Brain, Vol. 122, No. 3, 427-439,
March 1999
© 1999 Oxford University Press
Article |
Clinicopathological features of Churg–Strauss syndrome-associated neuropathy
1 Department of Neurology, Nagoya University School of Medicine and 2 Division of Neurology, Fourth Department of Internal Medicine, Aichi Medical University, Japan
Correspondence to:
Gen Sobue, MD, Department of Neurology, Nagoya University School of Medicine, Tsurumai, Nagoya 466 8550, Japan E-mail: sobueg{at}tsuru.med.nagoya-u.ac.jp
We assessed the clinicopathological features of 28 patients with peripheral neuropathy associated with Churg–Strauss syndrome. Initial symptoms attributable to neuropathy were acute painful dysaesthesiae and oedema in the dysaesthetic portion of the distal limbs. Sensory and motor involvement mostly showed a pattern of mononeuritis multiplex in the initial phase, progressing into asymmetrical polyneuropathy, restricted to the limbs. Parallel loss of myelinated and unmyelinated fibres due to axonal degeneration was evident as decreased or absent amplitudes of sensory nerve action potentials and compound muscle action potentials, indicating acute massive axonal loss. Epineurial necrotizing vasculitis was seen in 54% of cases; infiltrates consisted mainly of CD8-positive suppressor/cytotoxic and CD4-positive helper T lymphocytes. Eosinophils were present in infiltrates, but in smaller numbers than lymphocytes. CD20-positive B lymphocytes were seen only occasionally. Deposits of IgG, C3d, IgE and major basic protein were scarce. The mean follow-up period was 4.2 years, with a range of 8 months to 10 years. Fatal outcome was seen only in a single patient, indicating a good survival rate. The patients who responded well to the initial corticosteroid therapy within 4 weeks regained self-controlled functional status in long-term follow-up (modified Rankin score was 
2), while those not responding well to the initial corticosteroid therapy led a dependent existence (P < 0.01). In addition the patients with poor functional outcomes had significantly more systemic organ damage caused by vasculitis (P < 0.05). Necrotizing vasculitis mediated by cytotoxic T cells, leading to ischaemic changes, appears to be a major cause of Churg–Strauss syndrome-associated neuropathy. The initial clinical course and the extent of systemic vasculitic lesions may influence the long-term functional prognosis.
Churg–Strauss syndrome; vasculitic neuropathy; corticosteroids; T-cell infiltration; prognosis
CMAP = compound muscle action potential; ESR = erythrocyte sedimentation rate; LDH = lactate dehydrogenase; PAN = polyarteritis nodosa; RA = rheumatoid arthritis; SNAP = sensory nerve action potential; SNVDI = systemic necrotizing vasculitis damage index; p-ANCA = perinuclear antineutrophil cytoplasmic antibody
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  L. Cattaneo, E. Chierici, L. Pavone, C. Grasselli, P. Manganelli, C. Buzio, and G. Pavesi Peripheral neuropathy in Wegener's granulomatosis, Churg Strauss syndrome and microscopic polyangiitis J. Neurol. Neurosurg. Psychiatry, October 1, 2007; 78(10): 1119 - 1123. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Oki, H. Koike, M. Iijima, K. Mori, N. Hattori, M. Katsuno, T. Nakamura, M. Hirayama, F. Tanaka, M. Shiraishi, et al. Ataxic vs painful form of paraneoplastic neuropathy Neurology, August 7, 2007; 69(6): 564 - 572. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C.-C. Chao, S.-T. Hsieh, C.-T. Shun, and S.-C. Hsieh Skin Denervation and Cutaneous Vasculitis in Eosinophilia-Associated Neuropathy Arch Neurol, July 1, 2007; 64(7): 959 - 965. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Mathew, K. Talbot, S. Love, S. Puvanarajah, and M. Donaghy Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome QJM, January 1, 2007; 100(1): 41 - 51. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M.-T. Tseng, S.-C. Hsieh, C.-T. Shun, K.-L. Lee, C.-L. Pan, W.-M. Lin, Y.-H. Lin, C.-L. Yu, and S.-T. Hsieh Skin denervation and cutaneous vasculitis in systemic lupus erythematosus Brain, April 1, 2006; 129(4): 977 - 985. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H Koike, M Hirayama, M Yamamoto, H Ito, N Hattori, F Umehara, K Arimura, S Ikeda, Y Ando, M Nakazato, et al. Age associated axonal features in HNPP with 17p11.2 deletion in Japan J. Neurol. Neurosurg. Psychiatry, August 1, 2005; 76(8): 1109 - 1114. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M G Danieli, M Cappelli, G Malcangi, F Logullo, A Salvi, and G Danieli Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome Ann Rheum Dis, December 1, 2004; 63(12): 1649 - 1654. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Koike, K. Misu, M. Sugiura, M. Iijima, K. Mori, M. Yamamoto, N. Hattori, E. Mukai, Y. Ando, S. Ikeda, et al. Pathology of early- vs late-onset TTR Met30 familial amyloid polyneuropathy Neurology, July 13, 2004; 63(1): 129 - 138. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E Suresh, V B Dhillon, C Smith, and J W Ironside Churg-Strauss vasculitis diagnosed on muscle biopsy J. Clin. Pathol., March 1, 2004; 57(3): 334 - 334. [Full Text] [PDF]
|
|
|
|
|
|
M. P. Collins, M. I. Periquet, J. R. Mendell, Z. Sahenk, H. N. Nagaraja, and J. T. Kissel Nonsystemic vasculitic neuropathy: Insights from a clinical cohort Neurology, September 9, 2003; 61(5): 623 - 630. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Hattori, M. Yamamoto, T. Yoshihara, H. Koike, M. Nakagawa, H. Yoshikawa, A. Ohnishi, K. Hayasaka, O. Onodera, M. Baba, et al. Demyelinating and axonal features of Charcot-Marie-Tooth disease with mutations of myelin-related proteins (PMP22, MPZ and Cx32): a clinicopathological study of 205 Japanese patients Brain, January 1, 2003; 126(1): 134 - 151. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Della Rossa, C. Baldini, A. Tavoni, A. Tognetti, D. Neglia, G. Sambuceti, R. Puccini, C. Colangelo, and S. Bombardieri Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre Rheumatology, November 1, 2002; 41(11): 1286 - 1294. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. R. Watkins and S. F. Maier Beyond Neurons: Evidence That Immune and Glial Cells Contribute to Pathological Pain States Physiol Rev, October 1, 2002; 82(4): 981 - 1011. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K Mori, H Koike, K Misu, N Hattori, M Ichimura, and G Sobue Spinal cord magnetic resonance imaging demonstrates sensory neuronal involvement and clinical severity in neuronopathy associated with Sjogren's syndrome J. Neurol. Neurosurg. Psychiatry, October 1, 2001; 71(4): 488 - 492. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H Koike, K Misu, N Hattori, S Ito, M Ichimura, H Ito, M Hirayama, M Nagamatsu, I Sasaki, and G Sobue Postgastrectomy polyneuropathy with thiamine deficiency J. Neurol. Neurosurg. Psychiatry, September 1, 2001; 71(3): 357 - 362. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. Solans, J. A. Bosch, C. Perez-Bocanegra, A. Selva, P. Huguet, J. Alijotas, R. Orriols, L. Armadans, and M. Vilardell Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients Rheumatology, July 1, 2001; 40(7): 763 - 771. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Koike, K. Mori, K. Misu, N. Hattori, H. Ito, M. Hirayama, and G. Sobue Painful alcoholic polyneuropathy with predominant small-fiber loss and normal thiamine status Neurology, June 26, 2001; 56(12): 1727 - 1732. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K Misu, T Yoshihara, Y Shikama, E Awaki, M Yamamoto, N Hattori, M Hirayama, T Takegami, K Nakashima, and G Sobue An axonal form of Charcot-Marie-Tooth disease showing distinctive features in association with mutations in the peripheral myelin protein zero gene (Thr124Met or Asp75Val) J. Neurol. Neurosurg. Psychiatry, December 1, 2000; 69(6): 806 - 811. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. P. Collins, J. R. Mendell, M. I. Periquet, Z. Sahenk, A. A. Amato, G. S. Gronseth, R. J. Barohn, C. E. Jackson, and J. T. Kissel Superficial peroneal nerve/peroneus brevis muscle biopsy in vasculitic neuropathy Neurology, September 12, 2000; 55(5): 636 - 643. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K.-i. Misu, N. Hattori, M. Nagamatsu, S.-i. Ikeda, Y. Ando, M. Nakazato, Y.-i. Takei, N. Hanyu, Y. Usui, F. Tanaka, et al. Late-onset familial amyloid polyneuropathy type I (transthyretin Met30-associated familial amyloid polyneuropathy) unrelated to endemic focus in Japan: Clinicopathological and genetic features Brain, October 1, 1999; 122(10): 1951 - 1962. [Abstract] [Full Text] [PDF]
|
|