Nerve ultrasound in the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis with predominant lower motor neuron disease (ALS/LMND)

Loewenbrück, Kai F.; Liesenberg, Julia; Dittrich, Markus; Schäfer, Jochen; Patzner, Beate; B, Trausch; Machetanz, J.; Hermann, Andreas; Storch, Alexander

doi:10.1007/s00415-015-7927-9

Cited by 55 publications

(60 citation statements)

References 22 publications

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“…This finding was confirmed by a recent report employing high resolution sonography in MMN patients (3,15,16), but its utility for differential diagnosis, especially in MMN patients without solid diagnostic evidence (e.g., CB and/or anti-ganglioside antibodies), had been little explored. We have thus demonstrated that, in patients with otherwise typical MMN but no overt CB, sonography can provide evidence of demyelination.…”

Section: Discussionsupporting

confidence: 68%

Cervical root sonography to differentiate multifocal motor neuropathy from ALS

et al. 2016

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INTRODUCTIONMultifocal motor neuropathy (MMN) is a demyelinating neuropathy characterized by predominant involvement of motor nerves. MMN can mimic ALS and other lower motor neuron diseases, but, as MMN is treatable by intravenous immune globulin (IVIg) and other immunomodulating therapies (1), its differential diagnosis is important. The diagnosis of MMN is particularly difficult when its diagnostic hallmarks, conduction block (CB) and anti-ganglioside antibody are not demonstrable. In a recent study, CB was identified in only 60% of MMN patients (2). Sonography of the peripheral nerves was recently reported to detect signs of widespread demyelination including in proximal nerve segments of MMN patients with CB (3). Thus, the aim of the present study was to assess whether sonography of the nerve roots can identify nerve hypertrophy as a suggestive evidence of demyelination in MMN patients without CB and further, to help differentiate MMN from ALS. ALS was chosen for comparison because MMN is still one of the most common masqueraders of ALS when upper motor neuron sign is not obvious. SUBJECTS AND METHODS SubjectsRecordings were made from nine patients with MMN (all men), 22 with ALS (15 men), and from 17 normal controls (11 men) in Tokushima University Hospital, Japan. All the patients with MMN and ALS were ambulatory. The inclusion criteria for the patients with MMN were the following : (A) progressive muscle atrophy and weakness involving only a few peripheral nerves or roots, without sensory involvement, (B) lack of upper motor neuron signs, pathological reflexes, or bulbar symptoms/signs, (C) the lack of radiological abnormality of the cervical spine in an MRI scan, (D) lack of CB according to published electrodiagnostic criteria (4), and (E) most importantly, the presence of clinical improvement of the affected limb with IVIg therapy (2 g/kg given over 5 consecutive days), defined as the improvement of the pre -versus post-therapeutic modified MRC score (1 -5 scale including 4 -and 4 + grades) equal to or greater than 2 in at least two of the affected muscles (5, 6). The mean duration of our clinical follow up of the MMN patients was 42.3 months (range : 18 -107 months) and all the patients remained ambulatory. The mean number of patients undergoing IVIg treatment was 9.9 (range : 4 -17) with reproducible post-therapeutic improvement.Inclusion criterion for the ALS group was to meet either the clinically definite, or clinically probable (with or without laboratory support) revised El Escorial ALS criteria. Of note, our ALS patients had predominantly lower motor involvement at the time of the sonographic assessment, thus its clinical distinction from MMN was not straightforward. The control group was made up of individuals with no neurological symptoms or signs. Either MRI or plain four-angle x-ray of the cervical spine was performed in the control subjects to confirm the lack of significant degenerative spine disease. All the patients and control subjects gave informed consent to participate in the study. ...

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Section: Discussionsupporting

confidence: 68%

Cervical root sonography to differentiate multifocal motor neuropathy from ALS

et al. 2016

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“…Based on previous results, we expected to find a relationship between PGRN and CSA with the demographic data. As, indeed, CSF PGRN / Q alb , serum PGRN and CSA values were related to age, sex, height, and weight (for details, see the Results section), standardized regression residuals of those variables after regression on baseline age, sex, height, and baseline weight were used for bivariate correlations hypothesized to be found between CSF PGRN / Q alb , serum PGRN and CSA.…”

Section: Methodsmentioning

confidence: 98%

“…High‐resolution ultrasound is a useful tool to quantify structural nerve pathology and thus has a role in the diagnostic workup of peripheral nerve disorders, such as hereditary and immune‐mediated neuropathies . Recently, nerve sonography has been applied to amyotrophic lateral sclerosis (ALS) demonstrating evidence of both nerve root and distal peripheral nerve atrophy; this offers potential for ultrasound‐based differentiation of ALS and ALS‐mimicking disorders characterized by enlarged nerves, particularly multifocal motor neuropathy . Thus far, sonographic nerve measures, however, have failed to show robust associations with clinical data and other biomarkers in ALS, such as disease duration, revised ALS Functional Rating Scale (ALSFRS‐R), disease progression rate, and electrophysiological data .…”

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confidence: 99%

Peripheral nerve atrophy together with higher cerebrospinal fluid progranulin indicate axonal damage in amyotrophic lateral sclerosis

et al. 2017

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“…By contrast, in MMN, ultrasonography can detect enlargement of nerves at sites that are not clinically involved 94 , although the enlargement in cross-sectional area has a weak correlation with electrophysiological findings 95 . Nevertheless, ultra sonography might help to differentiate MMN from lower motor neuron disease 96,97 , as enlarged nerves or roots are more common in MMN.…”

Section: Notalgiamentioning

confidence: 99%

New technologies for the assessment of neuropathies

et al. 2017

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Peripheral neuropathies are among the most common neurological disorders 1 . In routine clinical practice, diagnosis of peripheral neuropathies is typically based on clinical assessment, nerve conduction studies and needle electromyography, which can determine whether symptoms are caused by primary axonal damage or demyelinating damage. This distinction is often relevant to therapeutic choices and to prognosis, as axonal damage typically reflects an inflammatory (for example, vasculitic) or degenerative (for example, hereditary or idiopathic) process that correlates with disability, whereas demyelinating damage can reflect a hereditary neuro pathy, but more often reflects an acquired immunemediated neuropathy. Besides metabolic, inflammatory or primary degenerative neuropathies, peripheral nerves can also be damaged by compression from adjacent tissue structures, such as tendons, cysts or haematomas. In this case, proper identification of the site, nature and degree of the lesion might be challenging, but is relevant to treatment and prognosis. Moreover, some patients present with symptoms that suggest damage to small nerve fibres, which nerve conduction studies cannot be used to detect.In the past 15 years, several techniques have become available to investigate focal and diffuse peripheral neuropathies, and their reliability has widened the spectrum of diagnostic tools for clinicians and improved assessment of neuropathies. In this Review, we provide an overview of these new and emerging technologies. We first discuss peripheral nerve imaging with MRI and ultrasonography, which can provide information about the precise localization of neuro pathies and the pathological processes involved in large-fibre neuropathies, and can complement clinical and electrophysiological evaluation. We then consider techniques that can provide diagnostic information when the pathological process is restricted to small nerve fibres and is consequently not detectable with nerve conduction studies or neuroimaging. These include skin biopsy, which is established in clinical practice, and the emerging techniques of corneal confocal microscopy, laser-evoked potentials and heat-related and pain-related evoked potentials, and microneurography.Nerve imaging technology Advances in imaging techniques have made it possible to gain great insight into nerve pathology with magnetic resonance and ultrasound. Each approach offers distinct advantages, and can provide different information about damage to large myelinated fibres. Abstract | Technical advances are rapidly changing the clinical and instrumental approach to peripheral nerve diseases. Magnetic resonance neurography, diffusion tensor imaging and nerve ultrasonography are increasingly entering the diagnostic workup of peripheral neuropathies as tools that complement neurophysiology and enable investigation of proximal structures, such as plexuses and roots. Progress in the design of magnetic resonance scanners and sequences, and the development of high-frequency ultrasound probes mean th...

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Nerve ultrasound in the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis with predominant lower motor neuron disease (ALS/LMND)

Cited by 55 publications

References 22 publications

Cervical root sonography to differentiate multifocal motor neuropathy from ALS

Cervical root sonography to differentiate multifocal motor neuropathy from ALS

Peripheral nerve atrophy together with higher cerebrospinal fluid progranulin indicate axonal damage in amyotrophic lateral sclerosis

New technologies for the assessment of neuropathies

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