Plexus MRI helps distinguish the immune-mediated neuropathies MADSAM and MMN

Beecher, Grayson; Howe, Benjamin M.; Shelly, Shahar; Nathan, P.; Mauermann, Michelle L.; Taylor, Bruce; Spinner, Robert J.; Tracy, Jennifer A.; Dyck, Peter J.; Klein, Christopher J.

doi:10.1016/j.jneuroim.2022.577953

Cited by 4 publications

(3 citation statements)

References 36 publications

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Section: Discussioncontrasting

confidence: 99%

“…Whilst the utilization of MRI in our cohort was limited, probably due to availability constraints, it still contributed to refining diagnoses in a subset of patients. Our results do not confirm the observations made by Beecher and coauthors who noted the absence of hypertrophy in the brachial plexus amongst MMN patients, with its occurrence solely in patients with Lewis−Sumner syndrome [21]. Notably, the average disease duration in the seven patients with a positive MRI (mean 5 years, range 1–12) still leaves the possibility of progression to Lewis−Sumner syndrome, based on previous observations by other authors [20].…”

Section: Discussioncontrasting

confidence: 99%

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Assessment of diagnostic criteria for multifocal motor neuropathy in patients included in the Italian database

Doneddu,

Gentile,

Cocito

et al. 2024

Euro J of Neurology

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Background and purposeThis study aimed to assess the diagnostic criteria, ancillary investigations and treatment response using real‐life data in multifocal motor neuropathy (MMN) patients.MethodsClinical and laboratory data were collected from 110 patients enrolled in the Italian MMN database through a structured questionnaire. Twenty‐six patients were excluded due to the unavailability of nerve conduction studies or the presence of clinical signs and symptoms and electrodiagnostic abnormalities inconsistent with the MMN diagnosis. Analyses were conducted on 73 patients with a confirmed MMN diagnosis and 11 patients who did not meet the diagnostic criteria.ResultsThe European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria were variably applied. AUTHOR:When applying the American Association of Electrodiagnostic Medicine criteria, an additional 17% of patients fulfilled the criteria for probable/definite diagnosis whilst a further 9.5% missed the diagnosis. In 17% of the patients only compound muscle action potential amplitude, but not area, was measured and subsequently recorded in the database by the treating physician. Additional investigations, including anti‐GM1 immunoglobulin M antibodies, cerebrospinal fluid analysis, nerve ultrasound and magnetic resonance imaging, supported the diagnosis in 46%–83% of the patients. Anti‐GM1 immunoglobulin M antibodies and nerve ultrasound demonstrated the highest sensitivity. Additional tests were frequently performed outside the EFNS/PNS guideline recommendations.ConclusionsThis study provides insights into the real‐world diagnostic and management strategies for MMN, highlighting the challenges in applying diagnostic criteria.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussioncontrasting

confidence: 99%

Assessment of diagnostic criteria for multifocal motor neuropathy in patients included in the Italian database

Doneddu,

Gentile,

Cocito

et al. 2024

Euro J of Neurology

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“…Another study measuring DTI metrics in cervical roots did not find any significant changes in MMN compared to healthy controls [ 20 ], suggesting that peripheral nerves may be more representative regions for MMN affection than plexus fibers. This is also in line with a study of Beecher et al, who reported no hypertrophies in the brachial and lumbosacral plexus of MMN patients [ 36 ].…”

Section: Discussionsupporting

confidence: 93%

Quantitative MR Neurography in Multifocal Motor Neuropathy and Amyotrophic Lateral Sclerosis

Foesleitner

Knop²,

Lindenau³

et al. 2023

Diagnostics

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Background: The aim of this study was to assess the phenotype of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) in quantitative MR neurography. Methods: In this prospective study, 22 patients with ALS, 8 patients with MMN, and 10 healthy volunteers were examined with 3T MR neurography, using a high-resolution fat-saturated T2-weighted sequence, diffusion-tensor imaging (DTI), and a multi-echo T2-relaxometry sequence. The quantitative biomarkers fractional anisotropy (FA), radial and axial diffusivity (RD, AD), mean diffusivity (MD), cross-sectional area (CSA), T2-relaxation time, and proton spin density (PSD) were measured in the tibial nerve at the thigh and calf, and in the median, radial, and ulnar nerves at the mid-upper arm. Results: MMN showed a characteristic imaging pattern of decreased FA (p = 0.018), increased RD (p = 0.014), increased CSA (p < 0.001), increased T2-relaxation time (p < 0.001), and increased PSD (p = 0.025) in the upper arm nerves compared to ALS and controls. ALS patients did not differ from controls in any imaging marker, nor were there any group differences in the tibial nerve (p > 0.05). Conclusions: MMN shows a characteristic pattern of quantitative DTI and T2-relaxometry parameters in the upper-arm nerves, primarily indicating demyelination. Peripheral nerve changes in ALS seem to be below the detection level of current state-of-the-art quantitative MR neurography.

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