Differentiation Between Guillain–Barré Syndrome and Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuritis—a Prospective Follow-up Study Using Ultrasound and Neurophysiological Measurements
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Differentiation of Guillain–Barré syndrome (GBS) and acute-onset chronic inflammatory demyelinating polyradiculoneuritis (CIDP) might be intricate in early stages. We compared electrodiagnostics (EDx) and nerve ultrasound (NUS) as tools for early distinction and follow-up. NUS and EDx have been performed at first visitation and after 6 months. The nerve conduction study score (NCSS), the ultrasound pattern sum score (UPSS), and clinical scores were used for comparison. Compared with the 33 GBS patients, the 34 CIDP patients (50% with symptoms < 4 weeks) revealed significant nerve enlargement in ultrasound (p < 0.001) except for the roots and vagus, which exhibited increased values in both groups. EDx has no significant differences between both groups except for the A-wave frequency and the sural sparing pattern, which is more frequent in GBS (Fisher’s exact p < 0.05). In the latter, particularly, pure sensory nerves were not enlarged in contrast to CIDP, in which those were mostly enlarged (p < 0.001). This ultrasonic sensory sparing pattern (uSSP) in combination with enlarged roots/vagus is the hallmark finding in GBS with sensitivity, specificity, and positive predictive value > 85%, whereas in CIDP, enlarged sensory und multifocally enlarged sensorimotor nerves are key differentiation features to GBS. Increased echointensity of the nerves further arises only in CIDP. After 6 months, in CIDP, the significant nerve enlargement persisted, whereas in GBS, all segments almost normalized. Clinical, ultrasonic, and NCS scores correlated significantly over the time. Enlarged roots/vagus in combination with uSSP might facilitate differentiation of GBS and CIDP in the early stage, and ultrasonic 6-month normalization underlines the diagnosis of GBS in cases of uncertainty. Trial Registration: DRKS-ID 00005253
Key WordsCIDP GBS high-resolution ultrasound UPSS immune-mediated neuropathies
acute inflammatory demyelinating polyradiculoneuritis
acute motor (and sensory) neuropathy
- C5 and 6
cervical roots 5 and 6
chronic inflammatory demyelinating polyradiculoneuritis
compound muscle action potential
treated chronic neuritis
European Federation of Neurological Societies
inflammatory neuropathy cause and treatment disability score
multifocal acquired demyelinating sensory and motor neuropathy
medical research council sum score
nerve conduction studies
positive predictive value
ultrasound pattern sum score
Required Author Forms
Disclosure forms provided by the authors are available with the online version of this article.
Hannah Oertl, Natalie Winter, and Alexander Grimm designed the study and participated in data acquisition, analysis, interpretation, and drafting of the manuscript. Eva Auffenberg, Christoph Ruschil, Hubertus Axer, and Victoria Schubert participated in data acquisition and were involved in drafting the manuscript. All authors read, critically revised, and approved the final manuscript.
AG received reimbursement for travel and accommodation costs from Pfizer for a polyneuropathy symposium. AG and NW received grant from German Ultrasound Society (DEGUM).
Compliance with Ethical Standards
The study was registered in the German clinical trial registry (DRKS-ID 00005253) and approved by the local ethics committee (Tübingen 702/2015BO2). Written informed consent to study participation was obtained from all patients. The diagnosis of immune-mediated neuropathy was done by EFNS guidelines as well as according to Asbury et al. [5, 11] as recommended.
Conflict of Interest
The authors declare that they have no conflict of interest.
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