Brain, Vol. 126, No. 2, 386-397,
February 2003
© 2003 Guarantors of Brain
doi: 10.1093/brain/awg039
Cutaneous innervation in Guillain–Barré syndrome: pathology and clinical correlations
1 Department of Neurology, National Taiwan University Hospital and 2 Department of Anatomy and Cell Biology, National Taiwan University College of Medicine, Taipei, Taiwan
Correspondence to: Dr Sung-Tsang Hsieh, Department of Anatomy and Cell Biology, National Taiwan University College of Medicine, 1 Jen-Ai Rd, Sec. 1, Taipei 10018, Taiwan E-mail: sthsieh{at}ha.mc.ntu.edu.tw
Guillain–Barré syndrome (GBS) is traditionally considered to be a large-fibre neuropathy. However, the presence of hypo-aesthesia, dysaesthesia and dysautonomia in GBS patients raises the possibility that small-diameter sensory and autonomic nerves may also be affected. To investigate small-fibre neuropathy in GBS, we performed a skin biopsy from the distal leg of 20 patients with the demyelinating form of GBS. Skin sections were immunohistochemically stained with antiserum against protein gene product 9.5 (PGP 9.5), a ubiquitin C-terminal hydrolase. Cutaneous innervation was evaluated by measuring epidermal nerve density (END), and END was further correlated with various clinical and electrophysiological parameters. In GBS patients, END values were much lower than in age- and gender-matched control subjects (5.03 ± 1.18 versus 10.16 ± 0.87 fibres/mm, P < 0.001). Eleven patients (55%) had reduced epidermal innervation with pathological evidence of active nerve degeneration in the dermis: fragmentation of subepidermal nerve plexuses and a beaded appearance of dermal nerves. GBS patients had significantly elevated thermal thresholds with higher warm threshold temperatures (44.54 ± 1.04 versus 39.00 ± 0.35°C, P < 0.001) and lower cold threshold temperatures (25.57 ± 1.11 versus 29.05 ± 0.21°C, P = 0.032). Reduced END values were associated with an elevated warm threshold (P = 0.027), ventilatory distress (P = 0.037) and dysautonomia (P = 0.001). END values were negatively correlated with disability grade on a scale of 1–6 (slope –0.134 ± 0.038, P = 0.0018). Patients with reduced END values tended to have a slower recovery than those with normal END values (P = 0.013, median time 12 versus 2 weeks). Patho logically, sudomotor innervation of the skin was reduced in five of 17 (29.4%) GBS patients in whom sweat glands could be recognized. These findings suggest that small-fibre sensory and autonomic neuropathies exist in a significant proportion of GBS patients, and that END values are correlated with functional disabilities. In summary, GBS should be considered a global neuropathy instead of a pure large-fibre neuropathy.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  C.-C. Chao, H.-Y. Sun, Y.-C. Chang, and S.-T. Hsieh Painful neuropathy with skin denervation after prolonged use of linezolid BMJ Case Reports, February 2, 2009; 2009(jan27_1): bcr0820080702 - bcr0820080702. [Abstract] [Full Text]
|
|
|
|
 |
|
 G. Devigili, V. Tugnoli, P. Penza, F. Camozzi, R. Lombardi, G. Melli, L. Broglio, E. Granieri, and G. Lauria The diagnostic criteria for small fibre neuropathy: from symptoms to neuropathology Brain, July 1, 2008; 131(7): 1912 - 1925. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C-C Chao, H-Y Sun, Y-C Chang, and S-T Hsieh Painful neuropathy with skin denervation after prolonged use of linezolid J. Neurol. Neurosurg. Psychiatry, January 1, 2008; 79(1): 97 - 99. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. J. Ebenezer, J. C. McArthur, D. Thomas, B. Murinson, P. Hauer, M. Polydefkis, and J. W. Griffin Denervation of skin in neuropathies: the sequence of axonal and Schwann cell changes in skin biopsies Brain, October 1, 2007; 130(10): 2703 - 2714. [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]
|
|
|
|
 |
|
 G. Lauria and R. Lombardi Skin biopsy: a new tool for diagnosing peripheral neuropathy BMJ, June 2, 2007; 334(7604): 1159 - 1162. [Full Text] [PDF]
|
|
|
|
 |
|
 C.-I Lu, C.-C. Huang, Y.-C. Chang, Y.-T. Tsai, H.-C. Kuo, Y.-H. Chuang, and T.-S. Shih Short-term Thallium Intoxication: Dermatological Findings Correlated With Thallium Concentration Arch Dermatol, January 1, 2007; 143(1): 93 - 98. [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]
|
|
|
|
|
|
J.-E. Lee, C.-T. Shun, S.-C. Hsieh, and S.-T. Hsieh Skin Denervation in Vasculitic Neuropathy Arch Neurol, October 1, 2005; 62(10): 1570 - 1573. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. D. de la Cour and J. Jakobsen Residual neuropathy in long-term population-based follow-up of Guillain-Barre syndrome Neurology, January 25, 2005; 64(2): 246 - 253. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C.-T. Shun, Y.-C. Chang, H.-P. Wu, S.-C. Hsieh, W.-M. Lin, Y.-H. Lin, T.-Y. Tai, and S.-T. Hsieh Skin denervation in type 2 diabetes: correlations with diabetic duration and functional impairments Brain, July 1, 2004; 127(7): 1593 - 1605. [Abstract] [Full Text] [PDF]
|
|