Evaluation of the Diagnostic Contributions of Nerve Ultrasound in Chronic Inflammatory Demyelinating Polyneuropathy Associating Systemic Diseases (CIDP Echo-nerf) (CIDP echo-nerf)

Evaluation of the Diagnostic Contributions of Nerve Ultrasound in Chronic Inflammatory Demyelinating Polyneuropathy Associating Systemic Diseases

Chronic inflammatory demyelinating polyradiculoneuritis (CIDP) is an autoimmune disorder of the peripheral nervous system, most commonly affecting the myelin sheath. The pathophysiology of CIDP is not completely understood, but both humoral and cellular immunity appear to be involved in the genesis of this disease. Some diseases are particularly associated with CIDP such as diabetes, monoclonal gammopathies and hematological diseases.

CIDP can occur before, after or simultaneously with the onset of systemic diseases. The systemic diseases most often seen in association with polyneuropathies are lupus, Gougerot-Sjögren's syndrome and sarcoidosis.

Ultrasound of peripheral nerves is a useful and accessible tool. In CIDP, this examination can reveal diffuse or segmental nerve hypertrophy. In addition to the size of the nerve, this exploration analyzes the echogenicity and the aspect of the different fascicles within the nerve. S. Goedee et al have shown that nerve ultrasound has very good diagnostic parameters and low interobserver variability in the diagnosis of CIDP. F. Härtig et al suggests that nerve ultrasound can predict the therapeutic response and describes 3 main patterns: hypoechoic enlargement (active inflammation), nerve enlargement with hyperechoic add-on fascicles (axonal degeneration) and almost no enlargement ("cured" CIDP).

Study Overview

• Status: Withdrawn
• Conditions: Chronic Inflammatory Demyelinating Polyradiculoneuropathy
• Intervention / Treatment: Other: None, pure observationnal study

Detailed Description

CIDP may be progressive or relapsing, most frequently clinically symmetric, sensorimotor with proximal and distal involvement developing over at least 8 weeks but variants as distal, multifocal, focal, motor or sensory CIDP have been also described. Cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to peripheral nerves but, by molecular mimicry it causes other autoimmune system diseases. This paper focuses on the intersection of CIDP and other autoimmune disease with an emphasis on shared pathology and mutually characteristics.

• Study Type: Observational

Contacts and Locations

Study Locations

• France
  • Nîmes, France, 30029
    • CHU de Nimes

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 16 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Patients followed at Nîmes University Hospital between 2012 and 2021 with CIDP

Description

Inclusion Criteria:

• Patients followed at Nîmes University Hospital between 2012 and 2021
• Age > 18 years
• Diagnosis of definite CIDP or possible CIDP according to the new EFNS/PNS 2021 criteria
• Diagnosis of definite CIDP/CIDP possible with systemic diseases and respectively diagnosis of definite CIDP/CIDP possible for the control group confirmed by electroneuromyography, concordant with the clinical examination

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Retrospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
CIDP associated with systemic diseases; Patients with CIDP associated with systemic diseases	Other: None, pure observationnal study; pure observationnal study
CIDP without systemic diseases; Patients with CIDP without other systemic diseases	Other: None, pure observationnal study; pure observationnal study

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Median nerve cross-sectional area	Comparison of median nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
ulnar nerve at wrist cross-sectional area	Comparison of ulnar nerve at wrist cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
ulnar at mid-arm nerve cross-sectional area	Comparison of ulnar nerve at mid-arm cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
ulnar nerve at elbow cross-sectional area	Comparison of ulnar nerve at elbow cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
radial nerve cross-sectional area	Comparison of radial nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Day of diagnosis of CIDP
superficial radial branch nerve cross-sectional area	Comparison of superficial radial branch nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
vagus nerve cross-sectional area	Comparison of vagus nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
C6 root nerve cross-sectional area	Comparison of C6 root nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
C5 root nerve cross-sectional area	Comparison of C5 root nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
sural nerve cross-sectional area	Comparison of sural nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
superficial fibula nerve cross-sectional area	Comparison of superficial fibula nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
tibial nerve at ankle cross-sectional area	Comparison of tibial nerve at ankle cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
popliteal nerve cross-sectional area	Comparison of popliteal nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
fibular nerve at neck cross-sectional area	Comparison of fibular nerve at neck cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0
fibular at supra-neck nerve cross-sectional area	Comparison of fibular at supra-neck nerve cross-sectional area on nerve ultrasound in patients with definite PIDC/PIDC with associated systemic disease with that of patients with PIDC/PIDC without systemic disease	Base line, Day 0

Collaborators and Investigators

• Sponsor: Centre Hospitalier Universitaire de Nīmes
• Investigators: Study Director: Anissa MEGZARI, Centre Hospitalier Universitaire de Nîmes

Publications and helpful links

General Publications

• Schmidt B, Toyka KV, Kiefer R, Full J, Hartung HP, Pollard J. Inflammatory infiltrates in sural nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996 Apr;19(4):474-87. doi: 10.1002/(SICI)1097-4598(199604)19:43.0.CO;2-9.
• Sommer C, Koch S, Lammens M, Gabreels-Festen A, Stoll G, Toyka KV. Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP. Neurology. 2005 Dec 27;65(12):1924-9. doi: 10.1212/01.wnl.0000188879.19900.b7.
• Spies JM, Westland KW, Bonner JG, Pollard JD. Intraneural activated T cells cause focal breakdown of the blood-nerve barrier. Brain. 1995 Aug;118 ( Pt 4):857-68. doi: 10.1093/brain/118.4.857.
• Madia F, Frisullo G, Nociti V, Conte A, Luigetti M, Del Grande A, Patanella AK, Iorio R, Tonali PA, Batocchi AP, Sabatelli M. pSTAT1, pSTAT3, and T-bet as markers of disease activity in chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2009 Jun;14(2):107-17. doi: 10.1111/j.1529-8027.2009.00220.x.
• Press R, Pashenkov M, Jin JP, Link H. Aberrated levels of cerebrospinal fluid chemokines in Guillain-Barre syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. J Clin Immunol. 2003 Jul;23(4):259-67. doi: 10.1023/a:1024532715775.
• Mei FJ, Ishizu T, Murai H, Osoegawa M, Minohara M, Zhang KN, Kira J. Th1 shift in CIDP versus Th2 shift in vasculitic neuropathy in CSF. J Neurol Sci. 2005 Jan 15;228(1):75-85. doi: 10.1016/j.jns.2004.10.001. Epub 2004 Nov 12.
• Kurihara M, Kurata Y, Sugimoto I, Hatanaka Y, Sakurai Y. High PR3-ANCA positivity in a patient with chronic inflammatory demyelinating polyneuropathy. eNeurologicalSci. 2016 Oct 5;6:4-5. doi: 10.1016/j.ensci.2016.10.001. eCollection 2017 Mar.
• Greenberg SA. P-ANCA vasculitic neuropathy with 12-year latency between onset of neuropathy and systemic symptoms. BMC Neurol. 2002 Oct 31;2(1):10. doi: 10.1186/1471-2377-2-10.
• Abraham H, Kuzhively J, Rizvi SW. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): An Uncommon Manifestation of Systemic Lupus Erythematosus (SLE). Am J Case Rep. 2017 Sep 12;18:980-983. doi: 10.12659/ajcr.903541.
• Siddiqui K, Cahalane E, Keogan M, Hardiman O. Chronic ataxic neuropathy with cold agglutinins: atypical phenotype and response to anti-CD20 antibodies. Neurology. 2003 Nov 11;61(9):1307-8. doi: 10.1212/wnl.61.9.1307. No abstract available.
• Rodriguez Y, Vatti N, Ramirez-Santana C, Chang C, Mancera-Paez O, Gershwin ME, Anaya JM. Chronic inflammatory demyelinating polyneuropathy as an autoimmune disease. J Autoimmun. 2019 Aug;102:8-37. doi: 10.1016/j.jaut.2019.04.021. Epub 2019 May 6.
• Seeliger T, Prenzler NK, Gingele S, Seeliger B, Korner S, Thiele T, Bonig L, Suhs KW, Witte T, Stangel M, Skripuletz T. Neuro-Sjogren: Peripheral Neuropathy With Limb Weakness in Sjogren's Syndrome. Front Immunol. 2019 Jul 11;10:1600. doi: 10.3389/fimmu.2019.01600. eCollection 2019.
• Wolbert J, Cheng MI, Meyer zu Horste G, Su MA. Deciphering immune mechanisms in chronic inflammatory demyelinating polyneuropathies. JCI Insight. 2020 Feb 13;5(3):e132411. doi: 10.1172/jci.insight.132411.

Study record dates

Study Major Dates

• Study Start (Actual): March 15, 2022
• Primary Completion (Estimated): December 1, 2023
• Study Completion (Estimated): December 1, 2023

Study Registration Dates

• First Submitted: February 15, 2022
• First Submitted That Met QC Criteria: February 24, 2022
• First Posted (Actual): February 25, 2022

Study Record Updates

• Last Update Posted (Actual): April 5, 2024
• Last Update Submitted That Met QC Criteria: April 4, 2024
• Last Verified: April 1, 2024

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Nervous System Diseases; Immune System Diseases; Autoimmune Diseases of the Nervous System; Demyelinating Diseases; Autoimmune Diseases; Disease Attributes; Neuromuscular Diseases; Peripheral Nervous System Diseases; Chronic Disease; Polyneuropathies; Polyradiculoneuropathy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
• Other Study ID Numbers: LOCAL/2022/II-01

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No