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

Diagnostics ◽  
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.

scholarly journals Carpal tunnel syndrome: long-term nerve conduction studies in 261 hands

2009 ◽  
Vol 67 (1) ◽  
pp. 69-73 ◽  
Author(s):  
Dante Guilherme Velasco Hardoim ◽  
Guilherme Bueno de Oliveira ◽  
João Aris Kouyoumdjian
Keyword(s):  
Carpal Tunnel Syndrome ◽  
Carpal Tunnel ◽  
Nerve Conduction ◽  
Sensory Nerve ◽  
Nerve Conduction Studies ◽  
Sensory Nerve Action ◽  
Tunnel Syndrome ◽  
The Mean ◽  
Compound Action

OBJECTIVE: To compare a long-term carpal tunnel syndrome (CTS) on nerve conduction studies (NCS) in hands treated non-surgically. METHOD: We retrospectively selected 261 symptomatic CTS hands (166 patients), all of them confirmed by NCS. In all cases, at least 2 NCS were performed in an interval greater than 12 months. Cases with associated polyneuropathy were excluded. NCS parameters for CTS electrodiagnosis included a sensory conduction velocity (SCV) <46.6 m/s (wrist to index finger, 14 cm) and distal motor latency (DML) >4.25 ms (wrist to APB, 8 cm). RESULTS: 92.8% were women; mean age was 49 years (20-76); the mean interval between NCS was 47 months (12-150). In the first exam, the median sensory nerve action potential (SNAP) and the compound action muscular potential were absent in 9.8% and 1.9%, respectively. In the second/last exam, SCV worsened in 54.2%, remained unchanged in 11.6% and improved in 34.2%. SNAP amplitude worsened in 57.7%, remained unchanged in 13.1% and improved in 29.2%. DML worsened in 52.9%, remained unchanged in 7.6% and improved in 39.5%. Overall, NCS parameters worsened in 54.9%, improved in 34.3% and remained unchanged in 10.8%. CONCLUSION: Long-term changing in NCS of CTS hands apparently were not related to clinical symptomatology and could lead to some difficulty in clinical correlation and prognosis. Aging, male gender and absent SNAP were more related to NCS worsening, regardless the mean interval time between the NCS.

Sensory nerve pathology in multifocal motor neuropathy

1996 ◽  
Vol 39 (3) ◽  
pp. 319-325 ◽  
Author(s):  
Andrea M. Corse ◽  
Vinay Chaudhry ◽  
Tom O. Crawford ◽  
David R. Cornblath ◽  
Ralph W. Kuncl ◽  
...  
Keyword(s):  
Sensory Nerve ◽  
Multifocal Motor Neuropathy ◽  
Motor Neuropathy

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.

W1.2 Multifocal motor neuropathy. Clinical and electrophysiological long term evolution in a group of patients

2011 ◽  
Vol 122 ◽  
pp. S6-S7
Author(s):  
N. Raguer ◽  
I. Serrano ◽  
B. Garcia ◽  
M. Gratacos ◽  
J.L. Seoane ◽  
...  
Keyword(s):  
Multifocal Motor Neuropathy ◽  
Long Term Evolution ◽  
Motor Neuropathy ◽  
Term Evolution

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.

The Long-term Treatment of Multifocal Motor Neuropathy with Intravenous Immunoglobulin

2012 ◽  
Vol 7 (2) ◽  
pp. 128 ◽  
Author(s):  
Leonard H van den Berg ◽  
Keyword(s):  
Muscle Strength ◽  
Intravenous Immunoglobulin ◽  
Initial Response ◽  
Multifocal Motor Neuropathy ◽  
Term Treatment ◽  
Response To Treatment ◽  
Motor Neuropathy ◽  
Double Blind ◽  
Long Term Treatment

Multifocal motor neuropathy (MMN) is a rare, purely motor neuropathy. It is a progressive disorder, most patients eventually developing severe fatigue and weakness in the arm muscles that severely impair daily functioning and quality of life. Unlike other motor neuropathies such as motor neurone disease, MMN is treatable with regular infusions of intravenous immunoglobulin (IVIg). Four double-blind, randomised, placebo-controlled studies have shown that in the short term, IVIg significantly improves muscle strength and disability in more than 70 % of patients. The 11 observational studies reviewed in this article confirm that long-term maintenance treatment with IVIg maintains clinical improvement compared to pre-treatment baseline in most patients. Infusions are generally well tolerated, but regular monitoring and re-evaluation of the IVIg maintenance regimen is essential, as most patients need progressive increases in dosage or reduced intervals between infusions to maintain their response to treatment. In the absence of accepted predictive markers, maintenance IVIg should be individualised, based on each patient’s initial response, disability and the interval between the first infusion and decline in muscle strength.

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