Su Xiaoyun1, Zheng Chuansheng1, Kong Xiangquan1, Lu Zuneng2, Osamah Alwalid1, and Zhang Huiting3
1Radiology, union hospital, tongji medical college, Huazhong University of science and technology, Wuhan, China, 2Neurology, Renming Hospital of Wuhan University,, Wuhan, China, 3MR Scientific Marketing, Siemens Healthcare, Shanghai, China
Synopsis
Conventional imaging is insufficient for
diagnosis and management of patients with chronic inflammatory demyelinating
polyneuropathy (CIDP). Brachial and lumbosacral plexus of 37 CIDP patients and
37 age and gender-matched controls were examined by using multi-sequences. Our
study showed that fractional anisotropy (FA) had the most
sensitivity, while contrast-enhanced ratio (CR) had the
most specificity in single-parameter. The combination of FA and CR value had
the best diagnostic efficiency. FA had a negative correlation with course duration, and CR positive correlation with CSF protein. SPACE combined with
DTI and contrast enhancement sequences serve as a recommended composite protocol.
INTRODUCTION
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an
acquired immune-mediated and treatable demyelinating disorder of the peripheral nervous system 1,2.
The
diagnosis and management of CIDP can be difficult, and is mainly based on
clinical features and nerve conduction studies 2-4.
CIDP is most frequently observed in adult men, and has an annual
incidence of 0.48 per 100,000 individuals in the population 5.
The aims of this study were to evaluate the diagnostic
performance and abnormalities of brachial and
lumbosacral (LS) plexus via quantitatively multimodal MR imaging for CIDP, and to come up
with optimal combined parameters. Concurrently, the potential correlations
between clinical parameters with multi-parameters of MR were investigated. METHODS
A total of 37 typical patients with CIDP
were recruited based on the European Federation of Neurological Societies /
Peripheral Nerve Society diagnostic criteria. The Inflammatory Rasch-built Overall Disability
Scale (I-RODS) questionnaire scores 6,
disease duration and cerebrospinal fluid protein values were documented. Brachial and LS plexus
of 37 CIDP patients and 37 age- and gender-matched healthy controls were examined on a 3T MR scanner (MAGNETOM Trio, Siemens
Healthcare, Erlangen, Germany). The sequences included the volumetric interpolated
breath-hold examination (VIBE), turbo
inversion recovery magnitude (TIRM), sampling perfection with application-optimized
contrasts using different flip angle evolution (SPACE) and diffusion tensor
imaging (DTI). Nerve diameter, nerve-to-muscle T2 signal intensity ratio (nT2),
contrast-enhanced ratio (CR),
fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were determined
in brachial and LS plexus, and tractography was performed. Wilcoxon signed-rank test was
used to assess the differences between the patients and controls. Receiver operating characteristic (ROC) curve analysis was used to
determine the diagnostic efficiency. Spearman rank
correlation test was performed to investigate for the correlations among parameters. Intraclass correlation coefficients (ICC) were calculated to the consistency of inter- and intra-observer. Two-tailed P <
0.05 was considered statistically significant.RESULTS
Hypertrophy
involved the brachial (n=22/37) and LS (n=27/36) plexus in the CIDP patients,
and no hypertrophy in controls. The
representative hypertrophy, enhancement and diffusion tensor tractographies
were illustrated as in the brachial and LS plexus of CIDP patients (Figure 1) The diameters of the C5-C8, L4-S1
nerve roots, sciatic and femoral nerves were significantly larger in CIDP
patients than in healthy controls (all P< .01). As shown in Figure 2,
CR, nT2 and ADC were
significantly higher while the FA was lower in CIDP patients than in controls (P< .01). The AUC among each parameter are higher in the LS plexus
than brachial plexus. The ROC curves of the single-parameter and combined parameters models in
the LS plexus were showed in Figure 3. FA had the highest sensitivity (.809),
accuracy (.73) and AUC (.925), while the highest specificity was .961 for CR in single-parameter in the LS
plexus. The combination of FA and CR
demonstrated the highest sensitivity (.922), specificity (.951), accuracy (.87)
and AUC (.973) in the LS plexus. FA had a weak negative correlation with course
duration (r=
-.302, P=.04), and CR had a
moderate positive correlation with CSF protein (r= .415, P=.01). No correlation was found between the I-RODs with MR
parameters (P range, 0.33-0.91). CR only had a weak correlation with nT2. ADC and diameter had moderate positive correlation with
nT2, and the diameter and nT2 had moderate negative correlation with FA in
CIDP; while there was only a weak or no correlation between these parameters in
controls. The correlation
results are summarized in Table 1. There were good inter- and intra-observer consistencies
(ICC range, 0.754-0.894) for each parameter assessed in the plexus.DISCUSSION
Although previous studies have exclusively
reported DTI parameters alterations in the lower limb nerves in CIDP patients, 7,8 our study
first assessed the diagnostic accuracy of DTI with
tractography in the brachial and LS plexus, which complemented the results
from the literature. FA value decrease is likely due to abnormal myelin
development as a result of repeated loss, repair and axonal degeneration, which
in turn leads to less molecule diffusion along long nerve axes. The tight junctions of blood-nerve
barrier provide a barrier to the diffusion of various tracers with larger
molecular weight 9. Early studies have indicated a decreased number of tight junction
proteins claudin 5 and ZO-1 in the sural nerve biopsy specimens of CIDP
patients 1,10. We reported the utility of contrast enhancement to quantify
alteration of the BNB permeability in the plexus. Our results revealed
that CR had the advantage of high specificity,
and had a moderate positive correlation with CSF protein. CR is likely to be a
potential non-invasive repeatable MR biomarker for detecting and monitoring
disease activity.CONCLUSION
Multimodal MR imaging possesses a high diagnostic accuracy of CIDP in
the LS plexus. SPACE combined with DTI and contrast enhancement serve as a recommended composite protocol for assessing
demyelinating polyneuropathy.Acknowledgements
This study was supported by the National Natural Science Foundation of China (Grant
No. 81470076). We thank our statistician colleagues who helped the data analysis.
References
1. Vallat
JM, Sommer C, Magy L. Chronic inflammatory demyelinating
polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable
condition. Lancet Neurol
2010;9(4):402-12. doi: 10.1016/S1474-4422(10)70041-7
2. Latov N. Diagnosis and treatment of chronic acquired demyelinating
polyneuropathies. Nat Rev Neurol
2014;10(8):435-46. doi: 10.1038/nrneurol.2014.117
3. Rajabally YA, Stettner M, Kieseier BC, et al. CIDP and other
inflammatory neuropathies in diabetes — diagnosis and management. Nature Reviews
Neurology 2017;13(10):599-611. doi:
10.1038/nrneurol.2017.123
4. Querol L, Devaux J, Rojas-Garcia R, et al. Autoantibodies in chronic inflammatory neuropathies: diagnostic and
therapeutic implications. Nat Rev Neurol
2017;13(9):533-47. doi: 10.1038/nrneurol.2017.84
5. Iijima M, Koike H, Hattori N, et al. Prevalence and incidence rates of
chronic inflammatory demyelinating polyneuropathy in the Japanese population. J Neurol Neurosurg Psychiatry
2008;79(9):1040-3. doi: 10.1136/jnnp.2007.128132 [published Online First:
2008/01/29]
6. van Nes SI, Vanhoutte EK, van Doorn PA, et al. Rasch-built Overall
Disability Scale (R-ODS) for immune-mediated peripheral neuropathies. Neurology 2011;76(4):337-45. doi: DOI 10.1212/WNL.0b013e318208824b
7. Lichtenstein T, Sprenger A, Weiss K, et al. MRI biomarkers of proximal nerve injury in CIDP. Ann Clin Transl Neurol 2018;5(1):19-28. doi: 10.1002/acn3.502
8. Kronlage M, Pitarokoili K, Schwarz D, et al. Diffusion Tensor Imaging
in Chronic Inflammatory Demyelinating Polyneuropathy: Diagnostic Accuracy and
Correlation With Electrophysiology. Invest
Radiol 2017;52(11):701-07. doi: 10.1097/RLI.0000000000000394
9. Olsson Y, Reese TS. Permeability of vasa nervorum and perineurium in
mouse sciatic nerve studied by fluorescence and electron microscopy. J Neuropathol Exp Neurol
1971;30(1):105-19.
10. Kanda T, Numata Y, Mizusawa H. Chronic inflammatory demyelinating
polyneuropathy: decreased claudin-5 and relocated ZO-1. J Neurol Neurosurg Psychiatry 2004;75(5):765-9.