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Clinical and Ultrasound Scores Helps Differentiate CIDP from Diabetic Polyneuropathy

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Key Takeaways

  • ONLS and UPSS effectively differentiate CIDP from DPN, with high accuracy in diabetic patients.
  • The study involved 211 participants, revealing significant predictive value of ONLS and UPSS for CIDP.
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A new study identified key clinical and ultrasound markers that improved the differentiation between 2 conditions with overlapping features.

Hubertus Axer, MD  (Credit: ResearchGate)

Hubertus Axer, MD

(Credit: ResearchGate)

In a recent study published in Scientific Reports, use of the Overall Neuropathy Limitation Scale (ONLS) and the Ultrasound Pattern Sum Score (UPSS) demonstrated high accuracy in distinguishing between patient with chronic inflammatory demyelinating polyneuropathy (CIDP) and those with diabetic polyneuropathy (DPN). These findings suggest that these measures can be effective tools to address the challenge with differentiating between the 2 similar conditions.1

Researchers examined 211 participants, including 32 diabetic patients with CIDP, 68 patients with CIDP without diabetes, 83 patients with DPN, and 28 individuals who were diabetic without polyneuropathy. Findings revealed that ONLS was significantly higher in the patients with CIDP who had diabetes compared with DPN (P <.001). The same observation was made when using the UPSS (P <.001). Notably, multiple binary logistic regression demonstrated that both UPSS and ONLS were statistically significant predictors to differentiate between CIDP with diabetes and DPN.

Senior author Hubertus Axer, MD, assistant professor of neurology at Jena University Hospital in Germany, and colleagues wrote, “this study reports that UPSS is well suited to differentiate between diabetic patients with DPN and diabetic patients with CIDP. This may provide important information to facilitate the differential diagnosis of CIDP or to promote further medical tests such as cerebrospinal fluid analysis or nerve biopsy. In addition, it is important to propagate the information to general practitioners treating people with diabetes mellitus that rapid loss of function in people with often short diabetes duration is uncommon for DPN and should lead to neurological referral.”1

Investigators retrospectively collected data of patients diagnosed with CIDP according to published criteria2 from the Department of Neurology at Jena University Hospital or to the Neuromuscular Center at University Hospital Tübingen between 2018 and 2021. In the analysis, the patients with CIDP included were subdivided into a group with and without diabetes mellitus. Additionally, researchers assessed a database of patients with type 2 diabetes mellitus, with and without DPN, recruited in the SELECT study (DRKS00023026). Ultrasound studies of peripheral nerves, in only the CIDP cohort, and nerve conduction studies, in both cohorts, were performed by experienced neurologists.

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In the receiver operating characteristic curve analysis, findings revealed that the ONLS had an area under the curve (AUC) of 0.918 (95% CI, 0.868-0.0.967; P <.001) and the UPSS total score had an AUC of 0.826 (95% CI, 0.743–0.909; P <.001). Results showed that a UPSS score of at least 2.5 had a sensitivity of 77.4% and a specificity of 68.7% to detect CIDP. Authors then noted that an ONLS of at least 1.5 had a sensitivity of 87.1% and a specificity of 81.9% to detect CIDP. Additionally, ROC curve analysis of a composite score of ONLS and UPSS displayed an AUC of 0.959 (95% CI: 0.928–0.991, P <.001).

All told, the data for the patient cohorts were collected separately, and different ultrasound probes were used, introducing potential systematic bias. Additionally, the retrospective nature of the CIDP data prevented interequipment reliability assessments, which could be a significant limitation. Intrarater and interrater reliability (ICC) were also not evaluated. However, the study included a relatively large patient cohort, and the findings for NCS and ultrasound were consistent with existing literature. Factors such as CIDP or diabetes duration were not controlled, and most patients were not therapy-naïve, which may have influenced ultrasound results. Furthermore, cerebrospinal fluid analysis and nerve biopsy were not included in the evaluation of patients with DPN.

“Although NCS measurements showed the CIDP cohorts having more demyelinating changes than the diabetic cohorts, ROC curve analysis showed that a differentiation between DPN and CIDP in diabetic patients can be done more precisely by using the ultrasound measurements (represented by the UPSS),” Axer et al noted.1 “We used the UPSS as a comprehensive ultrasound score to further stratify our patient cohort. The score itself proved to be a reliable tool to further differentiate neuropathies in general, especially inflammatory from non-inflammatory neuropathies.”

REFERENCES
1. Heiling B, Kneer K, He W, et al. Nerve ultrasound helps to distinguish CIDP patients with diabetes from patients with diabetic polyneuropathy. Sci Rep. 2024;14(1):30504. Published 2024 Dec 16. doi:10.1038/s41598-024-82235-8
2. Joint Task Force of the EFNS and the PNS. 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 [published correction appears in J Peripher Nerv Syst. 2010 Dec;15(4):373]. J Peripher Nerv Syst. 2010;15(1):1-9. doi:10.1111/j.1529-8027.2010.00245.x
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