The Peripheral Neuropathy Evaluation in an Office-Based Neurology Setting

ABSTRACT

Peripheral neuropathy is the most common neuromuscular disorder encountered in a neurologic practice. The evaluation of patients with suspected peripheral neuropathy can be a challenging endeavor for the neurologist. Determining the pattern of clinical involvement and the nerve modalities affected can help to generate a differential diagnosis and design an approach to determine a potential cause. The combination of clinical features, electrodiagnostic testing, and ancillary laboratory studies may be used to determine the etiology. As the most common acquired etiology of peripheral neuropathy is diabetes, careful testing for impaired glucose metabolism is an important part of any diagnostic evaluation.

KEYWORDS

Neuropathy

REFERENCES

• 1 England J D, Asbury A K. Peripheral neuropathy.  Lancet. 2004;  363 (9427) 2151-2161
• 2 Fagius J. Chronic cryptogenic polyneuropathy. The search for a cause.  Acta Neurol Scand. 1983;  67 (3) 173-180
• 3 Prineas J. Polyneuropathies of undetermined cause.  Acta Neurol Scand Suppl. 1970;  44 1-72
• 4 Smith A G, Singleton J R. The diagnostic yield of a standardized approach to idiopathic sensory-predominant neuropathy.  Arch Intern Med. 2004;  164 (9) 1021-1025
• 5 Dyck P J, Oviatt K F, Lambert E H. Intensive evaluation of referred unclassified neuropathies yields improved diagnosis.  Ann Neurol. 1981;  10 (3) 222-226
• 6 Barohn R J. Approach to peripheral neuropathy and neuronopathy.  Semin Neurol. 1998;  18 (1) 7-18
• 7 Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force.  Neurology. 1991;  41 (5) 617-618
• 8 Joint Task Force of the EFNS and the PNS . European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy. Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society.  J Peripher Nerv Syst. 2005;  10 (3) 220-228
• 9 Haq R U, Fries T J, Pendlebury W W, Kenny M J, Badger G J, Tandan R. Chronic inflammatory demyelinating polyradiculoneuropathy: a study of proposed electrodiagnostic and histologic criteria.  Arch Neurol. 2000;  57 (12) 1745-1750
• 10 Hughes R, Bensa S, Willison H Inflammatory Neuropathy Cause and Treatment (INCAT) Group et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy.  Ann Neurol. 2001;  50 (2) 195-201
• 11 Nicolas G, Maisonobe T, Le Forestier N, Léger J M, Bouche P. Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy.  Muscle Nerve. 2002;  25 (1) 26-30
• 12 Thaisetthawatkul P, Logigian E L, Herrmann D N. Dispersion of the distal compound muscle action potential as a diagnostic criterion for chronic inflammatory demyelinating polyneuropathy.  Neurology. 2002;  59 (10) 1526-1532
• 13 Bromberg M B. Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy.  Muscle Nerve. 1991;  14 (10) 968-976
• 14 Magda P, Latov N, Brannagan III T H, Weimer L H, Chin R L, Sander H W. Comparison of electrodiagnostic abnormalities and criteria in a cohort of patients with chronic inflammatory demyelinating polyneuropathy.  Arch Neurol. 2003;  60 (12) 1755-1759
• 15 Molenaar D S, Vermeulen M, de Haan R J. Comparison of electrodiagnostic criteria for demyelination in patients with chronic inflammatory demyelinating polyneuropathy (CIDP).  J Neurol. 2002;  249 (4) 400-403
• 16 Van den Bergh P Y, Piéret F. Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy.  Muscle Nerve. 2004;  29 (4) 565-574
• 17 Jann S, Beretta S, Bramerio M, Defanti C A. Prospective follow-up study of chronic polyneuropathy of undetermined cause.  Muscle Nerve. 2001;  24 (9) 1197-1201
• 18 Lubec D, Müllbacher W, Finsterer J, Mamoli B. Diagnostic work-up in peripheral neuropathy: an analysis of 171 cases.  Postgrad Med J. 1999;  75 (890) 723-727
• 19 Wolfe G I, Baker N S, Amato A A et al.. Chronic cryptogenic sensory polyneuropathy: clinical and laboratory characteristics.  Arch Neurol. 1999;  56 (5) 540-547
• 20 Notermans N C, Wokke J H, Franssen H et al.. Chronic idiopathic polyneuropathy presenting in middle or old age: a clinical and electrophysiological study of 75 patients.  J Neurol Neurosurg Psychiatry. 1993;  56 (10) 1066-1071
• 21 Notermans N C, Wokke J H, van der Graaf Y, Franssen H, van Dijk G W, Jennekens F G. Chronic idiopathic axonal polyneuropathy: a five year follow up.  J Neurol Neurosurg Psychiatry. 1994;  57 (12) 1525-1527
• 22 McLeod J G, Tuck R R, Pollard J D, Cameron J, Walsh J C. Chronic polyneuropathy of undetermined cause.  J Neurol Neurosurg Psychiatry. 1984;  47 (5) 530-535
• 23 Johannsen L, Smith T, Havsager A M et al.. Evaluation of patients with symptoms suggestive of chronic polyneuropathy.  J Clin Neuromuscul Dis. 2001;  3 (2) 47-52
• 24 Saperstein D S, Wolfe G I, Gronseth G S et al.. Challenges in the identification of cobalamin-deficiency polyneuropathy.  Arch Neurol. 2003;  60 (9) 1296-1301
• 25 Bednarik J, Vlckova-Moravcova E, Bursova S, Belobradkova J, Dusek L, Sommer C. Etiology of small-fiber neuropathy.  J Peripher Nerv Syst. 2009;  14 (3) 177-183
• 26 Sorenson E J, Windebank A J. Hypertriglyceridemia in idiopathic painful small fiber neuropathy.  Neurology. 2000;  54 (Suppl 3) A403
• 27 Singleton J R, Smith A G, Bromberg M B. Increased prevalence of impaired glucose tolerance in patients with painful sensory neuropathy.  Diabetes Care. 2001;  24 (8) 1448-1453
• 28 Hoffman-Snyder C, Smith B E, Ross M A, Hernandez J, Bosch E P. Value of the oral glucose tolerance test in the evaluation of chronic idiopathic axonal polyneuropathy.  Arch Neurol. 2006;  63 (8) 1075-1079
• 29 Centers for Disease Control and Prevention (CDC) . Prevalence of diabetes and impaired fasting glucose in adults—United States, 1999-2000.  MMWR Morb Mortal Wkly Rep. 2003;  52 (35) 833-837
• 30 Novella S P, Inzucchi S E, Goldstein J M. The frequency of undiagnosed diabetes and impaired glucose tolerance in patients with idiopathic sensory neuropathy.  Muscle Nerve. 2001;  24 (9) 1229-1231
• 31 Gabriel C M, Howard R, Kinsella N et al.. Prospective study of the usefulness of sural nerve biopsy.  J Neurol Neurosurg Psychiatry. 2000;  69 (4) 442-446
• 32 England J D, Gronseth G S, Franklin G American Academy of Neurology et al. 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.  Neurology. 2009;  72 (2) 177-184
• 33 Hlubocky A, Wellik K, Ross M A et al.. Skin biopsy for diagnosis of small fiber neuropathy: a critically appraised topic.  Neurologist. 2010;  16 (1) 61-63
• 34 Koskinen M, Hietaharju A, Kyläniemi M et al.. A quantitative method for the assessment of intraepidermal nerve fibers in small-fiber neuropathy.  J Neurol. 2005;  252 (7) 789-794
• 35 Løseth S, Lindal S, Stålberg E, Mellgren S I. Intraepidermal nerve fibre density, quantitative sensory testing and nerve conduction studies in a patient material with symptoms and signs of sensory polyneuropathy.  Eur J Neurol. 2006;  13 (2) 105-111
• 36 Shun C T, Chang Y C, Wu H P et al.. Skin denervation in type 2 diabetes: correlations with diabetic duration and functional impairments.  Brain. 2004;  127 (Pt 7) 1593-1605
• 37 Sinnreich M, Taylor B V, Dyck P J. Diabetic neuropathies. Classification, clinical features, and pathophysiological basis.  Neurologist. 2005;  11 (2) 63-79
• 38 Dyck P J, Kratz K M, Karnes J L et al.. The prevalence by staged severity of various types of diabetic neuropathy, retinopathy, and nephropathy in a population-based cohort: the Rochester Diabetic Neuropathy Study.  Neurology. 1993;  43 (4) 817-824
• 39 Dyck P J, Giannini C. Pathologic alterations in the diabetic neuropathies of humans: a review.  J Neuropathol Exp Neurol. 1996;  55 (12) 1181-1193
• 40 The Diabetes Control and Complications Trial Research Group . The effect of intensive diabetes therapy on the development and progression of neuropathy.  Ann Intern Med. 1995;  122 (8) 561-568
• 41 Kelly Jr J J, Kyle R A, O'Brien P C, Dyck P J. Prevalence of monoclonal protein in peripheral neuropathy.  Neurology. 1981;  31 (11) 1480-1483
• 42 Latov N, Braun P E, Gross R B, Sherman W H, Penn A S, Chess L. Plasma cell dyscrasia and peripheral neuropathy: identification of the myelin antigens that react with human paraproteins.  Proc Natl Acad Sci U S A. 1981;  78 (11) 7139-7142
• 43 Latov N, Hays A P, Sherman W H. Peripheral neuropathy and anti-MAG antibodies.  Crit Rev Neurobiol. 1988;  3 (4) 301-332
• 44 Comi G, Roveri L, Swan A Inflammatory Neuropathy Cause and Treatment Group et al. A randomised controlled trial of intravenous immunoglobulin in IgM paraprotein associated demyelinating neuropathy.  J Neurol. 2002;  249 (10) 1370-1377
• 45 Dalakas M C, Quarles R H, Farrer R G et al.. A controlled study of intravenous immunoglobulin in demyelinating neuropathy with IgM gammopathy.  Ann Neurol. 1996;  40 (5) 792-795
• 46 Dyck P J, Low P A, Windebank A J et al.. Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance.  N Engl J Med. 1991;  325 (21) 1482-1486
• 47 Ernerudh J H, Vrethem M, Andersen O, Lindberg C, Berlin G. Immunochemical and clinical effects of immunosuppressive treatment in monoclonal IgM neuropathy.  J Neurol Neurosurg Psychiatry. 1992;  55 (10) 930-934
• 48 Mariette X, Chastang C, Clavelou P, Louboutin J P, Leger J M, Brouet J C. A randomised clinical trial comparing interferon-alpha and intravenous immunoglobulin in polyneuropathy associated with monoclonal IgM. The IgM-associated Polyneuropathy Study Group.  J Neurol Neurosurg Psychiatry. 1997;  63 (1) 28-34
• 49 Nobile-Orazio E, Baldini L, Barbieri S et al.. Treatment of patients with neuropathy and anti-MAG IgM M-proteins.  Ann Neurol. 1988;  24 (1) 93-97
• 50 Nobile-Orazio E, Meucci N, Baldini L, Di Troia A, Scarlato G. Long-term prognosis of neuropathy associated with anti-MAG IgM M-proteins and its relationship to immune therapies.  Brain. 2000;  123 (Pt 4) 710-717
• 51 Lewis R A. Chronic inflammatory demyelinating polyneuropathy.  Neurol Clin. 2007;  25 (1) 71-87
• 52 Ruts L, Drenthen J, Jacobs B C, van Doorn P A. Dutch GBS Study Group . Distinguishing acute-onset CIDP from fluctuating Guillain-Barre syndrome: a prospective study.  Neurology. 2010;  74 (21) 1680-1686
• 53 Hahn A F, Bolton C F, Zochodne D, Feasby T E. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study.  Brain. 1996;  119 (Pt 4) 1067-1077
• 54 Dyck P J, Litchy W J, Kratz K M et al.. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy.  Ann Neurol. 1994;  36 (6) 838-845
• 55 Katz U, Achiron A, Sherer Y, Shoenfeld Y. Safety of intravenous immunoglobulin (IVIG) therapy.  Autoimmun Rev. 2007;  6 (4) 257-259
• 56 Brannagan III T H. Intravenous gammaglobulin (IVIg) for treatment of CIDP and related immune-mediated neuropathies.  Neurology. 2002;  59 (12, Suppl 6) S33-S40
• 57 Klein C J. The inherited neuropathies.  Neurol Clin. 2007;  25 (1) 173-207
• 58 Benson M D, Kincaid J C. The molecular biology and clinical features of amyloid neuropathy.  Muscle Nerve. 2007;  36 (4) 411-423
• 59 Wierzbicki A S, Lloyd M D, Schofield C J, Feher M D, Gibberd F B. Refsum's disease: a peroxisomal disorder affecting phytanic acid alpha-oxidation.  J Neurochem. 2002;  80 (5) 727-735
• 60 Pollock M, Nukada H, Frith R W, Simcock J P, Allpress S. Peripheral neuropathy in Tangier disease.  Brain. 1983;  106 (Pt 4) 911-928
• 61 Schiffmann R. Fabry disease.  Pharmacol Ther. 2009;  122 (1) 65-77
• 62 Albers J W, Fink J K. Porphyric neuropathy.  Muscle Nerve. 2004;  30 (4) 410-422
• 63 Hansen L M, Kristensen O, Friis M L. [Neuropathy in adult metachromatic leukodystrophy].  Ugeskr Laeger. 1994;  156 (15) 2252-2253
• 64 Marks H G, Scavina M T, Kolodny E H, Palmieri M, Childs J. Krabbe's disease presenting as a peripheral neuropathy.  Muscle Nerve. 1997;  20 (8) 1024-1028
• 65 van Geel B M, Assies J, Wanders R J, Barth P G. X linked adrenoleukodystrophy: clinical presentation, diagnosis, and therapy.  J Neurol Neurosurg Psychiatry. 1997;  63 (1) 4-14
• 66 Walker R H, Jung H H, Dobson-Stone C et al.. Neurologic phenotypes associated with acanthocytosis.  Neurology. 2007;  68 (2) 92-98
• 67 Di Donato S. Multisystem manifestations of mitochondrial disorders.  J Neurol. 2009;  256 (5) 693-710
• 68 Bennett C L, Lawson V H, Brickell K L et al.. Late-onset hereditary axonal neuropathies.  Neurology. 2008;  71 (1) 14-20
• 69 England J D, Gronseth G S, Franklin G American Academy of Neurology et al. Practice parameter: the evaluation of distal symmetric polyneuropathy: the role of laboratory and genetic testing (an evidence-based review). Report of the American Academy of Neurology, the American Association of Neuromuscular and Electrodiagnostic Medicine, and the American Academy of Physical Medicine and Rehabilitation.  PM R. 2009;  1 (1) 5-13
• 70 Goldstein D J, Lu Y, Detke M J, Lee T C, Iyengar S. Duloxetine vs. placebo in patients with painful diabetic neuropathy.  Pain. 2005;  116 (1-2) 109-118
• 71 Raskin J, Pritchett Y L, Wang F et al.. A double-blind, randomized multicenter trial comparing duloxetine with placebo in the management of diabetic peripheral neuropathic pain.  Pain Med. 2005;  6 (5) 346-356
• 72 Lesser H, Sharma U, LaMoreaux L, Poole R M. Pregabalin relieves symptoms of painful diabetic neuropathy: a randomized controlled trial.  Neurology. 2004;  63 (11) 2104-2110
• 73 Richter R W, Portenoy R, Sharma U, Lamoreaux L, Bockbrader H, Knapp L E. Relief of painful diabetic peripheral neuropathy with pregabalin: a randomized, placebo-controlled trial.  J Pain. 2005;  6 (4) 253-260
• 74 Rosenstock J, Tuchman M, LaMoreaux L, Sharma U. Pregabalin for the treatment of painful diabetic peripheral neuropathy: a double-blind, placebo-controlled trial.  Pain. 2004;  110 (3) 628-638