Nerve ultrasound for distinguishing inflammatory neuropathy from amyotrophic lateral sclerosis: Not black and white

Leeuw, Carola de; Wijntjes, Juerd; Lassche, Saskia; Alfen, Nens van

doi:10.1002/mus.26853

Cited by 4 publications

(3 citation statements)

References 14 publications

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“…In this independent cohort, we replicated our previous results and those of others reporting an ALS subgroup with larger CSA and, probably, increased nerve microvascular flow in the face of blood–nerve barrier leakage, supposedly pointing towards the existence of an inflammatory disease subtype [ 6 , 8 , 34 ]. Of clinical relevance is the hint that this subtype might display sustained motor function, and—most likely—better long-term outcomes [ 8 ].…”

Section: Discussionsupporting

confidence: 90%

Peripheral Nerve Ultrasound for the Differentiation between ALS, Inflammatory, and Hereditary Polyneuropathies

et al. 2023

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Background and Objectives: Ultrasound (US) is a non-invasive tool for the in vivo detection of peripheral nerve alterations. Materials and Methods: In this study, we applied nerve US to assist the discrimination between the spectrum of amyotrophic lateral sclerosis (ALS, n = 11), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP, n = 5), and genetically confirmed Charcot–Marie–Tooth disease (CMT, n = 5). All participants and n = 15 controls without neurological diseases underwent high-resolution US of the bilateral tibial nerve. The nerve cross-sectional area (CSA) and nerve microvascular blood flow were compared between the groups and related to cerebrospinal fluid (CSF) measures, clinical symptoms, and nerve conduction studies. The analyses are part of a larger multimodal study on the comparison between US and 7 Tesla (7T) magnetic resonance neurography (MRN). Results: The patients and controls were matched with respect to their demographical data. CMT had the longest disease duration, followed by CIDP and ALS. CSA was related to age, weight, and disease duration. CSA was larger in CMT and CIDP compared to ALS and controls. The blood flow was greatest in CIDP, and higher than in CMT, ALS, and controls. In ALS, greater CSA was correlated with greater CSF total protein and higher albumin quotient. The US measures did not correlate with clinical scores or nerve conduction studies in any of the subgroups. Conclusion: Our results point towards the feasibility of CSA and blood flow to discriminate between ALS, CIDP, and CMT, even in groups of small sample size. In ALS, larger CSA could indicate an inflammatory disease subtype characterized by reduced blood–nerve barrier integrity. Our upcoming analysis will focus on the additive value of 7T MRN in combination with US to disentangle the spectrum between more inflammatory or more degenerative disease variants among the disease groups.

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Section: Discussionsupporting

confidence: 90%

Peripheral Nerve Ultrasound for the Differentiation between ALS, Inflammatory, and Hereditary Polyneuropathies

et al. 2023

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“…Systematic HRUS studies reported mild (up to 1.4-fold) nerve enlargement in around 20% of all ALS patients as well [11,30]; in a single ALS case nerve area increase was even 1.8-fold [31]. ALS patients with some nerve enlargement also show a higher CSF albumin/serum albumin ratio indicative of a blood-nerve barrier breakdown and might include more male cases with longer disease duration and positive superoxide dismutase 1 mutation carrier status [30].…”

Section: Discussionmentioning

confidence: 98%

“…There is one case, where IVIG treatment led to the normalization of CSA nerve values [41], while another study reported fascicle area regression under IVIG therapy (but also in therapy naive MMN patients) [42]. By contrast, Leeuw et al [31] demonstrated a patient with multifocal nerve enlargement and initial suspicion of an immune-mediated neuropathy. After a clinical follow-up examination and generalized CSA decrease, diagnosis was changed to ALS.…”

Section: Discussionmentioning

confidence: 99%

Peripheral Nerve Imaging Aids in the Diagnosis of Immune-Mediated Neuropathies—A Case Series

et al. 2020

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Background: Diagnosis of immune-mediated neuropathies and their differentiation from amyotrophic lateral sclerosis (ALS) can be challenging, especially at early disease stages. Accurate diagnosis is, however, important due to the different prognosis and available treatment options. We present one patient with a left-sided dorsal flexor paresis and initial suspicion of ALS and another with multifocal sensory deficits. In both, peripheral nerve imaging was the key for diagnosis. Methods: We performed high-resolution nerve ultrasound (HRUS) and 7T or 3T magnetic resonance neurography (MRN). Results: In both patients, HRUS revealed mild to severe, segmental or inhomogeneous, nerve enlargement at multiple sites, as well as an area increase of isolated fascicles. MRN depicted T2 hyperintense nerves with additional contrast-enhancement. Discussion: Peripheral nerve imaging was compatible with the respective diagnosis of an immune-mediated neuropathy, i.e., multifocal motor neuropathy (MMN) in patient 1 and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) in patient 2. Peripheral nerve imaging, especially HRUS, should play an important role in the diagnostic work-up for immune-mediated neuropathies and their differentiation from ALS.

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Peripheral Nerve Ultrasound for the Differentiation between ALS, Inflammatory and Hereditary Polyneuropathies

Hildebrand¹,

Schreiber²,

Weber³

et al. 2023

Preprint

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Background and Objectives: Ultrasound (US) is a non-invasive tool for the in-vivo detection of peripheral nerve alterations. Materials and Methods: In this study we applied nerve US to assist the discrimination between the spectrum of Amyotrophic Lateral Sclerosis (ALS, n=11), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP, n=5) and genetically confirmed Charcot-Marie-Tooth disease (CMT, n=5). All participants and n=15 controls without neurological diseases underwent high-resolution US of the bilateral tibial nerve. Nerve cross-sectional area (CSA) and nerve microvascular blood flow were compared between the groups and related to cerebrospinal fluid (CSF) measures, clinical symptoms and nerve conduction studies. Analyses are part of a larger multimodal study on the comparison between US and 7 Tesla (T) magnetic resonance neurography (MRN). Results: Patients and controls were matched with respect to their demographical data. CMT had the longest disease duration, followed by CIDP and ALS. CSA was related to age, weight and disease duration. CSA was larger in CMT and CIDP compared to ALS and controls. Blood flow was greatest in CIDP, and higher than in CMT, ALS and controls. In ALS, greater CSA was correlated with greater CSF total protein and higher albumin quotient. US measures did not correlate with clinical scores or nerve conduction studies in any of the subgroups. Conclusion: Our results point towards the feasibility of CSA and blood flow to discriminate between ALS, CIDP and CMT, even in small sample sized groups. In ALS, larger CSA could indicate an inflammatory disease subtype characterized by reduced blood-nerve barrier integrity. Our upcoming analysis will focus on the additive value of 7T MRN in combination with US to disentangle the spectrum between more inflammatory or more degenerative disease variants along the disease groups.

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Nerve ultrasound for distinguishing inflammatory neuropathy from amyotrophic lateral sclerosis: Not black and white

Cited by 4 publications

References 14 publications

Peripheral Nerve Ultrasound for the Differentiation between ALS, Inflammatory, and Hereditary Polyneuropathies

Peripheral Nerve Ultrasound for the Differentiation between ALS, Inflammatory, and Hereditary Polyneuropathies

Peripheral Nerve Imaging Aids in the Diagnosis of Immune-Mediated Neuropathies—A Case Series

Peripheral Nerve Ultrasound for the Differentiation between ALS, Inflammatory and Hereditary Polyneuropathies

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