WED 223 Predicting disease activity in inflammatory neuropathies

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.

scholarly journals 086 Clinical, investigational and treatment factors do not determine prognosis of patients with inflammatory neuropathies

2019 ◽  
Vol 90 (e7) ◽  
pp. A27.3-A28
Author(s):  
Mahima Kapoor ◽  
Aisling Carr ◽  
Michael P Lunn ◽  
Mary M Reilly
Keyword(s):  
Upper Limb ◽  
Remission Rate ◽  
Ivig Treatment ◽  
Retrospective Data ◽  
Significant Difference ◽  
Recent Treatment ◽  
Successful Group ◽  
Inflammatory Neuropathies ◽  
Measure Disease Activity

IntroductionIdentifying patients who need long-term immunoglobulin (IVIg) treatment in patient with inflammatory neuropathies is essential as recent treatment trials show a remission rate of up to 40%.AimsCompare retrospective data on clinical, investigational and treatment factors in patients who have ceased IVIg with patients who have failed a cessation trial.Methods15 patients who successfully suspended IVIg infusions were compared with 15 in whom decreasing or stopping IVIg 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. 9.8mV p=0.007). There was no difference in 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.ConclusionThere is a need for objective biomarker to measure disease activity because other than one neurophysiology marker, other factors did not help predict a successful cessation trial of IVIg.

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

2019 ◽  
Vol 14 (1) ◽  
pp. 3-13 ◽  
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.

scholarly journals Nerve ultrasound improves detection of treatment-responsive chronic inflammatory neuropathies

Neurology ◽  
2020 ◽  
Vol 94 (14) ◽  
pp. e1470-e1479 ◽  
Author(s):  
Ingrid J.T. Herraets ◽  
H. Stephan Goedee ◽  
Johan A. Telleman ◽  
Ruben P.A. van Eijk ◽  
J. Thies van Asseldonk ◽  
...  
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Multifocal Motor Neuropathy ◽  
Clinical Suspicion ◽  
Added Value ◽  
Response To Treatment ◽  
European Federation ◽  
Motor Neuropathy ◽  
Nerve Ultrasound ◽  
Inflammatory Neuropathy ◽  
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.

Intravenous immunoglobulin treatment in multifocal motor neuropathy and other chronic immune-mediated neuropathies

1997 ◽  
Vol 3 (2) ◽  
pp. 93-97 ◽  
Author(s):  
G. Comi ◽  
R. Nemni ◽  
S. Amadio ◽  
G. Galardi ◽  
L. Leocani
Keyword(s):  
Plasma Exchange ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Case Series ◽  
Multifocal Motor Neuropathy ◽  
Initial Therapy ◽  
Motor Neuropathy ◽  
Short Term ◽  
Term Efficacy ◽  
Immune Mediated

This review deals with the use of intravenous IVIg immunoglobulines in the treatment of chronic immune-mediated neuropathies: multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy, neuropathies associated with monoclonal gammopathies. A particular attention is given to case series and trials which compare IVIg to other therapies, such as steroid treatment, immunosuppressors and plasma exchange. At present, clinical and instrumental data seem to indicate the short term efficacy of IVIg in multifocal motor neuropathies, especially as early treatment; further studies are need in order to prove its long term efficacy in this disease. Concerning chronic inflammatory demyelinating polyneuropathies, short term IVIg efficacy is comparable to that of plasma exchange and in the long term most patients need repeated treatments. Most patients respond to the initial therapy and the initial nonresponders usually improve with a second treatment modality.

Multifocal motor neuropathy: Long-term treatment with subcutaneous immunoglobulin

2021 ◽  
Vol 429 ◽  
pp. 117814
Author(s):  
Luca Gentile ◽  
Massimo Russo ◽  
Carmelo Rodolico ◽  
Ilenia Arimatea ◽  
Giuseppe Vita ◽  
...  
Keyword(s):  
Multifocal Motor Neuropathy ◽  
Term Treatment ◽  
Motor Neuropathy ◽  
Subcutaneous Immunoglobulin ◽  
Long Term Treatment

Pure Motor Onset and IgM-Gammopathy Occurrence in Multifocal Acquired Demyelinating Sensory and Motor Neuropathy

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012618
Author(s):  
Grayson Beecher ◽  
Shahar Shelly ◽  
P. James B. Dyck ◽  
Michelle L. Mauermann ◽  
Jennifer M Martinez-Thompson ◽  
...  
Keyword(s):  
Monoclonal Gammopathy ◽  
Motor Nerve ◽  
Multifocal Motor Neuropathy ◽  
Term Treatment ◽  
Motor Neuropathy ◽  
Point Reduction ◽  
Long Term Treatment ◽  
Motor Onset ◽  
Pure Motor

Objectives:To longitudinally investigate patients with multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, quantifying timing and location of sensory involvements in motor-onset patients, along with clinico-histopathological and electrophysiological findings to ascertain differences in patients with and without monoclonal gammopathy of uncertain significance (MGUS).Methods:Patients with MADSAM neuropathy seen at Mayo Clinic and tested for monoclonal gammopathy and ganglioside antibodies, were retrospectively reviewed (January 1st, 2007-December 31st, 2018).Results:Of 76 patients with MADSAM, 53% had pure motor, 16% pure sensory, 30% sensorimotor and 1% cranial nerve onsets. Motor-onset patients were initially diagnosed as multifocal motor neuropathy (MMN). MGUS occurred in 25% (89% IgM subtype), associating with ganglioside autoantibodies (p<0.001) and higher IgM titers (p<0.04). Median time to sensory involvements (confirmed by electrophysiology) in motor-onset patients was 18 months (range: 6-180). Compared to initial motor nerve involvements, subsequent sensory findings were within the same territory 35% (14/40), outside 20% (8/40), or both 45% (18/40). Brachial and lumbosacral plexus MRI was abnormal in 87% (34/39) and 84% (21/25), respectively, identifying hypertrophy and increased T2 signal predominantly in brachial plexus trunks (64%), divisions (69%), and cords (69%), and intrapelvic sciatic (64%) and femoral (44%) nerves. Proximal fascicular nerve biopsies (n=9) more frequently demonstrated onion-bulb pathology (p=0.001) and endoneurial inflammation (p=0.01) than distal biopsies (n=17). MRI and biopsy findings were similar amongst patient subgroups. Initial Inflammatory Neuropathy Cause and Treatment (INCAT) disability scores were higher in patients with MGUS relative to without (p=0.02). Long-term treatment responsiveness by INCAT score reduction ≥1 or motor Neuropathy Impairment Score (mNIS) >8 point reduction occurred in 75% (49/65) irrespective of MGUS or motor-onsets. Most required ongoing immunotherapy (86%). Patients with MGUS more commonly required dual-agent immunotherapy for stability (p=0.02).Discussion:Pure motor-onsets are the most common MADSAM presentation. Long-term follow-up, repeat electrophysiology and nerve pathology help distinguish motor-onset MADSAM from MMN. Better long-term immunotherapy responsiveness occurs in motor-onset MADSAM compared to MMN reports. Patients having MGUS commonly require dual immunotherapy.Classification of Evidence:This study provides Class II evidence that most clinical, electrophysiological, and histopathological findings were similar between patients with MADSAM with and without monoclonal gammopathy of unknown significance.

Disorders of peripheral nerves and motor neuron disease

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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