Use of Magnetic Resonance Neurography for Evaluating the Distribution and Patterns of Chronic Inflammatory Demyelinating Polyneuropathy

Abstract

Objective: To evaluate the distribution and characteristics of peripheral nerve abnormalities in chronic inflammatory demyelinating polyneuropathy (CIDP) using magnetic resonance neurography (MRN) and to examine the diagnostic efficiency. Materials and Methods: Thirty-one CIDP patients and 21 controls underwent MR scans. Three-dimensional sampling perfections with application-optimized contrasts using different flip-angle evolutions and T1-/T2-weighted turbo spinecho sequences were performed for neurography of the brachial and lumbosacral (LS) plexus and cauda equina, respectively. Clinical data and scores of the inflammatory Rasch-built overall disability scale (I-RODS) in CIDP were obtained. Results: The bilateral extracranial vagus (n = 11), trigeminal (n = 12), and intercostal nerves (n = 10) were hypertrophic. Plexus hypertrophies were observed in the brachial plexus of 19 patients (61.3%) and in the LS plexus of 25 patients (80.6%). Patterns of hypertrophy included uniform hypertrophy (17 [54.8%] brachial plexuses and 21 [67.7%] LS plexuses), and multifocal fusiform hypertrophy (2 [6.5%] brachial plexuses and 4 [12.9%] LS plexuses) was present. Enlarged and/or contrast-enhanced cauda equina was found in 3 (9.7%) and 13 (41.9%) patients, respectively. Diameters of the brachial and LS nerve roots were significantly larger in CIDP than in controls (p < 0.001). The largest AUC was obtained for the L5 nerve. There were no significant differences in the course duration, I-RODS score, or diameter between patients with and without hypertrophy. Conclusion: MRN is useful for the assessment of distribution and characteristics of the peripheral nerves in CIDP. Compared to other regions, LS plexus neurography is more sensitive for CIDP.

A Rare Phenotype of Uncommon Charcot–Marie–Tooth Genotypes Complicated With Inflammation Evaluated by Genetics and Magnetic Resonance Neurography

“…We found diffuse uniform hypertrophy of the brachial plexus, and multifocal fusiform hypertrophy of the LS plexus. The thickened pattern was similar to that of CIDP ( Su et al, 2020 ). The inherent mechanism should be evaluated further.…”

A rare presentation of POEMS syndrome on magnetic resonance neurography: a case series

“… 9 However, the presence of enhancement may not be useful in distinguishing between POEMS and CIDP, because it can also be observed in the latter group of patients. 12 – 14 Interestingly, it serves as a potential marker for detecting disease activity or treatment response.…”

Chronic inflammatory demyelinating polyneuropathy: A unique case of chronic disease with atypical features

“…The typical distribution of CIDP has been determined using MR Neurography in multiple studies, found to frequently involve the bilateral extracranial vagus, trigeminal, and intercostal nerves. The distribution of hypertrophy is typically uniform and bilateral [ 4 , 5 ]. The nerve roots, diffusely, are also known to hypertrophy in this disease.…”

“…The nerve roots, diffusely, are also known to hypertrophy in this disease. Other distributions, such as multifocal fusiform hypertrophy or unilateral involvement of the nerves may be seen in variant cases and atypical demonstrations of CIDP [ 4 , 5 ]. A 2003 case study presented a patient enduring a 10-year history of CIDP, with intercostal nerve enlargement in addition to brachial plexus and nerve root involvement illustrating the typical, expected distribution of disease [6] .…”

“…We propose that in addition to worsening symptomology, osseous remodeling and the widening of skull base foramina, spine foramina, and nerve tracks such as the mandibular groove of the inferior alveolar nerve, may have a correlation with chronicity of the disease. Chronicity of CIDP has been suggested to correlate with worsened symptomology, namely papilledema and proptosis in a 2013 case review article describing the ophthalmologic manifestations of CIDP [4] . In 1999, Duarte et al.…”