Neuroimmunology in Clinical Practice - part 6 pdf

132 THOMAS H. BRANNAGAN III (Rechthand et al., 1984). CIDP can occur with HIV infection and occurs in both the early and late stages of infection (Cornblath et al., 1987). Diabetes mellitus or Charcot–Marie –Tooth Disease (CMT), with superimposed CIDP Patients with diabetes mellitus and CMT may develop a superimposed CIDP which is a treatable condition. Both diabetes mellitus and CMT may predispose patients to CIDP, as a superimposed and treatable condition (Dyck et al., 1982b; Gorson et al., 2000; Sharma et al., 2002; Shy et al., 1997; Stewart et al., 1996; Vital et al., 2003). Subacute demyelinating polyneuropathy Patients with an intermediate time course of pro- gression, from 4–8 weeks, have been designated subacute inflammatory demyelinating neuropathy. This entity was first coined by Oh (Oh, 1977), and the same author and others have described subsequent patients (Hughes et al., 1992; Oh et al., 2003). Pati- ents respond to plasmapheresis, IVIg, and unlike Guillain–Barré syndrome, also improve with corticosteroids. Some patients have a monophasic course and other develop a chronic disorder. In the recent series by Oh, compared with CIDP, patients with subacute inflammatory demyelinating polyneuropathy had a higher incidence of preceding viral illnesses, a lower rate of relapses, and a higher rate of complete recovery to normal. Compared to patients with GBS, however, there is a lower rate of antecedent infection (39% versus 67%) and a higher rate of relapses (17% versus 3%) (Oh et al., 2003). References Adams, R.D., Asbury, A.K. and Michelson, J.J. 1965. Multifocal pseudohypertrophic neuropathy. Trans Am Neurol Assoc, 90, 30–4. Austin, J.H. 1958. Recurrent polyneuropathies and their corticosteroid treatment with 5-year observa- tions of a placebo-controlled case treated with corti- cotrophin, cortisone, and prednisone. Brain, 81, 157. Azulay, J.P., Blin, O., Pouget, J. et al. 1994. Intravenous immunoglobulin treatment in patients with motor- neuron syndromes associated with anti-Gm1 antibodies – A double-blind, placebo-controlled study. Neurology, 44, 429–32. Azulay, J.P., Rihet, P., Pouget, J. et al. 1997. Long-term follow up of multifocal motor neuropathy with conduction block under treatment. J Neurol Neurosurg Psych, 62, 391–4. Barohn, R.J. Approach to peripheral neuropathy and neuronopathy. Seminars Neurol, 1998;18, 7–18. Barohn, R.J., Kissel, J.T., Warmolts, J.R. and Mendell, J.R. 1989. Chronic inflammatory demyelinating polyradiculoneuropathy – Clinical characteristics, course, and recommendations for diagnostic-criteria. Arch Neurol, 46, 878–84. Barohn, R.J., Warmolts, J.R., Kissel, J.T. and Mendell, J.R. 1987. Electrophysiologic findings in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve, 10, 650–1. Bedlack, R.S., Vu, T., Hammans, S. et al. 2004. MNGIE neuropathy, five cases mimicking chronic inflammatory demyelinating polyneuropathy. Muscle Nerve, 29, 364–8. Berger, A.R., Bradley, W.G., Brannagan, T.H. et al. 2003. Guidelines for the diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy. J Periph Nerv Sys, 8, 282–4. Berger, A.R., Herskovitz, S. and Kaplan, J. 1995. Late motor involvement in cases presenting as chronic sensory demyelinating polyneuropathy. Muscle Nerve, 18, 440–4. Beydoun, S.R. and Copeland, D. 2000. Bilateral phrenic neuropathy as a presenting feature of multifocal motor neuropathy with conduction block. Muscle Nerve, 23, 556–9. Bird, S.J., Brown, M.J., Shy, M.E. and Scherer, S.S. 1996. Chronic inflammatory demyelinating polyneuropathy associated with malignant melanoma. Neurology, 46, 822–4. Bosboom, W.M., van den Berg, L.H., Mollee, I. et al. 2001. Sural nerve T-cell receptor Vbeta gene utilization in chronic inflammatory demyelinating polyneuropathy and vasculitic neuropathy. Neurology, 56, 74–81. Bouchard, C., Lacroix, C., Plante, V. et al. 1999. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neuro- logy, 52, 498–503. Boukhris, S., Magy, L., Kabore, R. et al. 2004. Atypical electrophysiologic findings in chronic inflammatory demyelinating polyneuropathy (CIDP) – Diagnosis confirmed by nerve biopsy. Neurophysiol Clin, 34, 71–9. Brannagan, T.H. 2002. Intravenous gammaglobulin (IVIg) for treatment of CIDP and related immune- mediated neuropathies. Neurology, 59, S33–S40. Brannagan, T.H. and Latov, N. 2005. Motor neuropathy and monoclonal gammopathy. In D.S. Younger (ed.), Motor Disorders, Lippincott Williams and Wilkins, Philadelphia, pp. 343–8. Brannagan, T.H., Nagle, K.J., Lange, D.J. and Rowland, L.P. 1996. Complications of intravenous immune globulin treatment in neurologic disease. Neurology, 47, 674–7. Brannagan, T.H., Pradhan, A., Heiman-Patterson, T. et al. 2002. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology, 58, 1856–8. NICP_C07 04/05/2007 02:44PM Page 132 Immune-mediated chronic demyelinating polyneuropathies 133 Briani, C., Brannagan, T.H., Trojaborg, W. and Latov, N. 1996. Chronic inflammatory demyelinating polyneuropathy. Neuromus Dis, 6, 311–25. Bromberg, M.B. 1991. Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy. Muscle Nerve, 14, 968–76. Brown, W.F. and Watson, B.V. 2002. Recording of electrical activity in nerve trunks and conduction in human sensory and motor nerve fibers. In W.F. Brown, C.F. Bolton and M.J. Aminoff (eds.), Neuromuscular Function and Disease, W.B. Sanders, Philadelphia, pp. 21–55. Buchthal, F. and Behse, F. 1977. Peroneal muscular atrophy (PMA) and related disorders. I. Clinical manifestations as related to biopsy findings, nerve conduction and electromyography. Brain, 100(1), 41–66. Carpo, M., Cappellari, A., Mora, G. et al. 1998. Deteriora- tion of multifocal motor neuropathy after plasma exchange. Neurology, 50, 1480–2. Chad, D.A., Hammer, K. and Sargent, J. 1986. Slow resolution of multifocal weakness and fasciculation – A reversible motor-neuron syndrome. Neurology, 36, 1260–3. Chaudhry, V., Cornblath, D.R., Griffin, J.W., O’Brien, R. and Drachman, D.B. 2001. Mycophenolate mofetil, A safe and promising immunosuppressant in neuromuscular diseases. Neurology, 56, 94–6. Chaudhry, V., Corse, A.M., Cornblath, D.R. et al. 1993. Multifocal motor neuropathy – Response to human immune globulin. Ann Neurol, 33, 237–42. Chaudhry, V., Corse, A.M., Cornblath, D.R., Kuncl, R.W., Freimer, M.L. and Griffin, J.W. 1994. Multifocal motor neuropathy – Electrodiagnostic features. Muscle Nerve, 17, 198–205. Chin, R.L., Latov, N., Sander, H.W. et al. 2004. Chronic inflammatory demyelinating polyneuropathy presenting as cryptogenic sensory polyneuropathy. J Periph Nerv Sys, 9, 128–33. Comi, G., Roveri, L., Swan, A., Willison, H. et al. 2002. A randomised controlled trial of intravenous immunoglobulin in IgM paraprotein associated demyelinating neuropathy. J Neurol, 249, 1370–7. Cornblath, D.R. 1991. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Neurology, 41, 617–18. Cornblath, D.R., McArthur, J.C., Kennedy, P.G., Witte, A.S. and Griffin, J.W. 1987. Inflammatory demyelinating peripheral neuropathies associated with human T-cell lymphotropic virus type III infection. Ann Neurol, 21, 32–40. Dalakas, M.C. and Engel, W.K. 1980. Immunoglobulin and complement deposits in nerves of patients with chronic relapsing polyneuropathy. Arch Neurol, 37, 637–40. Dalakas, M.C. and Engel, W.K. 1981. Chronic relapsing (dysimmune) polyneuropathy – Pathogenesis and treatment. Ann Neurol, 9, 134–45. Dalakas, M.C., Quarles, R.H., Farrer, R.G. et al. 1996. A controlled study of intravenous immunoglobulin in demyelinating neuropathy with IgM gammopathy. Ann Neurol, 40, 792–5. David, S., Hila, S., Fosbrink, M., Rus, H. and Koski, C.L. 2006. JNK1 activation mediates C5b-9-induced P0 mRNA instability and P0 gene expression in Schwann cells. J Periph Nerv Sys, 11, 77–87. Dispenzieri, A., Kyle, R.A., Lacy, M.Q. et al. 2003. POEMS syndrome, definitions and long-term outcome. Blood, 101, 2496–506. Donaghy, M. and Earl, C.J. 1985. Ocular palsy preceding chronic relapsing polyneuropathy by several weeks. Ann Neurol, 17, 49–50. Donaghy, M., Mills, K.R., Boniface, S.J. et al. 1994. Pure motor demyelinating neuropathy – Deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychi- atry, 57, 778–83. Drachman, D.B. 1990. How to recognize an antibody- mediated autoimmune disease, criteria. Research Publications – Association for Research in Nervous and Mental Disease, 68, 183–6. Dyck, P.J. and Dyck, P.J.B. 2000. Atypical varieties of chronic inflammatory demyelinating neuropathies. Lancet, 355, 1293–4. Dyck, P.J., Lais, A.C., Ohta, M., Bastron, J.A., Okazaki, H. and Groover, R.V. 1975. Chronic inflammatory polyradiculoneuropathy. Mayo Clinic Proc, 50, 621– 37. Dyck, P.J., O’Brien, P.C., Oviatt, K.F. et al. 1982a. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol, 11, 136–41. Dyck, P.J., Oviatt, K.F. and Lambert, E.H. 1981. Intensive evaluation of referred unclassified neuropathies yields improved diagnosis. Ann Neurol, 10, 222–6. Dyck, P.J., Pineda, A., Daube, J. et al. 1984. Plasma- exchange in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), A controlled, double- blind trial. Ann Med Interne (Paris), 135, 9. Dyck, P.J., Swanson, C.J., Low, P.A., Bartleson, J.D. and Lambert, E.H. 1982b. Prednisone-responsive hereditary motor and sensory neuropathy. Mayo Clinic Proc, 57, 239–46. Evangelista, T., Carvalho, M., Conceicao, I., Pinto, A. and Luis, M.D.S. 1996. Motor neuropathies mimicking amyotrophic lateral sclerosis motor neuron disease. J Neurol Sci, 139, 95–8. Feasby, T.E., Hahn, A.F., Koopman, W.J. and Lee, D.H. 1990. Central lesions in chronic inflammatory demyelinating polyneuropathy – An MRI study. Neurology, 40, 476–8. Federico, P., Zochodne, D.W., Hahn, A.F., Brown, W.F. and Feasby, T.E. 2000. Multifocal motor neuropathy improved by IVIg – Randomized, doubleblind, placebo-controlled study. Neurology, 55, 1256– 62. NICP_C07 04/05/2007 02:44PM Page 133 134 THOMAS H. BRANNAGAN III Feeney, D.J., Pollard, J.D., Mcleod, J.G., Stewart, G.J. and Doran, T.J. 1990. HLA antigens in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psych, 53, 170–2. Feldman, E.L., Bromberg, M.B., Albers, J.W. and Pestronk, A. 1991. Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol, 30, 397–401. Finsterer, J. and Derfler, K. 1999. Immunoadsorption in multifocal motor neuropathy. J Immunother, 22, 441–2. Gladstone, D.E., Prestrud, A.A. and Brannagan, T.H. III. 2005. High-dose cyclophosphamide results in long- term disease remission with restoration of a normal quality of life in patients with severe refractory chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Sys, 10, 11–16. Gondim, F.A., Brannagan, T.H. III, Sander, H.W., Chin, R.L. and Latov, N. 2005. Peripheral neuropathy in patients with inflammatory bowel disease. Brain, 128, 867–79. Good, J.L., Chehrenama, M., Mayer, R.F. and Koski, C.L. 1998. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neuro- logy, 51, 1735–8. Gordon, P.H., Brannagan, T.H. and Latov, N. 1998. Neurological manifestations of paraproteinemia and cryoglobulinemia. In M.J. Aminoff and C.G. Goetz (eds.), Handbook of Clinical Neurology, Elsevier Science, pp. 431–59. Gorson, K.C., Allam, G. and Ropper, A.H. 1997. Chronic inflammatory demyelinating polyneuropathy: Clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology, 48, 321–8. Gorson, K.C., Amato, A.A. and Ropper, A.H. 2004. Efficacy of mycophenolate mofetil in patients with chronic immune demyelinating polyneuropathy. Neurology, 63, 715–17. Gorson, K.C. and Ropper, A.H. 2003. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): A review of clinical syndromes and treatment approaches in clinical practice. J Clin Neuromusc Dis, 4, 174–89. Gorson, K.C., Ropper, A.H., Adelman, L.S. and Weinberg, D.H. 2000. Influence of diabetes mellitus on chronic inflammatory demyelinating polyneuropathy. Muscle Nerve, 23, 37–43. Gorson, K.C., Ropper, A.H. and Weinberg, D.H. 1999. Upper limb predominant, multifocal chronic inflammatory demyelinating polyneuropathy. Muscle Nerve, 22, 758–65. Gorson, K.C., Ropper, A.H., Weinberg, D.H. and Weinstein, R. 2001. Treatment experience in patients with anti–myelin–associated glycoprotein neuropathy. Muscle Nerve, 24, 778–86. Hahn, A.F., Bolton, C.F., Pillay, N. et al. 1996a. Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study. Brain, 119, 1055– 66. Hahn, A.F., Bolton, C.F., Zochodne, D. and Feasby, T.E. 1996b. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study. Brain, 119, 1067–77. Hahn, A.F., Hartung, H.P. and Dyck, P.J. 2005. Chronic inflammatory demyelinating polyradiculoneuropathy. In P.J. Dyck and P.K. Thomas (eds.), Peripheral Neuropathy, Elsevier Saunders, Philadel- phia, pp. 2221–53. Haq, R.U., Fries, T.J., Pendlebury, W.W., Kenny, M.J., Badger, G.J. and Tandan, R. 2000. Chronic inflammatory demyelinating polyradiculoneuropathy – A study of proposed electrodiagnostic and histologic criteria. Arch Neurol, 57, 1745–50. Harvey, G.K., Gold, R., Hartung, H.P. and Toyka, K.V. 1995. Non-neural-specific T lymphocytes can orche- strate inflammatory peripheral neuropathy. Brain, 118, 1263–72. Hattori, N., Misu, K., Koike, H. et al. 2001. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci, 184, 57–63. Hays, A.P., Lee, S.S.L. and Latov, N. 1988. Immune reactive C3D on the surface of myelin sheaths in neuropathy. J Neuroimmunol, 18, 231–44. Hughes, R., Bensa, S., Willison, H. et al. 2001. Random- ized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol, 50, 195–201. Hughes, R., Sanders, E., Hall, S., Atkinson, P., Colchester, A. and Payan, P. 1992. Subacute idiopathic demyelinating polyradiculoneuropathy. Arch Neurol, 49, 612–16. Hughes, R.A., Allen, D., Makowska, A. and Gregson, N.A. 2006a. Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst, 11, 30–46. Hughes, R.A., Bouche, P., Cornblath, D.R. et al. 2006b. Response to comment on European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst, 11, 90–1. Jung, S., Gaupp, S., Korn, T., Kollner, G., Hartung, H.P. and Toyka, K.V. 2004. Biphasic form of experimental autoimmune neuritis in dark Agouti rats and its oral therapy by antigen-specific tolerization. J Neurosci Res, 75, 524–35. Kaji, R., Mezaki, T., Hirota, N., Kimura, J. and Shibasaki, H. 1994. Multifocal motor neuropathy with exaggerated deep tendon reflex. Neurology, 44, A180. Kaji, R., Oka, N., Tsuji, T. et al. 1993. Pathological findings at the site of conduction block in multifocal motor neuropathy. Ann Neurol, 33, 152–8. NICP_C07 04/05/2007 02:44PM Page 134 Immune-mediated chronic demyelinating polyneuropathies 135 Kaji, R., Shibasaki, H. and Kimura, J. 1992. Multifocal demyelinating motor neuropathy – Cranial nerve involvement and immunoglobulin therapy. Neurology, 42, 506–9. Kaku, D.A., England, J.D. and Sumner, A.J. 1994. Distal accentuation of conduction slowing in polyneuropathy associated with antibodies to myelin- associated glycoprotein and sulfated glucuronyl paragloboside. Brain, 117, 941–7. Kanda, T., Numata, Y. and Mizusawa, H. 2004. Chronic inflammatory demyelinating polyneuropathy: De- creased claudin-5 and relocated ZO-1. J Neurol Neurosurg Psych, 75, 765–9. Katz, J.S., Saperstein, D.S., Gronseth, G., Amato, A.A. and Barohn, R.J. 2000. Distal acquired demyelinating symmetric neuropathy. Neurology, 54, 615–20. Kelly, J.J. 1990. The electrodiagnostic findings in polyneuropathies associated with Igm monoclonal gammopathies. Muscle Nerve, 13, 1113–17. Kelly, J.J., Kyle, R.A., Miles, J.M. and Dyck, P.J. 1983. Osteosclerotic myeloma and peripheral neuropathy. Neurology, 33, 202–10. Kiefer, R., Kieseier, B.C., Stoll, G. and Hartung, H.P. 2001. The role of macrophages in immune-mediated damage to the peripheral nervous system. Prog Neurobiol, 64, 109–27. Kinsella, L.J., Lange, D.J., Trojaborg, W., Sadiq, S.A., Younger, D.S. and Latov, N. 1994. Clinical and electrophysiologic correlates of elevated anti-Gm(1) antibody-titers. Neurology, 44, 1278–82. Kissel, J.T. 2003. The treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Sem Neurol, 23, 169–79. Koller, H., Kieseier, B.C., Jander, S. and Hartung, H.P. 2005. Chronic inflammatory demyelinating polyneuropathy. New Eng J Med, 352, 1343–56. Krarup, C. and Sethi, R.K. 1989. Idiopathic brachial– plexus lesion with conduction block of the ulnar nerve. Electroenceph Clin Neurophys, 72, 259–67. Krarup, C. and Trojaborg, W. 1996. Sensory patho- physiology in chronic acquired demyelinating neuropathy. Brain, 119, 257–70. Kuwabara, S., Misawa, S., Kanai, K. et al. 2006. Auto- logous peripheral blood stem cell transplantation for POEMS syndrome (Reprinted). Neurology, 66, 105–7. Lange, D.J., Trojaborg, W., Latov, N. et al. 1992. Multifocal motor neuropathy with conduction block – Is it a distinct clinical entity. Neurology, 42, 497–505. Latov, N. 2002. Diagnosis of CIDP. Neurology, 59, S2–S6. Latov, N., Hays, A.P., Donofrio, P.D. et al. 1988a. Monoclonal Igm with unique specificity to gangliosides Gm1 and Gd1B and to lacto-N-Tetraose associated with human motor neuron disease. Neurology, 38, 763–8. Latov, N., Hays, A.P. and Sherman, W.H. 1988c. Peripheral neuropathy and anti-MAG antibodies. Crit Rev Neurobiol, 3, 301–32. Latov, N. and Renaud, S. 2004. Effector mechanisms in anti-MAG antibody-mediated and other demyelinating neuropathies. J Neurol Sci, 220, 127–9. Lee, G., Brannagan, T.H., Chin, R.L., Sander, H., Latov, D. and Latov, N. 2006. CD1e polymorphism is associated with increased incidence of CIDP. Neurology, 66, A45. Leger, J.M., Chassande, B., Musset, L., Meininger, V., Bouche, P. and Baumann, N. 2001. Intravenous immunoglobulin therapy in multifocal motor neuropathy, a double-blind, placebo-controlled study. Brain, 124, 145–53. Levine, T.D. and Pestronk, A. 1999. IgM antibody- related polyneuropathies, B-cell depletion chemo- therapy using Rituximab. Neurology, 52, 1701–4. Lewis, R.A. and Sumner, A.J. 1982. The electrodiagnostic distinctions between chronic familial and acquired demyelinative neuropathies. Neurology, 32, 592–6. Lewis, R.A., Sumner, A.J., Brown, M.J. and Asbury, A.K. 1982. Multifocal demyelinating neuropathy with persistent conduction block. Neurology, 32, 958–64. Lunn, M.P.T., Crawford, T.O., Hughes, R.A.C., Griffin, J.W. and Sheikh, K.A. 2002. Anti-myelin-associated glycoprotein antibodies alter neurofilament spacing. Brain, 125, 904–11. Lunn, M.P.T., Manji, H., Choudhary, P.P., Hughes, R.A.C. and Thomas, P.K. 1999. Chronic inflammatory demyelinating polyradiculoneuropathy, a prevalence study in south east England. J Neurol Neurosurg Psych, 66, 677–80. Magda, P., Latov, N., Brannagan, T.H., Weimer, L.H., Chin, R.L. and Sander, H.W. 2003. Comparison of electrodiagnostic abnormalities and criteria in a cohort of patients with CIDP. Arch Neurol, 60, 1755–9. Magistris, M. and Roth, G. 1992. Motor neuropathy with multifocal persistent conduction blocks. Muscle Nerve, 15, 1056–7. Maurer, M., Toyka, K.V. and Gold, R. 2002. Immune mechanisms in acquired demyelinating neuropathies, lessons from animal models. Neuromusc Disord, 12, 405–14. McCombe, P.A., Pollard, J.D. and Mcleod, J.G. 1987. Chronic inflammatory demyelinating polyradiculoneuropathy – A clinical and electrophysiological study of 92 cases. Brain, 110, 1617–30. McLeod, J.G., Pollard, J.D., Macaskill, P., Mohamed, A., Spring, P. and Khurana, V. 1999. Prevalence of chronic inflammatory demyelinating polyneuropathy in New South Wales, Australia. Ann Neurol, 46, 910–13. Mehndiratta, M.M. and Hughes, R.A. 2002. Corti- costeroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev, CD002062. Mendell, J.R., Barohn, R.J., Freimer, M.L. et al. 2001. Randomized controlled trial of IVIg in untreated NICP_C07 04/05/2007 02:44PM Page 135 136 THOMAS H. BRANNAGAN III chronic inflammatory demyelinating polyradiculoneuropathy. Neurology, 56, 445–9. Midroni, G. and Dyck, P.J. 1996. Chronic inflammatory demyelinating polyradiculoneuropathy: Unusual clinical features and therapeutic responses. Neurology, 46, 1206–12. Mokrzycki, M.H. and Kaplan, A.A. 1994. Therapeutic plasma exchange, complications and management. Am J Kidney Dis, 23, 817–27. Murata, K. and Dalakas, M.C. 2000. Expression of the costimulatory molecule BB-1, the ligands CTLA-4 and CD28 and their mRNAs in chronic inflammatory demyelinating polyneuropathy. Brain, 123, 1660–6. Mygland, A. and Monstad, P. 2001. Chronic polyneuropathies in Vest-Agder, Norway. European J Neurol, 8, 157–65. Nagamatsu, M., Terao, S., Misu, K. et al. 1999. Axonal and perikaryal involvement in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psych, 66, 727–33. Nicolas, G., Maisonobe, T., Le Forestier, N., Leger, J.M. and Bouche, P. 2002. Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve, 25, 26–30. Nobile-Orazio, E., Meucci, N., Baldini, L., Di Troia, A. and Scarlato, G. 2000. Long-term prognosis of neuropathy associated with anti-MAG IgM M- proteins and its relationship to immune therapies. Brain, 123, 710–17. Nobile-Orazio, E., Latov, N., Hays, A.P. et al. 1984. Neuropathy and anti-MAG antibodies without detect- able serum M-protein. Neurology, 34, 218–21. Nobile-Orazio, E., Marmiroli, P., Baldini, L. et al. 1987. Peripheral neuropathy in macroglobulinemia – Incidence and antigen-specificity of M-proteins. Neurology, 37, 1506–14. Oh, S.J. 1977. Subacute demyelinating neuropathy responding to steroid treatment. Neurology, 27, 363–4. Oh, S.J., Joy, J.L. and Kuruoglu, R. 1992. Chronic sensory demyelinating neuropathy – Chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psych, 55, 677–80. Oh, S.J., Kurokawa, K., de Almeida, D.F., Ryan, H.F. and Claussen, G.C. 2003. Subacute inflammatory demyelinating polyneuropathy. Neurology, 61, 1507– 12. Ohkoshi, N., Harada, K., Nagata, H., Yato, M. and Shoji, S. 2001. Ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy: Clinical features and pathological study of the sural nerves. European J Neurol, 45, 241–8. Pakalnis, A., Drake, M.E., Barohn, R.J., Chakeres, D.W. and Mendell, J.R., 1988. Evoked-potentials in chronic inflammatory demyelinating polyneuropathy. Arch Neurol, 45, 1014–16. Parry, G.J. 1992. Motor neuropathy with multifocal persistent conduction blocks (a reply). Muscle Nerve, 15, 1057. Parry, G.J. and Clarke, S. 1988. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle Nerve, 11, 103–7. Pedersen, S.F., Pullman, S.L., Latov, N. and Brannagan, T.H. 1997. Physiological tremor analysis of patients with anti-myelin-associated glycoprotein associated neuropathy and tremor. Muscle Nerve, 20, 38–44. Pestronk, A., Cornblath, D.R., Ilyas, A.A. et al. 1988. A treatable multifocal motor neuropathy with antibodies to Gm1 ganglioside. Ann Neurol, 24, 73–8. Pestronk, A., Florence, J., Miller, T., Choksi, R., Al Lozi, M.T. and Levine, T.D. 2003. Treatment of IgM antibody associated polyneuropathies using rituximab. J Neurol Neurosurg Psych, 74, 485–9. Pollard, J.D., Westland, K.W., Harvey, G.K. et al. 1995. Activated T-cells of nonneural specificity open the blood–nerve barrier to circulating antibody. Ann Neurol, 37, 467–75. Prineas, J.W. and McLeod, J.G. 1976. Chronic relapsing polyneuritis. J Neurol Sci, 27, 427–58. Rechthand, E., Cornblath, D.R., Stern, B.J. and Meyerhoff, J.O. 1984. Chronic demyelinating polyneuropathy in systemic lupus erythematosus. Neurology, 34, 1375–7. Renaud, S., Gregor, M., Fuhr, P. et al. 2003. Rituximab in the treatment of polyneuropathy associated with anti-MAG antibodies. Muscle Nerve, 27, 611–15. Rodnitzky, R.L. and Goeken, J.A. 1982. Complications of plasma exchange in neurological patients. Arch Neurol, 39, 350–4. Ropper, A.H., Wijdicks, F.M. and Traux, B.T. 1991. Guillain–Barré Syndrome, F.A. Davis, Philadelphia. Rotta, F.T., Sussman, A.T., Bradley, W.G., Ayyar, D.R., Sharma, K.R. and Shebert, R.T. 2000. The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci, 173, 129–39. Rousseau, A., Salachas, F., Baccard, M., Delattre, J.Y. and Sanson, M. 2005. Chronic inflammatory polyneuropathy revealing malignant melanoma. J Neuro- oncol, 71, 335–6. Rubin, M., Karpati, G. and Carpenter, S. 1987. Combined central and peripheral myelinopathy. Neurology, 37, 1287–90. Saperstein, D.S., Amato, A.A., Wolfe, G.I. et al. 1999. Multifocal acquired demyelinating sensory and motor neuropathy, The Lewis-Sumner syndrome. Muscle Nerve, 22, 560–6. Saperstein, D.S., Katz, J.S., Amato, A.A. and Barohn, R.J. 2001. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve, 24, 311–24. Schimmer, B.P. and Parker, K.L. 2001. Adrenocorti- cotropic hormone; adrenocorticical steroids and their synthetic analogues, inhibitors of the syn- thesis and action of adrenocortical hormones. In J.G. Hardman, L.E. Limbird and A.G. Gilman (eds.), NICP_C07 04/05/2007 02:44PM Page 136 Immune-mediated chronic demyelinating polyneuropathies 137 Goodman and Gilman’s The Pharmacological Basis of Therapeutics, McGraw-Hill, New York, pp. 1679– 714. Sekul, E.A., Cupler, E.J. and Dalakas, M.C. 1994. Aseptic-meningitis associated with high-dose intravenous immunoglobulin therapy – Frequency and risk factors. Ann Int Med, 121, 259–62. Sharma, K.R., Cross, J., Farronay, O., Ayyar, D.R., Shebert, R.T. and Bradley, W.G. 2002. Demyelinat- ing neuropathy in diabetes mellitus. Arch Neurol, 59, 758–65. Sherman, W.H., Latov, N., Lange, D., Hays, R. and Younger, D. 1994. Fludarabine for Igm antibody- mediated neuropathies. Ann Neurol, 36, 326–7. Shy, M.E., Arroyo, E., Sladky, J. et al. 1997. Hetero- zygous P-0 knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP). J Neuropath Exp Neurol, 56, 811–21. Simmons, Z., Albers, J.W., Bromberg, M.B. and Feldman, E.L. 1993. Presentation and initial clinical course in patients with chronic inflammatory demyelinating polyradiculoneuropathy – Comparison of patients without and with monoclonal gammopathy. Neurology, 43, 2202–9. Simmons, Z. and Tivakaran, S. 1996. Acquired demyelinating polyneuropathy presenting as a pure clinical sensory syndrome. Muscle Nerve, 19, 1174–76. Singh, R.R. 2004. Prevention and control of reciprocal T-B cell diversification: Implications for lupus-like autoimmunity. Mol Immunol, 40, 1137–45. Sinnreich, M., Klein, C.J., Daube, J.R., Engelstad, J., Spinner, R.J. and Dyck, P.J. 2004. Chronic immune sensory polyradiculopathy, a possibly treatable sensory ataxia. Neurology, 63, 1662–9. Small, G.A. and Lovelace, R.E. 1993. Chronic inflammatory demyelinating polyneuropathy. Sem Neurol, 13, 305–12. Stewart, J.D, McKelvey, R., Durcan, L., Carpenter, S. and Karpati, G. 1996. Chronic inflammatory demyelinating polyneuropathy (CIDP) in diabetics. J Neurol Sci, 142, 59–64. Sumner, A.J. 1994. Electrophysiology of the inflammatory demyelinating polyneuropathies. Baillieres Clinical Neurology, 3, 25–43. Svennerholm, L. 1994. Designation and schematic structure of gangliosides and allied glycosphin- golipids. In L. Svennerholm, A.K. Asbury, R.A. Resifeld, K. Sandhoff, K. Suzuki and G. Tettamanti (eds.), Biological Function of Gangliosides: Progress in Brain Research. Vol 101, Elsevier, Amsterdam. Tatum, A.H. 1993. Experimental paraprotein neuropathy, demyelination by passive transfer of human-Igm anti-myelin-associated glycoprotein. Ann Neurol, 33, 502–6. Thaisetthawatkul, P., Logigian, E.L. and Herrmann, D.N. 2002. Dispersion of the distal compound muscle action potential as a diagnostic criterion for chronic inflammatory demyelinating polyneuropathy. Neuro- logy, 59, 1526–32. Thomas, P.K., Claus, D., Jaspert, A. et al. 1996. Focal upper limb demyelinating neuropathy. Brain, 119, 765–74. Thomas, P.K., Lascelle, R.G., Hallpike, J.F. and Hewer, R.L. 1969. Recurrent and chronic relapsing Guillain-Barre polyneuritis. Brain, 92, 589. Thomas, P.K., Walker, R.W.H., Rudge, P. et al. 1987. Chronic demyelinating peripheral neuropathy associated with multifocal central-nervous-system demyelination. Brain, 110, 53–76. Trojaborg, W., Hays, A.P., Vandenberg, L., Younger, D.S. and Latov, N. 1995. Motor conduction para- meters in neuropathies associated with anti–MAG antibodies and other types of demyelinating and axonal neuropathies. Muscle Nerve, 18, 730–5. Vallat, J.M., Hahn, A.F., Leger, J.M. et al. 2003. Interferon beta-1a as an investigational treatment for CIDP. Neurology, 60, S23–S28. Van den Berg, L.H., Kerkhoff, H., Oey, P.L. et al. 1995. Treatment of multifocal motor neuropathy with high-dose intravenous immunoglobulins – A double- blind, placebo-controlled study. J Neurol Neurosurg Psych, 59, 248–52. Van den Berg, L.H., Lokhorst, H. and Wokke, J.H.J. 1997. Pulsed high-dose dexamethasone is not effective in patients with multifocal motor neuropathy. Neurology, 48, 1135. Van den Berg-Vos, R.M., van den Berg, L.H., Franssen, H. et al. 2000. Multifocal inflammatory demyelinating neuropathy – A distinct clinical entity? Neurology, 54, 26–32. Van den Bergh, P.Y. and Pieret, F. 2004. Electrodia- gnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve, 29, 565–74. van Dijk, G.W., Notermans, N.C., Franssen, H. and Wokke, J.H. 1999. Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: A long-term follow-up study. J Neurol, 246, 1134–9. van Doorn, P.A., Schreuder, G.M., Vermeulen, M., d’Amaro, J. and Brand, A. 1991. HLA antigens in patients with chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol, 32, 133–9. Van Doorn, P.A. 1996. Treatment of patients with chronic inflammatory demyelinating polyneuropathy. Rev Neurolog, 152, 383–6. Vaughan, R.W., Adam, A.M., Gray, I.A. et al. 1990. Major histocompatibility complex class I and class II polymorphism in chronic idiopathic demyelinating polyradiculoneuropathy. J Neuroimmunol, 27, 149– 53. Veenstra, D.L., Best, J.H., Hornberger, J., Sullivan, S.D. and Hricik, D.E. 1999. Incidence and long-term cost of steroid-related side effects after renal transplantation. Am J Kidney Dis, 33, 829–39. NICP_C07 04/05/2007 02:44PM Page 137 138 THOMAS H. BRANNAGAN III Verma, A., Tandan, R., Adesina, A.M., Pendlebury, W.W., Fries, T.J. and Bradley, W.G. 1990. Focal neuropathy preceding chronic inflammatory demyelinating polyradiculoneuropathy by several years. Acta Neurol Scand, 81, 516–21. Vermeulen, M. and Van Oers, M.H. 2002. Successful autologous stem cell transplantation in a patient with chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psych, 72, 127–8. Viala, K., Renie, L., Maisonobe, T. et al. 2004. Follow- up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain, 127, 2010–17. Vital, A., Vital, C., Lagueny, A. et al. 2003. Inflam- matory demyelination in a patient with CMT1A. Muscle Nerve, 28, 373–6. Watanabe, O., Arimura, K., Kitajima, I., Osame, M. and Maruyama, I. 1996. Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet, 347, 702. Watanabe, O., Maruyama, I., Arimura, K. et al. 1998. Overproduction of vascular endothelial growth factor – Vascular permeability factor is causative in Crow–Fukase (POEMS) syndrome. Muscle Nerve, 21, 1390–7. Weiss. M.D., Luciano, C.A., Semino-Mora, C., Dalakas, M.C. and Quarles, R.H. 1998. Molecular mimicry in chronic inflammatory demyelinating polyneuropathy and melanoma. Neurology, 51, 1738– 41. Willison, H.J. 1994. Antiglycolipid antibodies in peripheral neuropathy: fact or fiction? J Neurol Neurosurg Psychiatry, 57, 1303–7. Wilson, H.C., Lunn, M.P.T., Schey, S. and Hughes, R.A.C. 1999. Successful treatment of IgM paraproteinaemic neuropathy with fludarabine. J Neurol Neurosurg Psychiatry, 66, 575–80. Yan, W.X., Archelos, J.J., Hartung, H.P. and Pollard, J.D. 2001. P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol, 50, 286–92. Zonana-Nacach, A., Barr, S.G., Magder, L.S. and Petri, M. 2000. Damage in systemic lupus erythematosus and its association with corticosteroids. Arthritis Rheumat, 43, 1801–8. NICP_C07 04/05/2007 02:44PM Page 138 Introduction Autonomic neuropathy refers to disorders in which autonomic nerve fibers are selectively or dispropor- tionately affected compared to sensory or motor nerve fibers. Some disorders selectively affect autonomic ganglia neurons and are referred to as autonomic neuronopathies. These processes can affect the sympathetic, parasympathetic, and enteric arms of the nervous system or a combination of systems and thus can present with a large variety of symptoms (Box 8.1). Orthostatic hypotension (OH) is usually a more severe and often late finding that many physicians consider the primary manifestation of symptomatic autonomic dysfunction. Dysautonomia results from numerous disease states with diverse pathogenic mechanisms, many of which have only symptomatic treatment available. This chapter will discuss immune-mediated (Box 8.2) or probable immune-mediated forms of autonomic dysfunction that have proven or supportive evidence for more effective forms of treatment. Examples include Guillain–Barré syndrome, primary immune-mediated autonomic neuropathies, paraneoplastic syndromes, and rheumatologic diseases such as Sjögren syndrome and systemic lupus erythematosus. These conditions are important to bear in mind when evaluating a patient with any form of autonomic dysfunction because early recognition of some disease states may lead to preventative treatment prior to significant nerve or neuronal injury or early detection of an underlying malignancy. 8 Immune-mediated autonomic neuropathies Louis H. Weimer and Mill Etienne Gastrointestinal: Constipation, diarrhea, postprandial bloating, fullness, nausea, vomiting, postprandial dizziness, sweating or orthostatic hypotension Genitourinary: Urinary retention, incomplete emptying, incontinence, frequency Orthostatic: Lightheadedness, weakness, fatigue, cognitive changes, visual disturbances, vertigo, anxiety, palpitations, pallor, nausea, syncope – exacerbated by prolonged standing, post-exercise, meals, warm environment, early morning, prolonged recumbency, physical countermaneuvers, speed of postural change, and medication effects Secretomotor: Dry eyes and mouth, need for natural tears, frequent sips of water Sexual: Erectile dysfunction, ejaculatory dysfunction, retrograde ejaculation into bladder Sudomotor: Reduction or loss of sweating (distally in polyneuropathies), excessive, paroxysmal, or inappropriate sweating, mixed pattern of distal loss and excessive proximal sweating, heat intolerance Vasomotor: Distal color changes, change in skin appearance, persistently cold extremities, Raynaud’s phenomenon, loss of skin wrinkling in water, heat intolerance, indoor gloves Visual: Blurred vision, sensitivity to light/glare, reduced night vision Other: Unexplained syncope Box 8.1 Autonomic review of systems. NICP_C08 04/05/2007 12:26PM Page 139 140 LOUIS H. WEIMER AND MILL ETIENNE Autonomic testing Many common autonomic symptoms, other than overt symptomatic orthostatic hypotension, are somewhat nonspecific. A combination of symptoms or exclusion of other causes is often needed before autonomic causes are suspected. More objective evidence to assess the validity, severity, and progress of disorders is frequently useful and desirable. Conventional nerve conduction studies and electromyography are often performed but frequently are of limited direct diagnostic benefit because many autonomic conditions spare or minimally involve somatic sensory and motor nerve fibers. Other conditions have clear somatic neuropathy but unclear degree of autonomic impairment. For this purpose there are numerous reliable noninvasive techniques available. Dedicated autonomic testing laboratories exist in most large and medium size cities in the United States and in many cities in Europe, Japan, and Australia. Unlike motor and sensory nerve conduction studies most techniques do not directly record autonomic nerve fiber activity. Instead testing involves induction of physiologic perturbations followed by the evaluation of the responses of complex overlapping reflex loops via the measurement of end-organ function. Although many systems are potentially testable, the func- tions deemed most reproducible, reliable, and most commonly evaluated are cardiovagal (parasympathetic), adrenergic vasoconstriction (sympathetic), and sudomotor function. Noninvasive measures of cardiovascular parasympathetic function involve the analysis of heart rate variability and the degree of sinus arrhythmia. The most common triggers are cyclic deep breathing, Valsalva maneuver, and active standing. Cardiovas- cular sympathetic function measures most commonly assess the blood pressure response to physiological stimuli, usually active standing or standardized head- up tilt. The beat-to-beat blood pressure response to Valsalva is another widely used method and many show changes prior to orthostatic challenge. Pro- longed tilt-table testing, with or without pharmacological provocation, has become an important tool in the investigation of a predisposition to neurally mediated syncope (Freeman, 2006). Tilt studies are also useful in the evaluation of autonomic failure. Although limited testing can be performed with conventional equipment and at the bedside, a battery of tests conducted in a dedicated laboratory is most desirable and dependable (Box 8.3). Formal laboratory testing can establish subclinical autonomic involvement, determine additional affected components not detected by bedside examination, grade disease severity, chart the clinical course, and assist in monitoring therapeutic response. Most physicians are familiar with and accustomed to performing the bedside examinations of parasympathetic and sympathetic function mentioned in Box 8.3 and described elsewhere in greater detail (Low, 2003); however, tests of sympathetic cholinergic function are not commonly measured or considered by many physicians. For that reason, these tests are briefly outlined in more detail, but are generally less available than other tests discussed earlier. The quantitative sudomotor action reflex test (QSART) tests postganglionic sudomotor function. It is performed by iontophoresing acetylcholine (ACh) Acute autonomic and sensory neuropathy Acute autonomic neuropathy (acute pandysautonomia) Acute cholinergic pandysautonomia Enteric neuronopathy Guillain–Barré syndrome Holmes–Adie syndrome Hyperexcitability syndromes (Isaacs syndrome, Morvan syndrome) Lambert–Eaton myasthenic syndrome (LEMS) Orthostatic intolerance (postural orthostatic tachycardia syndrome (POTS)) – some forms Other paraneoplastic syndromes (e.g. Anti-Hu, -CV2, -PCA-2, -CRMP-5) Rheumatologic diseases (e.g. rheumatoid arthritis, Sjögren syndrome, systemic lupus erythematosus, mixed connective tissue disease) Box 8.2 Immune-mediated autonomic neuropathies. NICP_C08 04/05/2007 12:26PM Page 140 Immune-mediated autonomic neuropathies 141 diluted in deionized water onto an isolated patch of skin. The ACh binds to muscarinic receptors on the eccrine sweat glands as well as nicotinic receptors on the postganglionic sudomotor axon. Retrograde impulses are generated that trigger an evoked sweat response from an adjacent but separate site and then recorded using a sudrometer; the test is sensitive, specific, and reproducibile (Hilz and Dutsch, 2006). The areas of QSART territories and actual induced sudomotor axon reflex sweating appear to be of similar size in human skin and vary according to site (Schlereth et al., 2005). QSART has also been shown to correlate well with epidermal nerve fiber density in small fiber painful neuropathy studies (Novak et al., 2001; Periquet et al., 1999). A device is commer- cially available and used in numerous centers as well as in clinical trials (Q-Sweat; WR Medical, Stillwell, MN). Silastic skin imprinting uses methods similar to the QSART to stimulate sweat production and measures sweat output by visualizing and quantify- ing sweat droplets by examining plastic or silicone which is allowed to harden over the treated area and subsequently transilluminated under a dissecting microscope or with video camera projection (Ravits, 1997). Although the silastic skin imprinting likely has similar sensitivity to QSART, it is not widely available (Hilz and Dutsch, 2006). The thermoregulatory sweat test (TST) evaluates both central and peripheral sudomotor function. A moisture-sensitive indicator powder, usually alizarin red, is dusted over the patient’s entire anterior skin surface. The patient is placed in a sweat chamber with temperature between 45–50 °C. As the patient is heated, the sweat changes the powder color and the areas of anhidrosis are visualized and quantified (Hilz and Dutsch, 2006). Although the TST is an excellent measure of both central and peripheral sudomotor function, it is only available at a few centers across the country. The sympathetic skin response (SSR) is the most commonly performed sudomotor test and is recorded with routine electromyography (EMG) equipment. The SSR is best recorded from the palms of the hands and soles of the feet, with the reference electrodes on the dorsum of the hands and feet. Responses can be evoked by various types of stimulation, such as electrical, star- tle, cough, or inspiratory gasp stimuli. The response is variable and tends to habituate with repeated triggers; moreover there is no clear consensus regard- ing abnormal responses or uniform testing methods. Although the test is easy to perform, it is probably the least sensitive and reproducible of these techniques and has the most technical constraints including interpretation (Etienne and Weimer, 2006; Hilz and Dutsch, 2006). Microneurography is one special technique that directly records bursts of efferent muscle sympathetic nerve activity (MSNA) and is probably the best and most direct measure of sympathetic activity. How- ever, because this test is somewhat invasive, tech- nically difficult, and time consuming it is primarily used for research purposes and is not a routine clinical test. MSNA, usually recorded from the peroneal nerve, is measured from a recording electrode inserted into a single nerve fascicle and through numerous calculations, sympathetic nerve activity can be measured (Hilz and Dutsch, 2006). There is a multitude of additional investigational tools that can be used to evaluate autonomic function such as for evaluation of pupillary function, gastric and intestinal motility, bladder function, and others used by certain laboratories. Cardiovagal (Parasympathetic function) Heart rate response to deep breathing (HRDB) Heart rate response to Valsalva maneuver Heart rate response to standing (30:15 ratio) Adrenergic (Sympathetic function) Blood pressure response to Valsalva maneuver Blood pressure response to standing or passive tilt Blood pressure response to exercise, handgrip, and pharmacologic agents Sudomotor (Sympathetic cholinergic function) Quantitative sudomotor action reflex test (QSART) (Q-Sweat) Thermoregulatory sweat test Silastic skin imprinting (sweat imprint methods) Sympathetic skin response Box 8.3 Most common and best-accepted autonomic battery measures. NICP_C08 04/05/2007 12:26PM Page 141 [...]... examination raise a suspicion for LEMS (Lennon, 1997) Additional AChR antibody tests include modulating and blocking antibodies AchR-modulating antibodies bind to external segments of the AChR, cross-linking them on the cell surface and triggering their degradation They are positive in approximately 86% of MG patients and only 3–4% of AChR-binding antibody negative patients (Howard et al., 1987) AchR-blocking... unexplained subacute autonomic neuropathy In addition to anti-Hu (ANNA-1) and ganglionic AChR antibodies, the syndrome is associated with other antibodies including antibodies against Purkinje cell cytoplasmic type-2 (PCA-2), CV-2, and collapsin response-mediator protein (CRMP-5) The evidence suggests considerable overlap of paraneoplastic antibodies, which often coexist but predict an underlying malignancy... exacerbating condition with autonomic failure Epoetin alpha (Epogen) increases hematocrit, reduces symptoms, and elevates systolic pressure an average of 10 to 15 mm Hg (Biaggioni et al., 1994) Other drugs of potential but less consistent benefit include indomethacin, somatostatin analogs, caffeine, ergot alkaloids, and nocturnal desmopressin L-threo-3, 4-dihydroxyphenylserine is used in the rare but distinctive... Guerrini, S., Barbara, G et al 2004 In ammatory neuropathies of the enteric nervous system Gastroenterology, 1 26, 1872–83 Dornonville de la Cour, C and Jakobsen, J 2005 Residual neuropathy in long-term population-based follow-up of Guillain–Barré syndrome Neurology, 64 , 2 46 53 Etienne, M and Weimer, L.H 20 06 Immune-mediated autonomic neuropathies Curr Neurol Neurosci Rep, 6, 57 64 Fagius, J and Wallin,... Sidell, A.D 1 960 The Guillain–Barré syndrome, the need for exact diagnostic criteria N Engl J Med, 262 , 964 – 9 Pan, C.L., Tseng, T.J., Lin, Y.H., Chiang, M.C., Lin, W.M and Hsieh, S.T 2003 Cutaneous innervation in Guillain–Barré syndrome: Pathology and clinical correlations Brain, 1 26( 2), 3 86 97 151 Pardi, D.S., Miller, S.M., Miller, D.L et al 2002 Paraneoplastic dysmotility: Loss of interstitial cells... thymoma In TFH, lymphoid follicules and germinal centers form at the corticomedullary junction This architecture brings AChR-bearing myoid cells into intimate contact with antigen-presenting cells, the major histocompatibility complex (MHC)-II positive interdigitating cells The myasthenic thymus has all the cellular components required for autoantibody production: MHC-II positive antigen-presenting cells,... 1 969 ; Young et al., 1975) Small in ammatory mononuclear cell in ltrates have been seen in epineurial sural nerve biopsies with concomitant decreases in small myelinated and unmyelinated fibers, supporting the immune-mediated disease basis (Suarez et al., 1994) Vernino and Lennon developed an assay to detect serum antibodies against the ganglionic nicotinic acetylcholine receptor (AChR) after suspecting... reversal with recombinant erythropoietin Ann Intern Med, 121, 181– 6 Boukhris, S., Magy, L., Li, Y., Debras, C and Vallat, J.M 2005 Autonomic nervous system involvement in chronic in ammatory demyelinating polyneuropathy Revue Neurol (Paris), 161 , 1228–31 Camdessanche, J.P., Antoine, J.C., Honnorat, J et al 2002 Paraneoplastic peripheral neuropathy associated with anti-Hu antibodies A clinical and electrophysiological... Autonomic failure and proximal skeletal myopathy in a patient with primary Sjögren syndrome Mayo Clin Proc, 74, 69 5–7 Stamboulis, E., Katsaros, N., Koutsis, G., Iakovidou, H., Giannakopoulou, A and Simintzi, I 20 06 Clinical and subclinical autonomic dysfunction in chronic NICP_C08 04/05/2007 12:26PM Page 152 152 LOUIS H WEIMER AND MILL ETIENNE in ammatory demyelinating polyradiculoneuropathy Muscle Nerve,... vasomotor changes and instability, urinary retention, gastrointestinal dysmotility and ileus, incontinence, facial flushing, pupillary changes and abnormalities on many of the autonomic tests previously discussed (Toth and Zochodne, 2003; Wheeler et al., 1984) Although GBS is a predominantly demyelinating disease, there is experimental, clinical, and NICP_C08 04/05/2007 12:26PM Page 143 Immune-mediated autonomic . Pillay, N. et al. 1996a. Plasma-exchange therapy in chronic in ammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study. Brain, 119, 1055– 66 . Hahn, A.F., Bolton,. Feasby, T.E. 1996b. Intravenous immunoglobulin treatment in chronic in ammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study. Brain, 119, 1 067 –77. Hahn, A.F.,. molecule BB-1, the ligands CTLA-4 and CD28 and their mRNAs in chronic in ammatory demyelinating polyneuropathy. Brain, 123, 166 0 6. Mygland, A. and Monstad, P. 2001. Chronic polyneuropathies in Vest-Agder,

Định dạng
Số trang	27
Dung lượng	288,94 KB