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Published online before print December 3, 2008, doi:10.1212/01.wnl.0000336345.70511.0f)
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Received April 24, 2008
Accepted August 29, 2008

Practice Parameter: Evaluation of distal symmetric polyneuropathy: Role of autonomic testing, nerve biopsy, and skin biopsy (an evidence-based review). Report of the American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Academy of Physical Medicine and Rehabilitation

J. D. England MD, G. S. Gronseth MD, FAAN, G. Franklin MD, G. T. Carter MD, L. J. Kinsella MD, J. A. Cohen MD, A. K. Asbury MD, K. Szigeti MD, PhD, J. R. Lupski MD, PhD, N. Latov MD, R. A. Lewis MD, P. A. Low MD, M. A. Fisher MD, D. N. Herrmann MD, J. F. Howard Jr MD, G. Lauria MD, R. G. Miller MD, M. Polydefkis MD, MHS, and A. J. Sumner MD

From the Louisiana State University Health Sciences Center (J.D.E., A.J.S.), New Orleans; University of Kansas (G.S.G.), Kansas City; University of Washington (G.F.), Seattle; Providence Health System (G.T.C.), Southwest Washington; St. Louis University School of Medicine (L.J.K.), St. Louis, MO; Dartmouth Hitchcock Medical Center (J.A.C.), Lebanon, NH; University of Pennsylvania School of Medicine (A.K.A.), Philadelphia; Baylor College of Medicine (K.S., J.R.L.), Houston, TX; Weill Medical College of Cornell (N.L.), New York, NY; Wayne State University School of Medicine (R.A.L.), Detroit, MI; Mayo Clinic (P.A.L.), Rochester, MN; Loyola University Chicago Stritch School of Medicine and the Hines VAH (M.A.F.), IL; University of Rochester Medical Center (D.H.), NY; University of North Carolina (J.F.H.), Chapel Hill; Fondazione IRCCS National Neurological Institute "Carlo Besta" (G.L.), Milan, Italy; California Pacific Medical Center (R.G.M.), San Francisco; and Johns Hopkins Medical Institutions (M.P.), Baltimore, MD.


Background: Distal symmetric polyneuropathy (DSP) is the most common variety of neuropathy. Since the evaluation of this disorder is not standardized, the available literature was reviewed to provide evidence-based guidelines regarding the role of autonomic testing, nerve biopsy, and skin biopsy for the assessment of polyneuropathy.

Methods: A literature review using MEDLINE, EMBASE, and Current Contents was performed to identify the best evidence regarding the evaluation of polyneuropathy published between 1980 and March 2007. Articles were classified according to a four-tiered level of evidence scheme and recommendations were based upon the level of evidence.

Results and Recommendations: 1) Autonomic testing should be considered in the evaluation of patients with polyneuropathy to document autonomic nervous system dysfunction (Level B). Such testing should be considered especially for the evaluation of suspected autonomic neuropathy (Level B) and distal small fiber sensory polyneuropathy (SFSN) (Level C). A battery of validated tests is recommended to achieve the highest diagnostic accuracy (Level B). 2) Nerve biopsy is generally accepted as useful in the evaluation of certain neuropathies as in patients with suspected amyloid neuropathy, mononeuropathy multiplex due to vasculitis, or with atypical forms of chronic inflammatory demyelinating polyneuropathy (CIDP). However, the literature is insufficient to provide a recommendation regarding when a nerve biopsy may be useful in the evaluation of DSP (Level U). 3) Skin biopsy is a validated technique for determining intraepidermal nerve fiber density and may be considered for the diagnosis of DSP, particularly SFSN (Level C). There is a need for additional prospective studies to define more exact guidelines for the evaluation of polyneuropathy.


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Practice Parameter: Evaluation of distal symmetric polyneuropathy: Role of laboratory and genetic testing (an evidence-based review): Report of the American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Academy of Physical Medicine and Rehabilitation
J. D. England, G. S. Gronseth, G. Franklin, G. T. Carter, L. J. Kinsella, J. A. Cohen, A. K. Asbury, K. Szigeti, J. R. Lupski, N. Latov, R. A. Lewis, P. A. Low, M. A. Fisher, D. N. Herrmann, J. F. Howard, Jr, G. Lauria, R. G. Miller, M. Polydefkis, and A. J. Sumner
Neurology 2009 72: 185-192. [Abstract] [Full Text] [PDF]



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