Хроническая воспалительная демиелинизирующая полинейропатия (ХВДП) – гетерогенная по течению и клиническим проявлениям хроническая дизиммунная нейропатия, относящаяся к категории курабельных. Многообразие клинических форм и особенности ее течения в ряде случаев обусловливают трудности в своевременной диагностике и инициации патогенетической терапии. В 1/4 случаев ХВДП дебютирует остро, имитируя при этом синдром Гийена–Барре. Крайне важно как можно раньше уточнить диагноз, поскольку терапевтическая тактика и прогноз данных заболеваний различаются. В настоящей статье мы представляем клинический случай острого дебюта ХВДП с подробным разбором дифференциальной диагностики между острой и хронической иммуноопосредованной нейропатией.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous treatable dysimmune neuropathy. The variety of clinical forms and course of the disease can be challenging for proper diagnosis and early treatment. In a quarter of cases CIDP starts acutely, mimicking Guillain–Barré syndrome. The early diagnosis is especially important regarding differences in treatment and prognosis of these conditions. In this article, we present a clinical case of acute onset CIDP with a detailed analysis of the differential diagnosis between acute and chronic immune-mediated neuropathies.
1. Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. J Peripher Nerv Syst. 2021;26(3):242-68. DOI:10.1111/jns.12455
2. Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain–Barre syndrome in ten steps. Nat Rev Neurol. 2019;15:671-83.
DOI:10.1038/s41582-019-0250-9
3. Doneddu PE, 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
4. Ruts L, Drenthen J, Jacobs BС, et al. Distinguishing acute-onset CIDP from fluctuating Guillain–Barre syndrome: a prospective study. Neurology. 2010;74(21):1680-6. DOI:10.1212/WNL.0b013e3181e07d14
5. Никитин С.С., Супонева Н.А., Пирадов М.А., и др. Синдром Гийена–Барре и хроническая воспалительная демиелинизирующая полинейропатия с острым началом: проблемы дифференциальной диагностики. Клиническая неврология. 2009;2:30-5 [Nikitin SS, Suponevа NА, Piradov МА, et al. Guillain–Barre syndrome and chronic inflammatory demyelinating polyneuropathy with acute start: differential diagnosis problems. Klinicheskaia nevrologiia. 2009;2:30-5 (in Russian)].
6. Fokke C, van den Berg B, Drenthen J, et al. Diagnosis of Guillain–Barre syndrome and validation of Brighton criteria. Brain. 2014;137(1):33-43. DOI:10.1093/brain/awt285
7. Kerasnoudis A, Pitarokoili K, Behrendt V, et al. Nerve ultrasound score in distinguishing chronic from acute inflammatory demyelinating polyneuropathy. Clin Neurophysiol. 2014;125:635-41. DOI:10.1016/j.clinph.2013.08.014
8. Grimm A, Decard BF, 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
9. Uncini A, Kuwabara S. Electrodiagnostic criteria for Guillain–Barre syndrome: a critical revision and the need for an update. Clin Neurophysiol. 2012;123(8):1487-95.
DOI:10.1016/j.clinph.2012.01.025
10. Uncini A, Ippoliti L, Shahrizaila N, et al. Optimizing the electrodiagnostic accuracy in Guillain–Barre syndrome subtypes: criteria sets and sparse linear discriminant analysis. Clin Neurophysiol. 2017;128(7):1176-83. DOI:10.1016/j.clinph.2017.03.048
11. Пирадов М.А., Супонева Н.А., Гришина Д.А. Полинейропатии: алгоритмы диагностики и лечения. М.: Горячая линия-Телеком, 2019 [Piradov MA, Suponeva NA, Grishina DA. Polineiropatii: algoritmy diagnostiki i lecheniia. Moscow: Goryachaya liniya-Telekom, 2019 (in Russian)].
12. Dionne A, Nicolle MW, Hahn AF. Clinical and electrophysiological parameters distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy. Muscle Nerve. 2010;41(2):202-7. DOI:10.1002/mus.21480
13. Alessandro L, Rueda JMP, Wilken M, et al. Differences between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and acute inflammatory demyelinating polyneuropathy (AIDP) in adult patients. J Peripher Nerv Syst. 2018;23:154-8. DOI:10.1111/jns.12266
14. Супонева Н.А., Мочалова Е.Г., Гришина Д.А., Пирадов М.А. Особенности течения СГБ в России: анализ 186 случаев. Нервно-мышечные болезни. 2014;(1):37-46 [Suponeva NA, Mochalova EG, Grishina DA, Piradov MA. The specific features of Guillain–Barre syndrome in Russia: Analysis of 186 cases. Neuromuscular Diseases. 2014;(1):37-46 (in Russian)]. DOI:10.17650/2222-8721-2014-0-1-40-47
15. Hughes RAC, Brassington R, Gunn AA, van Doorn PA. Corticosteroids for Guillain–Barre syndrome. Cochr Database Syst Rev. 2016;10(10):CD001446. DOI:10.1002/14651858.CD001446.pub5
16. Zivkovic SA, Peltier AC, Iacob T, Lacomis D. Chronic inflammatory demyelinating polyneuropathy and ventilatory failure: report of seven new cases and review of the literature. Acta Neurol Scand. 2011;124:59‑63. DOI:10.1111/j.1600-0404.2010.01431.x
17. Вуйцик Н.Б., Супонева Н.А., Чечеткин А.О., и др. Ультразвуковая нейровизуализация при хронической воспалительной демиелинизирующей полиневропатии. Анналы клинической и экспериментальной неврологии. 2014;8(3):9-13 [Vuytsik NV, Suponevа NА, Chechetkin AO, et al. Ultrasound neuroimaging in chronic inflammatory demyelinating polyneuropathy. Annals of Clinical and Experimental Neurology. 2014;8(3):9-13 (in Russian)].
18. Kerasnoudis A, Pitarokoili K, Behrendt V, et al. Bochum ultrasound score versus clinical and electrophysiological parameters in distinguishing acute-onset chronic from acute inflammatory demyelinating polyneuropathy. Muscle Nerve. 2015;51(6):846-52. DOI:10.1002/mus.24484
19. Grimm A, Oertl H, Auffenberg E, et al. Differentiation Between Guillain–Barre Syndrome and Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuritis – a Prospective Follow-up Study Using Ultrasound and Neurophysiological Measurements. Neurotherapeutics. 2019;16(3):838-47. DOI:10.1007/s13311-019-00716-5
20. Berciano J, Sedano MJ, Pelayo-Negro AL, et al. Proximal nerve lesions in early Guillain–Barre syndrome: implications for pathogenesis and disease classification. J Neurol. 2017;264(2):221-36. DOI:10.1007/s00415-016-8204-2
21. Fehmi J, Scherer SS, Willison HJ. Nodes, paranodes and neuropathies. J Neurol Neurosurg Psychiatry. 2018;89(1):61-71. DOI:10.1136/jnnp-2016-315480
22. AlMohammedi R, AlJohani S, Bakheet M. Pulse-steroid therapy in a 37-year-old man with acute motor and sensory axonal neuropathy: A case report. Clin Case Rep. 2019;7(3):506-8. DOI:10.1002/ccr3.2000
________________________________________________
1. Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. J Peripher Nerv Syst. 2021;26(3):242-68. DOI:10.1111/jns.12455
2. Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain–Barre syndrome in ten steps. Nat Rev Neurol. 2019;15:671-83.
DOI:10.1038/s41582-019-0250-9
3. Doneddu PE, 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
4. Ruts L, Drenthen J, Jacobs BС, et al. Distinguishing acute-onset CIDP from fluctuating Guillain–Barre syndrome: a prospective study. Neurology. 2010;74(21):1680-6. DOI:10.1212/WNL.0b013e3181e07d14
5. Nikitin SS, Suponevа NА, Piradov МА, et al. Guillain–Barre syndrome and chronic inflammatory demyelinating polyneuropathy with acute start: differential diagnosis problems. Klinicheskaia nevrologiia. 2009;2:30-5 (in Russian).
6. Fokke C, van den Berg B, Drenthen J, et al. Diagnosis of Guillain–Barre syndrome and validation of Brighton criteria. Brain. 2014;137(1):33-43. DOI:10.1093/brain/awt285
7. Kerasnoudis A, Pitarokoili K, Behrendt V, et al. Nerve ultrasound score in distinguishing chronic from acute inflammatory demyelinating polyneuropathy. Clin Neurophysiol. 2014;125:635-41. DOI:10.1016/j.clinph.2013.08.014
8. Grimm A, Decard BF, 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
9. Uncini A, Kuwabara S. Electrodiagnostic criteria for Guillain–Barre syndrome: a critical revision and the need for an update. Clin Neurophysiol. 2012;123(8):1487-95.
DOI:10.1016/j.clinph.2012.01.025
10. Uncini A, Ippoliti L, Shahrizaila N, et al. Optimizing the electrodiagnostic accuracy in Guillain–Barre syndrome subtypes: criteria sets and sparse linear discriminant analysis. Clin Neurophysiol. 2017;128(7):1176-83. DOI:10.1016/j.clinph.2017.03.048
11. Piradov MA, Suponeva NA, Grishina DA. Polineiropatii: algoritmy diagnostiki i lecheniia. Moscow: Goryachaya liniya-Telekom, 2019 (in Russian).
12. Dionne A, Nicolle MW, Hahn AF. Clinical and electrophysiological parameters distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy. Muscle Nerve. 2010;41(2):202-7. DOI:10.1002/mus.21480
13. Alessandro L, Rueda JMP, Wilken M, et al. Differences between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and acute inflammatory demyelinating polyneuropathy (AIDP) in adult patients. J Peripher Nerv Syst. 2018;23:154-8. DOI:10.1111/jns.12266
14. Suponeva NA, Mochalova EG, Grishina DA, Piradov MA. The specific features of Guillain–Barre syndrome in Russia: Analysis of 186 cases. Neuromuscular Diseases. 2014;(1):37-46 (in Russian). DOI:10.17650/2222-8721-2014-0-1-40-47
15. Hughes RAC, Brassington R, Gunn AA, van Doorn PA. Corticosteroids for Guillain–Barre syndrome. Cochr Database Syst Rev. 2016;10(10):CD001446. DOI:10.1002/14651858.CD001446.pub5
16. Zivkovic SA, Peltier AC, Iacob T, Lacomis D. Chronic inflammatory demyelinating polyneuropathy and ventilatory failure: report of seven new cases and review of the literature. Acta Neurol Scand. 2011;124:59‑63. DOI:10.1111/j.1600-0404.2010.01431.x
17. Vuytsik NV, Suponevа NА, Chechetkin AO, et al. Ultrasound neuroimaging in chronic inflammatory demyelinating polyneuropathy. Annals of Clinical and Experimental Neurology. 2014;8(3):9-13 (in Russian).
18. Kerasnoudis A, Pitarokoili K, Behrendt V, et al. Bochum ultrasound score versus clinical and electrophysiological parameters in distinguishing acute-onset chronic from acute inflammatory demyelinating polyneuropathy. Muscle Nerve. 2015;51(6):846-52. DOI:10.1002/mus.24484
19. Grimm A, Oertl H, Auffenberg E, et al. Differentiation Between Guillain–Barre Syndrome and Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuritis – a Prospective Follow-up Study Using Ultrasound and Neurophysiological Measurements. Neurotherapeutics. 2019;16(3):838-47. DOI:10.1007/s13311-019-00716-5
20. Berciano J, Sedano MJ, Pelayo-Negro AL, et al. Proximal nerve lesions in early Guillain–Barre syndrome: implications for pathogenesis and disease classification. J Neurol. 2017;264(2):221-36. DOI:10.1007/s00415-016-8204-2
21. Fehmi J, Scherer SS, Willison HJ. Nodes, paranodes and neuropathies. J Neurol Neurosurg Psychiatry. 2018;89(1):61-71. DOI:10.1136/jnnp-2016-315480
22. AlMohammedi R, AlJohani S, Bakheet M. Pulse-steroid therapy in a 37-year-old man with acute motor and sensory axonal neuropathy: A case report. Clin Case Rep. 2019;7(3):506-8. DOI:10.1002/ccr3.2000