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Right arrow Chronic inflammatory demyelinating polyneuropathy
NEUROLOGY 2005;65:1924-1929
© 2005 American Academy of Neurology

Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP

C. Sommer, MD, S. Koch, M. Lammens, MD, A. Gabreels-Festen, MD, G. Stoll, MD and K. V. Toyka, MD

From the Department of Neurology (Drs. Sommer, Stoll, and Toyka, and S. Koch), Julius-Maximilians Universität, Würzburg, Germany; and Departments of Pathology (Dr. Lammens) and Neurology (Drs. Lammens and Gabreels-Festen), Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.

Address correspondence and reprint requests to Dr. Claudia Sommer, Neurologische Klinik der Universität, Josef-Schneider-Str. 11, D-97080 Würzburg, Germany; e-mail: sommer{at}mail.uni-wuerzburg.de

Background: In adult patients with a slowly progressive demyelinating neuropathy, it may be difficult to distinguish between a hereditary neuropathy and chronic inflammatory demyelinating polyneuropathy (CIDP). The authors previously observed clustering of macrophages around endoneurial blood vessels in sural nerve biopsies from patients with CIDP.

Objectives: To quantitate macrophage clustering around endoneurial blood vessels in CIDP vs hereditary neuropathies.

Methods: The authors studied 21 patients with CIDP, 18 patients with hereditary neuropathies, and 5 normal sural nerves. Numbers of macrophages, T-cells, and blood vessels were counted after immunohistochemical staining. The presence of three or more macrophages around one blood vessel was defined as a cluster. In a subsequent validation analysis, 65 stored biopsy specimens obtained from patients with a chronic neuropathy were re-evaluated for perivascular macrophage clustering according to criteria derived from the quantitative analysis of the first 221 biopsies in a blinded fashion.

Results: The percentage of endoneurial vessels with macrophage clusters was higher in CIDP than in hereditary neuropathies (CIDP median = 9.4, range 0 to 48; hereditary NP median = 0, range 0 to 7.7; p < 0.001). The evaluation of the 65 further biopsies showed that the presence of one perivascular macrophage cluster per fascicle proved to be a valid criterion to differentiate between inflammatory and other forms of neuropathy ({chi}2 test p = 0.0000025, sensitivity 75%, specificity 72%).

Conclusion: The presence of clusters of macrophages around endoneurial vessels in sural nerve biopsies may serve as a useful additional marker for establishing the pathologic diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).

Supported by intramural funds of the University of Würzburg.

Disclosure: The authors report no conflicts of interest.

Received June 7, 2005. Accepted in final form August 29, 2005.






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