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Multiple paraneoplastic syndromes in a patient with antibodies to neuronal nucleoproteins (anti-Hu)

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Abstract

We report the clinical and autopsy studies of a patient with an unusual combination of multiple paraneoplastic neurological syndromes in association with antibodies to a 35–40 kDa neuronal nucleoprotein (anti-Hu). Neurological disease preceded the detection of a small cell carcinoma of the lung. The patient had combined sensory and motor neuronopathy or neuropathy, cerebellar degeneration, brain-stem and limbic encephalitis, and clinical evidence of the Lambert-Eaton myasthenic syndrome and gastrointestinal pseudo-obstruction of paraneoplastic origin.

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References

  1. Anderson NE, Rosenblum MK, Graus G, Wiley RG, Posner JB (1988) Autoantibodies in paraneoplastic syndromes associated with small cell lung cancer. Neurology 38:1391–1398

    Google Scholar 

  2. Anderson NE, Rosenblum MK, Posner JB (1988) Paraneoplastic cerebellar degeneration: clinical-immunological correlations. Ann Neurol 24:559–567

    Google Scholar 

  3. Bakheit AMO, Kennedy PGE, Behan PO (1990) Paraneoplastic limbic encephalitis: clinico-pathological correlations. J Neurol Neurosurg Psychiatry 53:1084–1088

    Google Scholar 

  4. Blumenfeld AM, Recht LD, Chad DA, DeGirolami U, Griffin T, Jaeckle KA (1991) Coexistence of Lambert-Eaton myasthenic syndrome and subacute cerebellar degeneration: differential effects of treatment. Neurology 41:1682–1685

    Google Scholar 

  5. Brennan LV, Craddock PR (1983) Limbic encephalitis as a nonmetastatic complication of oat cell lung cancer. Am J Med 75:518–520

    Google Scholar 

  6. Chinn JS, Schuffler MD (1988) Paraneoplastic visceral neuropathy as a cause of severe gastrointestinal motor dysfunction. Gastroenterology 95:1279–1286

    Google Scholar 

  7. Clouston PD, Super CB, Arbizu T, Johnston I, Lang B, Newsom-Davis J, Posner JB (1992) Paraneoplastic cerebellar degeneration III. Cerebellar degeneration, cancer, and the LambertEaton myasthenic syndrome. Neurology 42:1944–1950

    Google Scholar 

  8. Corsellis JAN, Goldberg GJ, Norton RA (1968) “Limbic encephalitis” and its association with carcinoma. Brain 91:481–496

    Google Scholar 

  9. Dalmeau J, Fumeaux HM, Gralla RJ, Kris MG, Posner JB (1990) Detection of the anti-Hu antibody in the serum of patients with small cell lung cancer — a quantitative western blot analysis. Ann Neurol 27:544–552

    Google Scholar 

  10. Dalmeau J, Fumeaux HM, Rosenblum MK, Graus F, Posner JB (1991) Detection of the anti-Hu antibody in specific regions of the nervous system and tumor from patients with paraneoplastic encephalomyelitis/sensory neuronopathy. Neurology 41:1757–1764

    Google Scholar 

  11. Dalmeau J, Fumeaux HM, CordonCardo C, Posner JB (1992) The expression of the Hu paraneoplastic encephalomyelitis/sensory neuronopathy antigen in human normal and tumor tissues. Am J Pathol 141:881–886

    Google Scholar 

  12. Dalmeau J, Graus F, Rosenblum MK, Posner JB (1992) Anti-Hu-associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients. Medicine (Baltimore) 71:59–72

    Google Scholar 

  13. Dropcho EJ, Stanton C, OH SJ (1989) Neuronal antinuclear antibodies in a patient with Lambert-Eaton myasthenic syndrome and small-cell lung carcinoma. Neurology 39:249–251

    Google Scholar 

  14. Furneaux HF, Reich L, Posner JB (1990) Autoantibody synthesis in the central nervous system of patients with paraneoplastic syndromes. Neurology 40:1085–1091

    Google Scholar 

  15. Grauss F, Dalmau J (1991) Enteric neuronal antibodies and pseudo-obstruction. Gastroenterology 101:1142–1144

    Google Scholar 

  16. Graus F, Cordon-Cardo C, Posner JB (1985) Neuronal antinuclear antibody in sensory neuronopathy from lung cancer. Neurology 35:538–543

    Google Scholar 

  17. Greenlee JE; Brashear HR (1983) Antibodies to Purkinje cells in patients with paraneoplastic cerebellar degeneration and ovarian carcinoma. Ann Neurol 14:609–613

    Google Scholar 

  18. Heidenreich F, Thomas C, Wechsler W, Hartung HP (1990) Paraneoplastic Lambert-Eaton myasthenic syndrome, interstitial myositis and posterior column degeneration with anti-Hu antibodies (abstract). J Neurol Sci 98 [Suppl]:309

    Google Scholar 

  19. Henson RA, Urich H (1982) Cancer and the nervous system: the neurological manifestations of systemic malignant disease. Blackwell, Oxford

    Google Scholar 

  20. Jaeckle KA, Graus F, Houghton A, Cordon-Cardo C, Nielson SL, Posner JB (1985) Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen. Ann Neurol 18:592–600

    Google Scholar 

  21. Lennon VA, Sas DF, Busk MF, Scheithauer B, Malagelada J-R, Camilleri M, Miller LJ (1991) Enteric autoantibodies in pseudoobstruction with small-cell lung carcinoma. Gastroenterology 100:137–142

    Google Scholar 

  22. Leveque C, Hoshino T, David P, ShojiKasai Y, Leys K, Omori A, Lang B, El Far O, Sato K, Martin-Moutot N, Newsom-Davis J, Takahashi M, Seagar M (1992) The synaptic vesicle protein synaptotagmin associates with calcium channels and is a putative LambertEaton myasthenic syndrome antigen. Proc Natl Acad Sci USA 89:3625–3629

    Google Scholar 

  23. Leys K, Lang B, Johnston I, Newsom-Davis J (1991) Calcium channel autoantibodies in the Lambert-Eaton myasthenic syndrome. Ann Neurol 29:307–314

    Google Scholar 

  24. O'Neill JH, Murray NM, Newsom-Davis J (1988) The Lambert-Eaton myasthenic syndrome. A review of 50 cases. Brain 111:577–596

    Google Scholar 

  25. Peterson K, Rosenblum MK, Kotanides H, Posner JB (1992) Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody-positive patients. Neurology 42:1931–1937

    Google Scholar 

  26. Posner JB, Furneaux HM (1990) Paraneoplastic neurological syndromes. In: Waksman BH (ed) Immunologic mechanisms in neurologic and psychiatric disease. Raven Press, New York, pp 187–219

    Google Scholar 

  27. Pou Serradell A, Fueyo J, Gomez C, Oliveras C, Espadaler JM, Alameda F, Graus F (1992) Encephalomyelite et syndrome de Lambert-Eaton paraneoplasiques. Rev Neurol (Paris) 148:605–609

    Google Scholar 

  28. Rosenfeld MR, Wong E, Dalmau J, Manley G, Posner JB, Sher E, Furneaux HM (1993) Cloning and characterization of a Lambert-Eaton myasthenic syndrome antigen. Ann Neurol 33:113–120

    Google Scholar 

  29. Sher E, Canal N, Piccolo G, Gotti C, Scoppetta C, Evoli A, Clementi F (1989) Specificity of calcium channel autoantibodies in Lambert Eaton myasthenic syndrome. Lancet II:640–643

    Google Scholar 

  30. Szabo A, Dalmau J, Manley G, Rosenfeld M, Wong E, Henson J, Posner JB, Furneaux HM (1991) HuD, a paraneoplastic encephalomyelitis antigen, contains RNA-binding domains and is homologous to Elav and Sex-lethal. Cell 67:325–333

    Google Scholar 

  31. Tsukamoto T, Yamamoto H, Iwasaki Y, Yoshie O, Terunuma H, Suzuki H (1989) Antineural autoantibodies in patients with paraneoplastic cerebellar degeneration. Arch Neurol 46:1225–1229

    Google Scholar 

  32. Vincent A, Lang B, Newsom-Davis J (1989) Autoimmunity to the voltagegated calcium channel underlies the Lambert-Eaton myasthenic syndrome, a paraneoplastic disorder. Trends Neurol Sci 12:496–502

    Google Scholar 

  33. Zweifel TJ, Albers JW (1980) Multiple neurologic paraneoplastic syndromes. Report of a case. Arch Neurol 37:315–316

    Google Scholar 

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Heidenreich, F., Schober, R., Brinck, U. et al. Multiple paraneoplastic syndromes in a patient with antibodies to neuronal nucleoproteins (anti-Hu). J Neurol 242, 210–216 (1995). https://doi.org/10.1007/BF00919593

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  • DOI: https://doi.org/10.1007/BF00919593

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