Skip to main content

Advertisement

SpringerLink
Log in

Neuropathic pain in patients with Charcot-Marie-Tooth type 1A

  • Original Article
  • Published:
Neurological Sciences Aims and scope Submit manuscript

Abstract

Background

Only several studies analyzed the characteristics of neuropathic pain (NeP) more extensively in patients with Charcot-Marie-Tooth type 1A (CMT1A). Therefore, we sought to determine the frequency and features of NeP in CMT1A patients and to assess the association between NeP and sociodemographic and clinical characteristics of patients with CMT1A.

Methods

Our research included 51 genetically diagnosed CMT1A patients. The International Association for the Study of Pain (IASP) criteria were used for diagnosis of NeP. PainDETECT questionnaire (PD-Q) was used to assess NeP features. The Medical Research Council (MRC) Sum Score, CMT Neuropathy Score (CMTNS), Overall Neuropathy Limitation Scale (ONLS) score, and Beck Depression Inventory were also used.

Results

NeP was present in 15 (29.4%) patients with CMT1A. The average intensity of pain was 5.7 ± 2.2 out of 10. The most sensitive neuropathic symptoms were numbness, then tingling, and burning sensations, while the most specific symptom was allodynia. Patients with NeP more frequently reported pain in the back (p < 0.01) and the trunk (p < 0.05). Patients with NeP had more pronounced disability of the upper extremities and overall disability, as assessed by the ONLS score (p < 0.05). Depression was more frequent in patients with NeP compared with patients without NeP (66.7 to 13.9%, p < 0.01).

Conclusion

NeP was present in almost one-third of the patients with CMT1A and it was moderate on average. Presence of NeP was associated with worse functional disability and depression.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Braathen GJ, Sand JC, Lobato A, Høyer H, Russell MB (2011) Genetic epidemiology of Charcot-Marie-Tooth in the general population. Eur J Neurol 18:39–48

    Article  CAS  Google Scholar 

  2. Saporta MA (2014) Charcot-Marie-Tooth disease and other inherited neuropathies. Continuum (MinneapMinn) 20:1208–1225

    Google Scholar 

  3. Jani-Ascadi, Krajewski K, Shy ME (2008) Charcot-Marie-Tooth: diagnosis and management. Semin Neurol 29:185–194

    Article  Google Scholar 

  4. Murphy SM, Laura M, Fawcett K et al (2012) Charcot-Marie-Tooth disease: frequency of genetic subtypes and guidelines for genetic testing. J Neurol Neurosurg Psychiatry 83:706–710

    Article  Google Scholar 

  5. Lupski JR, De Oca-Luna RM, Slaugenhaupt S et al (1991) DNA duplication associated with Charcot-Marie-Tooth disease type 1A. Cell 66:219–232

    Article  CAS  Google Scholar 

  6. Haanpää ML, Backonja MM, Bennett MI et al (2009) Assessment of neuropathic pain in primary care. Am J Med 122:13–21

    Article  Google Scholar 

  7. Pazzaglia C, Vollono C, Ferraro D, Virdis D, Lupi V, le Pera D, Tonali P, Padua L, Valeriani M (2010) Mechanisms of neuropathic pain in patients with Charcot-Marie Tooth 1A: a laser-evoked potential study. Pain 149:379–385

    Article  Google Scholar 

  8. Ribiere C, Bernardin M, Sacconi S, Delmont E, Fournier-Mehouas M, Rauscent H, Benchortane M, Staccini P, Lantéri-Minet M, Desnuelle C (2012) Pain assessment in Charcot-Marie-Tooth (CMT) disease. Ann Phys Rehabil Med 55(3):160–173

    Article  CAS  Google Scholar 

  9. Laurà M, Hutton EJ, Blake J, Lunn MP, Fox Z, Pareyson D, Solari A, Radice D, Koltzenburg M, Reilly MM (2014) Pain and small fiber function in Charcot-Marie-Tooth disease type 1A. Muscle Nerve 50(3):366–371

    Article  Google Scholar 

  10. Aarskog NK, Vedeler CA (2000) Real-time quantitative polymerase chain reaction. A new method that detects both the peripheral myelin protein 22 duplication in Charcot-Marie-Tooth type 1A disease and the peripheral myelin protein 22 deletion in hereditary neuropathy with liability to pressure palsies. Hum Genet 107(5):494–498

    Article  CAS  Google Scholar 

  11. Kleyweg RP, van der Meché FG, Schmitz PI (1991) Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain-Barré syndrome. Muscle Nerve 14(11):1103–1109

    Article  CAS  Google Scholar 

  12. Shy ME, Blake J, Krajewski K et al (2005) Reliability and validity of the CMT neuropathy score as a measure of disability. Neurology 64(7):1209–1214

    Article  CAS  Google Scholar 

  13. Graham R, Hughes R (2006) A modified peripheral neuropathy scale: the Overall Neuropathy Limitations Scale. J Neurol Neurosurg Psychiatry 77(8):973–976

    Article  CAS  Google Scholar 

  14. Beck AT, Steer RA, Carbin MG (1988) Psychometric properties of the Beck Depression Inventory: twenty-five years of evaluation. Clin Psychol Rev 8(1):77–100

    Article  Google Scholar 

  15. Treede RD, Jensen TS, Campbell JN, Cruccu G, Dostrovsky JO, Griffin JW, Hansson P, Hughes R, Nurmikko T, Serra J (2008) Neuropathic pain: redefinition and a grading system for clinical and research purposes. Neurology 70(18):1630–1635

    Article  CAS  Google Scholar 

  16. Freynhagen R, Baron R, Gockel U, Tölle TR (2006) painDETECT: a new screening questionnaire to identify neuropathic components in patients with back pain. Curr Med Res Opin 22(10):1911–1920

    Article  Google Scholar 

  17. Carter GT, Jensen MP, Galer BS, Kraft GH, Crabtree LD, Beardsley RM, Abresch RT, Bird TD (1998) Neuropathic pain in Charcot-Marie-Tooth disease. Arch Phys Med Rehabil 79:1560–1564

    Article  CAS  Google Scholar 

  18. Ramchandren S, Jaiswal M, Feldman E, Shy M (2014) Effect of pain in pediatric inherited neuropathies. Neurology 82(9):793–797

    Article  CAS  Google Scholar 

  19. Jensen MP, Abresch RT, Carter GT, McDonald CM (2005) Chronic pain in persons with neuromuscular disease. Arch Phys Med Rehabil 86(6):1155–1163

    Article  Google Scholar 

  20. Abresch RT, Carter GT, Jensen MP, Kilmer DD (2002) Assessment of pain and health-related quality of life in slowly progressive neuromuscular disease. Am J Hosp Palliat Med 19:39–48

    Article  Google Scholar 

  21. Tiffreau V, Viet G, Thevenon A (2006) Pain and neuromuscular disease. Am J Phys Med Rehabil 85(9):756–766

    Article  Google Scholar 

  22. Singleton JR (2005) Evaluation and treatment of painful peripheral polyneuropathy. Semin Neurol 25:185–195

    Article  Google Scholar 

  23. Van Paassen B, Van der Kooi A, Spaendonck-Zwarts VK, Verhamme C, Baas F, De Visser M (2014) PMP22 related neuropathies: Charcot-Marie-Tooth disease type 1A and hereditary neuropathy with liability to pressure palsies. Orphanet J Rare Dis 9:38

    Article  Google Scholar 

  24. Liao JP, Waclawik AJ (2004) Nerve root hypertrophy in CMT type 1A. Neurology 62(5):783

    Article  Google Scholar 

  25. Argoff CE (2007) The coexistence of neuropathic pain, sleep, and psychiatric disorders: a novel treatment approach. Clin J Pain 23(1):15–22

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, 6, Dr Subotic Street, Belgrade, 11 000, Serbia

    Bogdan Bjelica, Stojan Peric, Ivana Basta, Ivo Bozovic, Aleksandra Kacar, Ana Marjanovic, Vukan Ivanovic, Marija Brankovic, Milena Jankovic & Vidosava Rakocevic Stojanovic

  2. Institute of Human Genetics, School of Medicine, University of Belgrade, Belgrade, Serbia

    Ivana Novakovic

Authors
  1. Bogdan Bjelica
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Stojan Peric
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ivana Basta
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ivo Bozovic
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Aleksandra Kacar
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ana Marjanovic
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Vukan Ivanovic
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Marija Brankovic
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Milena Jankovic
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Ivana Novakovic
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Vidosava Rakocevic Stojanovic
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Stojan Peric.

Ethics declarations

This study was approved by the Ethical Committee of the Faculty of Medicine, University of Belgrade, and informed consent was obtained from all individual participants included in the study.

Conflict of interest

The authors declare that they have no conflict of interest.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Bjelica, B., Peric, S., Basta, I. et al. Neuropathic pain in patients with Charcot-Marie-Tooth type 1A. Neurol Sci 41, 625–630 (2020). https://doi.org/10.1007/s10072-019-04142-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10072-019-04142-5

Keywords

Search

Navigation