Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy

Introduction. Atypical variants of chronic inflammatory demyelinating polyneuropathy are marked by its clinical heterogeneity and variable disease course.

Aim of the study. To describe clinical, anamnestic and neurophysiological features of patients with atypical variants of chronic inflammatory demyelinating polyneuropathy, characterized by benign disease course, minimal motor involvement and not required pathogenic therapy.

Materials and methods. 8 patients (7 men (87 %) and 1 woman (13 %) at the age of 52–77 years) with atypical variants of chronic inflammatory demyelinating polyneuropathy were analyzed: 5 patients (62.5 %) with asymmetric variant – multifocal acquired demyelinating sensorimotor neuropathy and 3 patients (37.5 %) with sensory variant. All patients were observed at the Research Center of Neurology for the period of 2016– 2019. In each patient the proper clinical and laboratory evaluation was performed along with nerve conduction study and nerve ultrasound.

Results. The disease duration at the time of first visit was 1–8 years. By INCAT disability score 3 (37.5 %) patients had 0 points (normal), 3 (37.5 %) patients – 1 point and 1 patient had 2 (25 %) points. Nerve conduction study showed multifocal, asymmetric demyelinating changes in motor nerves. For the whole period of observation all patients were stable, so no one required pathogenic therapy.

Conclusion. Chronic inflammatory demyelinating polyneuropathy is a clinically heterogeneous disorder, required clinical suspicion in all patients over 50 years with features of multiple nerve involvement; nerve conduction study helps to detect typical changes, including subclinical ones. The primary strategy of management typical and atypical disease variants in stable course and minimal symptoms is a case follow-up with precise assessment of advantages and disadvantages of pathogenic therapy.

1. Broers M.C., Bunschoten C., Nieboer D. et al. Incidence and prevalence of chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis. Neuroepidemiology 2019;52 (3–4):161. DOI: 10.1159/000494291. PMID: 30669140.

2. Dyck P.J.B., Tracy J.A. History, diagnosis, and management of chronic inflammatory demyelinating polyradiculoneuropathy. Mayo Clinic Proc 2018;93 (6):777–93. DOI: 10.1016/j.mayocp.2018.03.026. PMID: 29866282.

3. Ikeda S., Koike H., Nishi R. et al. Clinicopathological characteristics of subtypes of chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Neurosurg Psychiatry 2019; 90(9):988–96. DOI: 10.1136/jnnp-2019–320741. PMID: 31227562.

4. Doneddu P.E., Cocito D., Manganelli F. et al. Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP database. J Neurol Neurosurg Psychiatry 2019;90(2):125–32. DOI: 10.1136/jnnp-2018–318714. PMID: 30297520.

5. van den Bergh P.Y., Hadden R.D., Bouche P. et al. 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, first revision. Joint Task Force of the EFNS and the PNS. J Peripher Nerv Syst 2010;15:1–9. DOI: 10.1111/j.1529–8027.2010.00245.x. PMID: 20456730.

6. Suponeva N.A., Naumova E.S., Nikitin S.S., Gnedovskaya E.V. Chronic inflammatory demyelinating polyneuropathy in adults: the principles of diagnostics and first-line therapy. Neuromuscular diseases = Neuromuscular diseases 2016;6(1):44–53. (In Russ.). DOI: 10.17650/2222-8721-2016-6-1-44-53.

7. Saperstein D., Katz J.S., Amato A., Barohn R.J. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve 2001;24:311–24. DOI: 10.1002/1097–4598(200103)24:33.0.co;2-a. PMID: 11353415.

8. Mathey E.K., Park S.B., Hughes R.A. et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry 2015;86:973–85. DOI: 10.1136/jnnp-2014–309697. PMID: 25677463.

9. Lucke I.M., Wieske L., van der Kooi A.J. et al. Diagnosis and treatment response in the asymmetric variant of chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst 2019;24(2):174–9. DOI: 10.1111/jns.12325. PMID: 31090121.

10. Kuwabara S., Misawa S., Mori M. Atypical chronic inflammatory demyelinating polyneuropathies. J Neurol Neurosurg Psychiatry 2019;90(2):121. DOI: 10.1136/jnnp-2018–319411. PMID: 16361595.

11. Chin R.L., Latov N., Sander H.W. et al. Sensory CIDP presenting as cryptogenic sensory polyneuropathy. J Peripher Nerv Syst 2004;9(3):132–7. DOI: 10.1111/j.1085–9489.2004.09302.x. PMID: 15363060.

12. Ayrignac X., Viala K., Koutlidis R.M. et al. Sensory chronic inflammatory demyelinating polyneuropathy: an underrecognized entity? Muscle Nerve 2013;48 (5):727–32. DOI: 10.1002/mus.23821. PMID: 23424105.

13. Breiner A., Barnett C., Bril V. INCAT disability score: a critical analysis of its measurement properties. Muscle Nerve 2014;50(2):164–9. DOI: 10.1002/mus.24207. PMID: 24723454.

14. Preston D.C., Shapiro B.E. Electromyography and neuromuscular disorders (Third Edition). Philadelphia: Elsevier Health Sciences, 2012. 664 p.

15. Grimm A., Décard B.F., Axer H., Fuhr P. The Ultrasound pattern sum score – UPSS. A new method to differentiate acute and subacute neuropathies using ultrasound of the peripheral nerves. Clin Neurophysiol 2015;126(11):2216–25. DOI: 10.1016/j.clinph.2015.01.011. PMID: 25691156.

16. Padua L., Granata G., Sabatelli M. et al. Heterogeneity of root and nerve ultrasound pattern in CIDP patients. Clin Neurophysiol 2014;125:160–5. DOI: 10.1016/j.clinph.2013.07.023. PMID: 24099922.

17. Lehmann H.C., Burke D., Kuwabara S. Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment. J Neurol Neurosurg Psychiatry 2019; 90(9):981–7. DOI: 10.1136/jnnp-2019–32031. PMID: 30992333.

18. Antonova K.V., Suponeva N.A., Shcherbakova N.I. et al. Glucocorticoid induced osteoporosis in neurological patients: prevention and treatment. Nervno-myshechnye bolezni = Neuromuscular Diseases 2017;7(2): 10–20. (In Russ.). DOI: 10.17650/2222-8721-2017-7-2-10-20.

19. Suponeva N.A., Piradov M.A. Intravenous immune therapy in neurology. M.: Goryachaya liniya telekom, 2013. 312 p. (In Russ.).

20. Gwathmey K.G., Tracy J.A., Dyck P.J.B. Peripheral nerve vasculitis: classification and disease associations. Neurol Clin 2019;37 (2):303–33. DOI: 10.1016/j.ncl.2019.01.013. PMID: 30952411.

21. Campagnolo M., Taioli F., Cacciavillani M. et al. Sporadic hereditary neuropathies misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy: Pitfalls and red flags. J Peripher Nerv Syst 2020;9:19–26. DOI: 10.1111/jns.12362. PMID:31919945.

22. Allen J.A., Lewis R.A. CIDP diagnostic pitfalls and perception of treatment benefit. Neurology 2015;11;85 (6): 498–504. DOI: 10.1212/WNL.0000000000001833. PMID: 26180143.

23. Grimm A., Vittore D., Schubert V. et al. Ultrasound pattern sum score, homogeneity score and regional nerve enlargement index for differentiation of demyelinating inflammatory and hereditary neuropathies. Clin Neurophysiol 2016;127(7):2618–24. DOI: 10.1016/j.clinph.2016.04.009. PMID: 27291881.

24. Vuytsik N.B., Suponeva N.A., Chechetkin A.O. et al. Nerve ultrasound in chronic inflammatory demyelinating polyneuropathy. Annaly klinicheskoy i eksperimental»noy nevrologii = Annals of clinical and experimental neurology 2014;8(3): 9–13. (In Russ.).

25. Scelsa S.N. Familial, demyelinating sensory and motor polyneuropathy with conduction block. Muscle Nerve 2010; 41(4):558–62. DOI: 10.1002/mus.21558. PMID: 19941344.

26. Allen J.A., Bril V. Improving the management of chronic inflammatory demyelinating polyradiculoneuropathy. Neurodegener Dis Manag 2016; 6(3):237–47. DOI: 10.2217/nmt2015–0011. PMID: 27230584.

27. Breiner A., Bourque P.R., Allen J.A. Updated cerebrospinal fluid total protein reference values improve chronic inflammatory demyelinating polyneuropathy diagnosis. Muscle Nerve 2019;60(2):180–3. DOI: 10.1002/mus.26488. PMID: 30989684.

28. Jomier F., Bousson V., Viala K. et al. Prospective study of the additional benefit of plexus magnetic resonance imaging in the diagnosis of chronic inflammatory demyelinating polyneuropathy. Eur J Neurol 2020;27(1):181–7. DOI: 10.1111/ene.14053. PMID: 31348848.

29. Kuwabara S., Isose S., Mori M. et al. Different electrophysiological profiles and treatment response in «typical» and «atypical» chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 2015;86:1054–9. DOI: 10.1136/jnnp-2014–308452. PMID: 25424435.

30. Oh S.J., Joy J.L., Kuruoglu R. «Chronic sensory demyelinating neuropathy»: chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry 1992;5(8):677–80. DOI: 10.1136/jnnp.55.8.677. PMID: 1326601.

31. Uncini A., Di Muzio A., De Angelis M.V. et al. Minimal and asymptomatic chronic inflammatory demyelinating polyneuropathy. Clin Neurophysiol 1999;110(4):694–8. DOI: 10.1016/s1388–2457(98)00070–4. PMID: 10378740