Nerve ultrasound improves detection of treatment-responsive chronic inflammatory neuropathies

ObjectiveTo examine the diagnostic accuracy of nerve ultrasound in a prospective cohort of consecutive patients with a clinical suspicion of chronic inflammatory neuropathies, including chronic inflammatory demyelinating polyneuropathy, Lewis-Sumner syndrome, and multifocal motor neuropathy, and to determine the added value in the detection of treatment-responsive patients.MethodsBetween February 2015 and July 2018, we included 100 consecutive incident patients with a clinical suspicion of chronic inflammatory neuropathy. All patients underwent nerve ultrasound, extensive standardized nerve conduction studies (NCS), and other relevant diagnostic investigations. We evaluated treatment response using predefined criteria. A diagnosis of chronic inflammatory neuropathy was established when NCS were abnormal (fulfilling criteria of demyelination of the European Federation of Neurological Societies/Peripheral Nerve Society) or when the degree of nerve enlargement detected by sonography was compatible with chronic inflammatory neuropathy and there was response to treatment.ResultsA diagnosis of chronic inflammatory neuropathy was established in 38 patients. Sensitivity and specificity of nerve ultrasound and NCS were 97.4% and 69.4% and 78.9% and 93.5%, respectively. The added value of nerve ultrasound in detection of treatment-responsive chronic inflammatory neuropathy was 21.1% compared to NCS alone.ConclusionsNerve ultrasound and NCS are complementary techniques with superior sensitivity in the former and specificity in the latter. Addition of nerve ultrasound significantly improves the detection of chronic inflammatory neuropathies. Therefore, it deserves a prominent place in the diagnostic workup of chronic inflammatory neuropathies.Classification of evidenceThis study provides Class IV evidence that nerve ultrasound is an accurate diagnostic tool to detect chronic inflammatory neuropathies.

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Complications of Immunoglobulin Therapy and Implications for Treatment of Inflammatory Neuropathy: A Review

Current Drug Safety ◽

10.2174/1574886313666181017121139 ◽

2019 ◽

Vol 14 (1) ◽

pp. 3-13 ◽

Cited By ~ 5

Author(s):

Ahmed Abbas ◽

Yusuf A. Rajabally.

Keyword(s):

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Kidney Injury ◽

Immunoglobulin Therapy ◽

Multifocal Motor Neuropathy ◽

Evidence Base ◽

Motor Neuropathy ◽

Inflammatory Neuropathy ◽

Inflammatory Neuropathies ◽

Delayed Complications ◽

Associated Risk Factors

Background: Intravenous Immunoglobulin (IVIg) forms a cornerstone of effective treatment for acute and chronic inflammatory neuropathies, with a class I evidence base in Guillain-Barré Syndrome (GBS), Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). It is generally considered to be a safe therapy however there are several recognised complications which are reviewed in this article. Discussion and Conclusion: Most adverse events are immediate and mild such as headache, fever and nausea although more serious immediate reactions such as anaphylaxis may rarely occur. Delayed complications are rare but may be serious, including thromboembolic events and acute kidney injury, and these and associated risk factors are also discussed. We emphasise the importance of safe IVIg administration and highlight practical measures to minimise complications of this therapy.

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Nerve ultrasound for diagnosing chronic inflammatory neuropathy

Neurology ◽

10.1212/wnl.0000000000010369 ◽

2020 ◽

Vol 95 (12) ◽

pp. e1745-e1753

Author(s):

Ingrid J.T. Herraets ◽

H. Stephan Goedee ◽

Johan A. Telleman ◽

Ruben P.A. van Eijk ◽

Camiel Verhamme ◽

...

Keyword(s):

Diagnostic Accuracy ◽

Sensitivity And Specificity ◽

Multicenter Study ◽

Added Value ◽

Response Alternative ◽

European Federation ◽

Motor Neuropathy ◽

Reference Standard ◽

Nerve Ultrasound ◽

Inflammatory Neuropathy

ObjectiveTo validate the diagnostic accuracy of a previously described short sonographic protocol to identify chronic inflammatory neuropathy (CIN), including chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis Sumner syndrome, and multifocal motor neuropathy (MMN), and to determine the added value of nerve ultrasound to detect treatment-responsive patients compared to nerve conduction studies (NCS) in a prospective multicenter study.MethodsWe included 100 consecutive patients clinically suspected of CIN in 3 centers. The study protocol consisted of neurologic examination, laboratory tests, NCS, and nerve ultrasound. We validated a short sonographic protocol (median nerve at forearm, upper arm, and C5 nerve root) and determined its diagnostic accuracy using the European Federation of Neurological Societies/Peripheral Nerve Society criteria of CIDP/MMN (reference standard). In addition, to determine the added value of nerve ultrasound in detecting treatment-responsive patients, we used previously published diagnostic criteria based on clinical, NCS, and sonographic findings and treatment response (alternative reference standard).ResultsSensitivity and specificity of the sonographic protocol for CIN according to the reference standard were 87.4% and 67.3%, respectively. Sensitivity and specificity of this protocol according to the alternative reference standard were 84.6% and 72.8%, respectively, and of NCS 76.1% and 93.4%. With addition of nerve ultrasound, 44 diagnoses of CIN were established compared to 33 diagnoses with NCS alone.ConclusionsA short sonographic protocol shows high diagnostic accuracy for detecting CIN. Nerve ultrasound is able to detect up to 25% more patients who respond to treatment.Classification of evidenceThis multicenter study provides Class IV evidence that nerve ultrasound improves diagnosis of CIN.

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Quantitative assessment of brachial plexus MRI for the diagnosis of chronic inflammatory neuropathies

Journal of Neurology ◽

10.1007/s00415-020-10232-8 ◽

2020 ◽

Author(s):

Marieke H. J. van Rosmalen ◽

H. Stephan Goedee ◽

Anouk van der Gijp ◽

Theo D. Witkamp ◽

Ruben P. A. van Eijk ◽

...

Keyword(s):

Brachial Plexus ◽

Nerve Root ◽

Interrater Reliability ◽

Quantitative Assessment ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Diagnostic Value ◽

Response To Treatment ◽

Motor Neuropathy ◽

Nerve Ultrasound ◽

Cross Sectional

Abstract Objective This study aimed at developing a quantitative approach to assess abnormalities on MRI of the brachial plexus and the cervical roots in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) and to evaluate interrater reliability and its diagnostic value. Methods We performed a cross-sectional study in 50 patients with CIDP, 31 with MMN and 42 disease controls. We systematically measured cervical nerve root sizes on MRI bilaterally (C5, C6, C7) in the coronal [diameter (mm)] and sagittal planes [area (mm2)], next to the ganglion (G0) and 1 cm distal from the ganglion (G1). We determined their diagnostic value using a multivariate binary logistic model and ROC analysis. In addition, we evaluated intra- and interrater reliability. Results Nerve root size was larger in patients with CIDP and MMN compared to controls at all predetermined anatomical sites. We found that nerve root diameters in the coronal plane had optimal reliability (intrarater ICC 0.55–0.87; interrater ICC 0.65–0.90). AUC was 0.78 (95% CI 0.69–0.87) for measurements at G0 and 0.81 (95% CI 0.72–0.91) for measurements at G1. Importantly, our quantitative assessment of brachial plexus MRI identified an additional 10% of patients that showed response to treatment, but were missed by nerve conduction (NCS) and nerve ultrasound studies. Conclusion Our study showed that a quantitative assessment of brachial plexus MRI is reliable. MRI can serve as an important additional diagnostic tool to identify treatment-responsive patients, complementary to NCS and nerve ultrasound.

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WED 223 Predicting disease activity in inflammatory neuropathies

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-abn.112 ◽

2018 ◽

Vol 89 (10) ◽

pp. A32.3-A32

Author(s):

Kapoor Mahima ◽

Carr Aisling ◽

Lunn Michael ◽

M Reilly Mary

Keyword(s):

Disease Activity ◽

Upper Limb ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Multifocal Motor Neuropathy ◽

Ivig Treatment ◽

Motor Neuropathy ◽

Significant Difference ◽

Successful Group ◽

Inflammatory Neuropathies

BackgroundThere is currently no accurate way to determine who will need long-term immunoglobulin (IVIg) treatment in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN).AimsRetrospectively compare clinical, investigational and treatment factors in patients who have successfully ceased IVIg with patients who have active disease.Methods15 patients who have successfully suspended IVIg infusions were compared with 15 in whom decreasing the IVIg dose was unsuccessful.Results30 patients (12 with CIDP and 3 with MMN in both groups) were diagnosed 39.5 months from onset of symptoms in the successful group vs 40.7 months in the unsuccessful group (p=0.953). There was a significant difference in the summed upper limb sensory amplitudes on electrophysiology prior to starting IVIg between the patients with CIDP (17.4 mV vs 9.8 mV p=0.007). There was no difference between the average doses between the groups. A successful cessation trial was attempted at a mean of 60.5 months post starting treatment, compared with 60 months in the unsuccessful patients.ConclusionOther than the differences in initial upper limb amplitudes, other factors did not help predict a successful cessation trial of IVIg. This reinforces the need for an objective biomarker to measure disease activity.

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Intravenous immunoglobulin for treatment of chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy in France: are daily practices in accordance with guidelines?

European Journal of Neurology ◽

10.1111/ene.13841 ◽

2018 ◽

Vol 26 (4) ◽

pp. 575-580 ◽

Cited By ~ 3

Author(s):

C. Rosier ◽

N. Graveline ◽

A. Lacour ◽

J.‐C. Antoine ◽

J.‐P. Camdessanché

Keyword(s):

Intravenous Immunoglobulin ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Multifocal Motor Neuropathy ◽

Demyelinating Polyneuropathy ◽

Motor Neuropathy ◽

Inflammatory Demyelinating Polyneuropathy

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Disorders of peripheral nerves and motor neuron disease

10.1093/med/9780199664368.003.0007 ◽

2016 ◽

Author(s):

Sathiji Nageshwaran ◽

Heather C Wilson ◽

Anthony Dickenson ◽

David Ledingham

Keyword(s):

Motor Neuron ◽

Motor Neuron Disease ◽

Peripheral Nerves ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Multifocal Motor Neuropathy ◽

Evidence Based ◽

Motor Neuropathy ◽

Treatment Regimens ◽

Peripheral Nerve Involvement ◽

Inflammatory Demyelinating Polyneuropathy

This chapter discusses the clinical features and evidence-based drug treatment regimens of polyneuropathies (Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy, paraproteinaemic neuropathies, and vasculitic neuropathies), mononeuropathies (Bell’s palsy), systemic conditions with peripheral nerve involvement (Sjögren’s and sarcoidosis), and motor neuron disease (MND).

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Small Fibre Involvement in Multifocal Motor Neuropathy Explored with Sudoscan: A Single-Centre Experience

Diagnostics ◽

10.3390/diagnostics10100755 ◽

2020 ◽

Vol 10 (10) ◽

pp. 755

Author(s):

Marco Luigetti ◽

Silvia Giovannini ◽

Angela Romano ◽

Giulia Bisogni ◽

Francesco Barbato ◽

...

Keyword(s):

Sensory Nerve ◽

Multifocal Motor Neuropathy ◽

Motor Neuropathy ◽

Disease Course ◽

Small Fibre ◽

Single Centre Experience ◽

Inflammatory Neuropathy ◽

Sensory Nerve Action ◽

Neurophysiological Data

Objective: Multifocal motor neuropathy (MMN) is a rare inflammatory neuropathy, clinically characterized by exclusive motor involvement. We wished to evaluate the possible presence of sensory dysfunction, including the evaluation of small fibres, after a long-term disease course. Patients and methods: seven MMN patients, regularly followed in our Neurology Department, underwent clinical evaluation, neurophysiological examination by nerve conduction studies (NCSs), and Sudoscan. We compared neurophysiological data with a group of patients with other disorders of the peripheral nervous system. Results: NCSs showed a reduction of sensory nerve action potential amplitude in 2/7 MMN patients. Sudoscan showed borderline electrochemical skin conductance (ESC) values in 3/7 MMN patients (two of them with abnormal sensory NCSs). Conclusions: Our results confirm that sensory involvement may be found in some MMN after a long-term disease course, and it could also involve the small fibres.

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