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01-10-2016 | Review

Chronic idiopathic axonal polyneuropathy: a systematic review

Authors: Panagiotis Zis, Ptolemaios Georgios Sarrigiannis, Dasappaiah Ganesh Rao, Channa Hewamadduma, Marios Hadjivassiliou

Published in: Journal of Neurology | Issue 10/2016

Abstract

Chronic idiopathic axonal polyneuropathy (CIAP) is a term describing neuropathies with both sensory and motor involvement in a length dependant distribution where neurophysiology reveals axonal damage, neuropathy onset is insidious and shows slow or no progression of the disease over at least 6 months with no aetiology being identified despite appropriate investigations. This entity merits further consideration given how common it is, the absence of clarity regarding aetiopathogenesis, natural history and therapies. A systematic computer-based literature search was conducted on PubMed database. We used two Medical Subject Headings terms in title. Term A was “axonal”, “cryptogenic”, “idiopathic” or “unknown” and Term B was “neuropathy” or “polyneuropathy”. This search strategy resulted in the identification of 658 articles. After eligibility assessment, 48 papers were used for this review. CIAP is usually diagnosed in the sixth decade of life and it is more prevalent in males (ratio 3:2). It is usually slowly progressive. Some data support a potential role of autoimmunity in CIAP and further larger prospective studies are required to address such potential link and any treatment implications. CIAP is a common type of polyneuropathy but the least studied. Increasing awareness and research into this entity may result in better understanding and in the development of treatment strategies.

Martyn CN, Hughes RA (1997) Epidemiology of peripheral neuropathy. J Neurol Neurosurg Psychiatry 62(4):310–318PubMedPubMedCentralCrossRef

Hughes RA (2002) Peripheral neuropathy. BMJ (Clini Res ed) 324(7335):466–469CrossRef

Sveen KA, Karime B, Jorum E, Mellgren SI, Fagerland MW, Monnier VM et al (2013) Small- and large-fiber neuropathy after 40 years of type 1 diabetes: associations with glycemic control and advanced protein glycation: the Oslo Study. Diabetes Care 36(11):3712–3717PubMedPubMedCentralCrossRef

Lacomis D (2002) Small-fiber neuropathy. Muscle Nerve 26(2):173–188PubMedCrossRef

Marquez-Infante C, Murphy SM, Mathew L, Alsanousi A, Lunn MP, Brandner S et al (2013) Asymmetric sensory ganglionopathy: a case series. Muscle Nerve 48(1):145–150PubMedCrossRef

Visser NA, Notermans NC, Degen LA, de Kruijk JR, van den Berg LH, Vrancken AF (2014) Chronic idiopathic axonal polyneuropathy and vitamin B6: a controlled population-based study. J Periph Nerv Syst JPNS 19(2):136–144CrossRef

Lindh J, Tondel M, Osterberg A, Vrethem M (2005) Cryptogenic polyneuropathy: clinical and neurophysiological findings. J Periph Nerv Syst JPNS 10(1):31–37CrossRef

Vrancken AF, Franssen H, Wokke JH, Teunissen LL, Notermans NC (2002) Chronic idiopathic axonal polyneuropathy and successful aging of the peripheral nervous system in elderly people. Arch Neurol 59(4):533–540PubMedCrossRef

Teunissen LL, Notermans NC, Franssen H, van der Graaf Y, Oey PL, Linssen WH et al (1997) Differences between hereditary motor and sensory neuropathy type 2 and chronic idiopathic axonal neuropathy. A clinical and electrophysiological study. Brain J Neurol 120(Pt 6):955–962CrossRef

10.

Wolfe GI, Baker NS, Amato AA, Jackson CE, Nations SP, Saperstein DS et al (1999) Chronic cryptogenic sensory polyneuropathy: clinical and laboratory characteristics. Arch Neurol 56(5):540–547CrossRef

11.

Erdmann PG, van Genderen FR, Teunissen LL, Notermans NC, Lindeman E, van Wijck AJ et al (2010) Pain in patients with chronic idiopathic axonal polyneuropathy. Eur Neurol 64(1):58–64PubMedCrossRef

12.

Dalakas MC (1986) Chronic idiopathic ataxic neuropathy. Ann Neurol 19(6):545–554PubMedCrossRef

13.

Notermans NC, Wokke JH, van den Berg LH, van der Graaf Y, Franssen H, Teunissen LL et al (1996) Chronic idiopathic axonal polyneuropathy. Comparison of patients with and without monoclonal gammopathy. Brain J Neurol 119(Pt 2):421–427CrossRef

14.

Notermans NC, Wokke JH, van der Graaf Y, Franssen H, van Dijk GW, Jennekens FG (1994) Chronic idiopathic axonal polyneuropathy: a five year follow up. J Neurol Neurosurg Psychiatry 57(12):1525–1527PubMedPubMedCentralCrossRef

15.

Notermans NC, Wokke JH, Franssen H, van der Graaf Y, Vermeulen M, van den Berg LH et al (1993) Chronic idiopathic polyneuropathy presenting in middle or old age: a clinical and electrophysiological study of 75 patients. J Neurol Neurosurg Psychiatry 56(10):1066–1071PubMedPubMedCentralCrossRef

16.

Bharucha NE, Bharucha AE, Bharucha EP (1991) Prevalence of peripheral neuropathy in the Parsi community of Bombay. Neurology 41(8):1315–1317PubMedCrossRef

17.

Italian General Practitioner Study Group (IGPSG) (1995) Chronic symmetric symptomatic polyneuropathy in the elderly: a field screening investigation in two Italian regions. I. Prevalence and general characteristics of the sample. Neurology 45(10):1832–1836

18.

Rosenberg NR, Vermeulen M (2004) Chronic idiopathic axonal polyneuropathy revisited. J Neurol 251(9):1128–1132PubMedCrossRef

19.

Smith AG, Singleton JR (2004) The diagnostic yield of a standardized approach to idiopathic sensory-predominant neuropathy. Arch Intern Med 164(9):1021–1025PubMedCrossRef

20.

Singer MA, Vernino SA, Wolfe GI (2012) Idiopathic neuropathy: new paradigms, new promise. J Periph Nerv Syst JPNS 17(Suppl 2):43–49CrossRef

21.

Vilming ST, Stave R (1987) Idiopathic polyneuropathy. A prospective study including gastrointestinal investigation. J Oslo City Hosp 37(11–12):129–134

22.

Rajabally YA, Shah RS (2011) Dyslipidaemia in chronic acquired distal axonal polyneuropathy. J Neurol 258(8):1431–1436PubMedCrossRef

23.

Sachedina S, Toth C (2013) Progression in idiopathic, diabetic, paraproteinemic, alcoholic, and B12 deficiency neuropathy. J Periph Nerv Syst JPNS 18(3):247–255CrossRef

24.

Latov N, Kumar G, Vo ML, Chin RL, Carey BT, Langsdorf JA et al (2015) Elevated blood mercury levels in idiopathic axonal neuropathy. JAMA Neurol 72(4):474–475PubMedCrossRef

25.

Tondel M, Lindh J, Jonsson P, Vrethem M, Persson B (2006) Occupational determinants of cryptogenic polyneuropathy. Neuroepidemiology 26(4):187–194PubMedCrossRef

26.

Smith AG (2012) Impaired glucose tolerance and metabolic syndrome in idiopathic neuropathy. J Periph Nerv Syst JPNS 17(Suppl 2):15–21CrossRef

27.

Novella SP, Inzucchi SE, Goldstein JM (2001) The frequency of undiagnosed diabetes and impaired glucose tolerance in patients with idiopathic sensory neuropathy. Muscle Nerve 24(9):1229–1231PubMedCrossRef

28.

Singleton JR, Smith AG, Bromberg MB (2001) Increased prevalence of impaired glucose tolerance in patients with painful sensory neuropathy. Diabetes Care 24(8):1448–1453PubMedCrossRef

29.

Sumner CJ, Sheth S, Griffin JW, Cornblath DR, Polydefkis M (2003) The spectrum of neuropathy in diabetes and impaired glucose tolerance. Neurology 60(1):108–111PubMedCrossRef

30.

Hoffman-Snyder C, Smith BE, Ross MA, Hernandez J, Bosch EP (2006) Value of the oral glucose tolerance test in the evaluation of chronic idiopathic axonal polyneuropathy. Arch Neurol 63(8):1075–1079PubMedCrossRef

31.

Nebuchennykh M, Loseth S, Jorde R, Mellgren SI (2008) Idiopathic polyneuropathy and impaired glucose metabolism in a Norwegian patient series. Euro J Neurol Off J Euro Feder Neurol Soc 15(8):810–816

32.

Smith AG, Rose K, Singleton JR (2008) Idiopathic neuropathy patients are at high risk for metabolic syndrome. J Neurol Sci 273(1–2):25–28PubMedPubMedCentralCrossRef

33.

Singleton JR, Smith AG, Bromberg MB (2001) Painful sensory polyneuropathy associated with impaired glucose tolerance. Muscle Nerve 24(9):1225–1228PubMedCrossRef

34.

American Diabetes Association (2010) Standards of medical care in diabetes–2010. Diabetes Care 33(Suppl 1):S11–S61

35.

Hughes RA, Umapathi T, Gray IA, Gregson NA, Noori M, Pannala AS et al (2004) A controlled investigation of the cause of chronic idiopathic axonal polyneuropathy. Brain J Neurol 127(Pt 8):1723–1730CrossRef

36.

Gaist D, Jeppesen U, Andersen M, Garcia Rodriguez LA, Hallas J, Sindrup SH (2002) Statins and risk of polyneuropathy: a case-control study. Neurology 58(9):1333–1337PubMedCrossRef

37.

Anderson JL, Muhlestein JB, Bair TL, Morris S, Weaver AN, Lappe DL et al (2005) Do statins increase the risk of idiopathic polyneuropathy? Am J Cardiol 95(9):1097–1099PubMedCrossRef

38.

Fitchett D (2015) The metabolic syndrome is an important concept in therapeutic decision-making. Canadian J Cardiol 31(5):596–600CrossRef

39.

Visser NA, Vrancken AF, van der Schouw YT, van den Berg LH, Notermans NC (2013) Chronic idiopathic axonal polyneuropathy is associated with the metabolic syndrome. Diabetes Care 36(4):817–822PubMedPubMedCentralCrossRef

40.

Rezania K, Soliven B, Rezai KA, Roos RP (2011) Impaired glucose tolerance and metabolic syndrome in idiopathic polyneuropathy: the role of pain and depression. Med Hypotheses 76(4):538–542PubMedCrossRef

41.

Teunissen LL, Franssen H, Wokke JH, van der Graaf Y, Linssen WH, Banga JD et al (2002) Is cardiovascular disease a risk factor in the development of axonal polyneuropathy? J Neurol Neurosurg Psychiatry 72(5):590–595PubMedPubMedCentralCrossRef

42.

Merlini G, Palladini G (2012) Differential diagnosis of monoclonal gammopathy of undetermined significance. Hematol Edu Prog Am Soc Hematol Am Soc Hematol Edu Prog 2012:595–603

43.

Gorson KC, Ropper AH (1997) Axonal neuropathy associated with monoclonal gammopathy of undetermined significance. J Neurol Neurosurg Psychiatry 63(2):163–168PubMedPubMedCentralCrossRef

44.

Eurelings M, van den Berg LH, Wokke JH, Franssen H, Vrancken AF, Notermans NC (2003) Increase of sural nerve T cells in progressive axonal polyneuropathy and monoclonal gammopathy. Neurology 61(5):707–709PubMedCrossRef

45.

Gherardi RK, Farcet JP, Creange A, Claudepierre P, Malapert D, Authier FJ et al (1998) Dominant T-cell clones of unknown significance in patients with idiopathic sensory neuropathies. Neurology 51(2):384–389PubMedCrossRef

46.

Wolfe GI, El-Feky WH, Katz JS, Bryan WW, Wians FH Jr, Barohn RJ (1997) Antibody panels in idiopathic polyneuropathy and motor neuron disease. Muscle Nerve 20(10):1275–1283PubMedCrossRef

47.

Kelkar P, McDermott WR, Parry GJ (2002) Sensory-predominant, painful, idiopathic neuropathy: inflammatory changes in sural nerves. Muscle Nerve 26(3):413–416PubMedCrossRef

48.

Sobue G, Yasuda T, Kachi T, Sakakibara T, Mitsuma T (1993) Chronic progressive sensory ataxic neuropathy: clinicopathological features of idiopathic and Sjogren’s syndrome-associated cases. J Neurol 240(1):1–7PubMedCrossRef

49.

Teunissen LL, Notermans NC, Jansen GH, Banga JD, Veldman H, Wokke JH (2000) Thickness of endoneurial vessel basal lamina area in chronic idiopathic axonal polyneuropathy. Acta Neuropathol 100(4):445–450PubMedCrossRef

50.

Vrancken AF, Notermans NC, Jansen GH, Wokke JH, Said G (2004) Progressive idiopathic axonal neuropathy–a comparative clinical and histopathological study with vasculitic neuropathy. J Neurol 251(3):269–278PubMedCrossRef

51.

Erdmann PG, Teunissen LL, van Genderen FR, Notermans NC, Lindeman E, Helders PJ et al (2007) Functioning of patients with chronic idiopathic axonal polyneuropathy (CIAP). J Neurol 254(9):1204–1211PubMedPubMedCentralCrossRef

52.

Teunissen LL, Eurelings M, Notermans NC, Hop JW, van Gijn J (2000) Quality of life in patients with axonal polyneuropathy. J Neurol 247(3):195–199PubMedCrossRef

53.

Lindh J, Tondel M, Persson B, Vrethem M (2011) Health-related quality of life in patients with cryptogenic polyneuropathy compared with the general population. Disabil Rehabil 33(7):617–623PubMedCrossRef

54.

Rudolph T, Larsen JP, Farbu E (2009) Is there a need for long-term follow-up in chronic idiopathic polyneuropathy? Acta Neurol Scand 120(5):347–352PubMedCrossRef

55.

Takeuchi H, Misu K, Hattori N, Nagamatsu M, Sobue G (2000) Immunoglobulin therapy for idiopathic chronic sensory ataxic neuropathy. Neurology 54(4):1008–1010PubMedCrossRef

56.

Deleplanque P, Pourrat O, Alcalay D, Robert R, Neau JP, Gil R (1990) Treatment of idiopathic chronic polyneuropathy with plasma exchange alone. Prog Clin Biol Res 337:293–296PubMed

57.

Coyle PK, Schutzer SE, Sterman AB, Peress N, Miller F (1985) Humoral abnormalities in three patients with idiopathic chronic polyneuropathy. New York State J Med 85(6):248–250

58.

Uncini A, Sabatelli M, Mignogna T, Lugaresi A, Liguori R, Montagna P (1996) Chronic progressive steroid responsive axonal polyneuropathy: a CIDP vaariant or a primary axonal disorder? Muscle Nerve 19(3):365–371PubMedCrossRef

59.

Vrancken AF, van Schaik IN, Hughes RA, Notermans NC (2004) Drug therapy for chronic idiopathic axonal polyneuropathy. Cochrane Database Syst Rev 2004(2):Cd003456

60.

Attal N, Cruccu G, Baron R, Haanpaa M, Hansson P, Jensen TS et al (2010) EFNS guidelines on the pharmacological treatment of neuropathic pain: 2010 revision. Euro J Neurol Off J Euro Feder Neurol Soc 17(9):1113-e88

61.

Herrmann DN, Barbano RL, Hart-Gouleau S, Pennella-Vaughan J, Dworkin RH (2005) An open-label study of the lidocaine patch 5% in painful idiopathic sensory polyneuropathy. Pain Med (Malden, Mass) 6(5):379–384

62.

Schroder C, Johnston M, Teunissen L, Notermans N, Helders P, van Meeteren N (2007) Perceived control is a concurrent predictor of activity limitations in patients with chronic idiopathic axonal polyneuropathy. Arch Phys Med Rehabil 88(1):63–69PubMedCrossRef

Title: Chronic idiopathic axonal polyneuropathy: a systematic review
Authors: Panagiotis Zis
Ptolemaios Georgios Sarrigiannis
Dasappaiah Ganesh Rao
Channa Hewamadduma
Marios Hadjivassiliou
Publication date: 01-10-2016
Publisher: Springer Berlin Heidelberg
Published in: Journal of Neurology / Issue 10/2016
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-016-8082-7

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Chronic idiopathic axonal polyneuropathy: a systematic review

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