryoglobulinaemia and neuropathy. J Neurol Neurosurg Psychiatry. 1974 Jan;37(1):82–87. doi: 10.1136/jnnp.37.1.82. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Dayan A. D., Lewis P. D. Demyelinating neuropathy in macrocryoglobulinemia. Neurology. 1966 Nov;16(11):1141–1144. doi: 10.1212/wnl.16.11.1141. [DOI] [PubMed] [Google Scholar]
  7. Dyck P. J., Benstead T. J., Conn D. L., Stevens J. C., Windebank A. J., Low P. A. Nonsystemic vasculitic neuropathy. Brain. 1987 Aug;110(Pt 4):843–853. doi: 10.1093/brain/110.4.843. [DOI] [PubMed] [Google Scholar]
  8. Dyck P. J., Johnson W. J., Lambert E. H., O'Brien P. C. Segmental demyelination secondary to axonal degeneration in uremic neuropathy. Mayo Clin Proc. 1971 Jun;46(6):400–431. [PubMed] [Google Scholar]
  9. Farivar M., Wands J. R., Benson G. D., Dienstag J. L., Isselbacher K. J. Cryoprotein complexes and peripheral neuropathy in a patient with chronic active hepatitis. Gastroenterology. 1976 Sep;71(3):490–493. [PubMed] [Google Scholar]
  10. Feiner H. D. Relationship of tissue deposits of cryoglobulin to clinical features of mixed cryoglobulinemia. Hum Pathol. 1983 Aug;14(8):710–715. doi: 10.1016/s0046-8177(83)80144-0. [DOI] [PubMed] [Google Scholar]
  11. Garcia-Bragado F., Fernandez J. M., Navarro C., Villar M., Bonaventura I. Peripheral neuropathy in essential mixed cryoglobulinemia. Arch Neurol. 1988 Nov;45(11):1210–1214. doi: 10.1001/archneur.1988.00520350048015. [DOI] [PubMed] [Google Scholar]
  12. Gemignani F., Marbini A., Bragaglia M. M., Govoni E. Pathological study of the sural nerve in Fabry's disease. Eur Neurol. 1984;23(3):173–181. doi: 10.1159/000115700. [DOI] [PubMed] [Google Scholar]
  13. Gorevic P. D., Kassab H. J., Levo Y., Kohn R., Meltzer M., Prose P., Franklin E. C. Mixed cryoglobulinemia: clinical aspects and long-term follow-up of 40 patients. Am J Med. 1980 Aug;69(2):287–308. doi: 10.1016/0002-9343(80)90390-3. [DOI] [PubMed] [Google Scholar]
  14. Géraud G., Laclergue J. P., Bès A., Géraud J. Neuropathies périphériques associées à une cryoglobulinémie. J Neurol Sci. 1978 Feb;35(2-3):225–233. doi: 10.1016/0022-510x(78)90005-9. [DOI] [PubMed] [Google Scholar]
  15. Kissel J. T., Slivka A. P., Warmolts J. R., Mendell J. R. The clinical spectrum of necrotizing angiopathy of the peripheral nervous system. Ann Neurol. 1985 Aug;18(2):251–257. doi: 10.1002/ana.410180213. [DOI] [PubMed] [Google Scholar]
  16. Konishi T., Saida K., Ohnishi A., Nishitani H. Perineuritis in mononeuritis multiplex with cryoglobulinemia. Muscle Nerve. 1982 Feb;5(2):173–177. doi: 10.1002/mus.880050216. [DOI] [PubMed] [Google Scholar]
  17. Lippa C. F., Chad D. A., Smith T. W., Kaplan M. H., Hammer K. Neuropathy associated with cryoglobulinemia. Muscle Nerve. 1986 Sep;9(7):626–631. doi: 10.1002/mus.880090708. [DOI] [PubMed] [Google Scholar]
  18. Migliorini P., Bombardieri S., Castellani A., Ferrara G. B. HLA antigens in essential mixed cryoglobulinemia. Arthritis Rheum. 1981 Jul;24(7):932–936. doi: 10.1002/art.1780240711. [DOI] [PubMed] [Google Scholar]
  19. Moore P. M. Immune mechanisms in the primary and secondary vasculitides. J Neurol Sci. 1989 Nov;93(2-3):129–145. doi: 10.1016/0022-510x(89)90184-6. [DOI] [PubMed] [Google Scholar]
  20. Nemni R., Corbo M., Fazio R., Quattrini A., Comi G., Canal N. Cryoglobulinaemic neuropathy. A clinical, morphological and immunocytochemical study of 8 cases. Brain. 1988 Jun;111(Pt 3):541–552. doi: 10.1093/brain/111.3.541. [DOI] [PubMed] [Google Scholar]
  21. Ng Y. C., Peters D. K., Walport M. J. Monoclonal rheumatoid factor-IgG immune complexes. Poor fixation of opsonic C4 and C3 despite efficient complement activation. Arthritis Rheum. 1988 Jan;31(1):99–107. doi: 10.1002/art.1780310114. [DOI] [PubMed] [Google Scholar]
  22. Ohi T., Kyle R. A., Dyck P. J. Axonal attenuation and secondary segmental demyelination in myeloma neuropathies. Ann Neurol. 1985 Mar;17(3):255–261. doi: 10.1002/ana.410170306. [DOI] [PubMed] [Google Scholar]
  23. Parry G. J. Mononeuropathy multiplex (AAEE case report #11). Muscle Nerve. 1985 Jul-Aug;8(6):493–498. doi: 10.1002/mus.880080603. [DOI] [PubMed] [Google Scholar]
  24. SIGUIER F., GODEAU P., LEVY R., BINET J. L., BEN HAMIDA A PROPOS D'UN CAS DE NEUROPATHIE CRYOGLOBULIN'EMIQUE ('ETUDE CLINIQUE ET BIOLOGIQUE) Sem Hop. 1964 Jul 10;40:1928–1934. [PubMed] [Google Scholar]
  25. Testa D., Salmaggi A., Eoli M., Corbo M., Nemni R. Cryoglobulinemic neuropathy: case report. Ital J Neurol Sci. 1988 Aug;9(4):391–395. doi: 10.1007/BF02334005. [DOI] [PubMed] [Google Scholar]
  26. Thomas P. K., Hollinrake K., Lascelles R. G., O'Sullivan D. J., Baillod R. A., Moorhead J. F., Mackenzie J. C. The polyneuropathy of chronic renal failure. Brain. 1971;94(4):761–780. doi: 10.1093/brain/94.4.761. [DOI] [PubMed] [Google Scholar]
  27. Vallat J. M., Desproges-Gotteron R., Leboutet M. J., Loubet A., Gualde N., Treves R. Cryoglobulinemic neuropathy: a pathological study. Ann Neurol. 1980 Aug;8(2):179–185. doi: 10.1002/ana.410080208. [DOI] [PubMed] [Google Scholar]
  28. Valli G., De Vecchi A., Gaddi L., Nobile-Orazio E., Tarantino A., Barbieri S. Peripheral nervous system involvement in essential cryoglobulinemia and nephropathy. Clin Exp Rheumatol. 1989 Sep-Oct;7(5):479–483. [PubMed] [Google Scholar]
  29. Vital C., Deminière C., Lagueny A., Bergouignan F. X., Pellegrin J. L., Doutre M. S., Clement A., Beylot J. Peripheral neuropathy with essential mixed cryoglobulinemia: biopsies from 5 cases. Acta Neuropathol. 1988;75(6):605–610. doi: 10.1007/BF00686206. [DOI] [PubMed] [Google Scholar]
  30. Wees S. J., Sunwoo I. N., Oh S. J. Sural nerve biopsy in systemic necrotizing vasculitis. Am J Med. 1981 Oct;71(4):525–532. doi: 10.1016/0002-9343(81)90195-9. [DOI] [PubMed] [Google Scholar]
  31. Yasuda H., Dyck P. J. Abnormalities of endoneurial microvessels and sural nerve pathology in diabetic neuropathy. Neurology. 1987 Jan;37(1):20–28. doi: 10.1212/wnl.37.1.20. [DOI] [PubMed] [Google Scholar]
  32. el-Hazmi M. A., Lehmann H. Interaction between iron deficiency and alpha-thalassaemia: the in vitro effect of haemin on alpha-chain synthesis. Acta Haematol. 1978;60(1):1–9. doi: 10.1159/000207689. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES