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Neurotherapeutics

, Volume 16, Issue 3, pp 838–847 | Cite as

Differentiation Between Guillain–Barré Syndrome and Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuritis—a Prospective Follow-up Study Using Ultrasound and Neurophysiological Measurements

  • Alexander GrimmEmail author
  • Hannah Oertl
  • Eva Auffenberg
  • Victoria Schubert
  • Christoph Ruschil
  • Hubertus Axer
  • Natalie Winter
Original Article

Abstract

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 Words

CIDP GBS high-resolution ultrasound UPSS immune-mediated neuropathies 

Abbreviations

AIDP

acute inflammatory demyelinating polyradiculoneuritis

AM(S)AN

acute motor (and sensory) neuropathy

C5 and 6

cervical roots 5 and 6

CIDP

chronic inflammatory demyelinating polyradiculoneuritis

CMAP

compound muscle action potential

TN

treated chronic neuritis

CSA

cross-sectional area

CSF

cerebrospinal fluid

EDx

electrodiagnostics

EFNS

European Federation of Neurological Societies

GBS

Guillain–Barré syndrome

HRUS

high-resolution ultrasound

INCAT

inflammatory neuropathy cause and treatment disability score

IVIG

intravenous immunoglobulin

MADSAM

multifocal acquired demyelinating sensory and motor neuropathy

MFS

Miller–Fisher syndrome

MRCSS

medical research council sum score

NCS

nerve conduction studies

NCSS

NCS score

PPV

positive predictive value

RTX

rituximab

UPSS

ultrasound pattern sum score

Notes

Required Author Forms

Disclosure forms provided by the authors are available with the online version of this article.

Authors’ Contributions

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.

Funding Information

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.

Supplementary material

13311_2019_716_MOESM1_ESM.pdf (490 kb)
ESM 1 (PDF 489 kb)

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Copyright information

© The American Society for Experimental NeuroTherapeutics, Inc. 2019

Authors and Affiliations

  • Alexander Grimm
    • 1
    • 2
    Email author
  • Hannah Oertl
    • 1
  • Eva Auffenberg
    • 1
    • 2
  • Victoria Schubert
    • 1
    • 2
  • Christoph Ruschil
    • 1
    • 2
  • Hubertus Axer
    • 3
  • Natalie Winter
    • 1
    • 2
  1. 1.Center of NeurologyTübingen University HospitalTübingenGermany
  2. 2.Hertie Institute for Clinical Brain ResearchUniversity of TübingenTübingenGermany
  3. 3.Department of NeurologyJena University HospitalJenaGermany

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