Nerve ultrasound for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy

Grimm, Alexander; Décard, Bernhard F.; Αθανασοπούλου, Ιωάννα; Schweikert, Kathi; Sinnreich, Michael; Axer, Hubertus

doi:10.1007/s00415-015-7648-0

Cited by 95 publications

(130 citation statements)

References 43 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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“…MRI [24, 25], and more frequently, peripheral nerve ultrasound [26, 27] is used to ascertain the diagnosis of immune-mediated neuropathies. In our series, however, such imaging studies have not been applied in a systematic matter and no conclusions regarding imaging can be drawn.…”

Section: Discussionmentioning

confidence: 99%

Multifocal motor neuropathy in Austria: a nationwide survey of clinical features and response to treatment

Löscher

Oberreiter

Erdler³

et al. 2018

J Neurol

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Background and objectivesMultifocal motor neuropathy (MMN) is a rare neuropathy and detailed descriptions of larger patient cohorts are scarce. The objective of this study was to evaluate epidemiological, clinical, and laboratory features of MMN patients and their response to treatment in Austria and to compare these data with those from the literature.MethodsAnonymized demographic and clinical data about MMN patients until 31.12.2017 were collected from registered Austrian neurologists. Exploratory statistics on clinical and laboratory features as well as treatment regimens and responses were performed.Results57 Patients with MMN were identified, resulting in a prevalence of 0.65/100.000. Mean age of onset was 44.1 ± 13.1 years, the diagnostic delay 5.5 ± 8.4 years. In 77% of patients, symptom onset was in the upper limbs, and in 92%, it occurred in distal muscles. Proximal onset was never observed in the lower limbs. At the final follow-up, the majority of patients had atrophy (88%) in affected regions. Definite motor conduction blocks (CB) were found in 54 patients. Anti-GM1-IgM antibodies were present in 43%. Treatment with intravenous immunoglobulins improved muscle strength and INCAT score initially, but at last follow-up, both scores deteriorated to values before treatment.DiscussionThe findings of the present study corroborate the previous findings in MMN. Onset typically occurs in the upper limbs and mostly distal, CBs are found in the majority of cases, while anti-GM1-IgM antibodies are detected in only approximately 40%. Our study underlines that the initial good response to treatment fades over time.

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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 for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy

Cited by 95 publications

References 43 publications

Cervical root sonography to differentiate multifocal motor neuropathy from ALS

Cervical root sonography to differentiate multifocal motor neuropathy from ALS

Multifocal motor neuropathy in Austria: a nationwide survey of clinical features and response to treatment

New technologies for the assessment of neuropathies

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