Longitudinal study on nerve ultrasound and corneal confocal microscopy in NF155 paranodopathy

Athanasopoulos, Diamantis; Motte, Jeremias; Fisse, Anna Lena; Grueter, Thomas; Trampe, Nadine; Sturm, Dietrich; Tegenthoff, Martin; Sgodzai, Melissa; Klimas, Rafael; Querol, Luís; Gold, Ralf; Pitarokoili, Kalliopi

doi:10.1002/acn3.51061

Cited by 16 publications

(16 citation statements)

References 33 publications

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“…This good response to RTX is in accordance with the literature. 6 , 8 , 35 – 40 Therefore, RTX should be considered for patients with autoantibody related disease.…”

Section: Discussionmentioning

confidence: 99%

Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study

Motte

Fisse

Köse

et al. 2021

Ther Adv Neurol Disord

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Background: Up to 20% of patients with chronic immune-mediated sensorimotor neuropathies (CIN) do not respond adequately to first-line therapies. However, studies on further treatment are scarce. Methods: We analyzed retrospectively 200 CIN patients regarding disease characteristics and response to therapy with cyclophosphamide (CYP), rituximab (RTX), and bortezomib (BTZ). Treatment response was defined as improvement or stabilization of inflammatory neuropathy cause and treatment overall disability score (INCAT-ODSS). Results: A total of 48 of 181 patients (26.5%) received therapy with CYP, RTX, or BTZ. The most frequently and first used therapy was CYP (69%). More than 40% of patients needed a second or third treatment. Overall, 71 treatments were applied in 48 patients. The combination of up to all three treatments enhanced the response-rate to 90%. Treatment within 24 months after initial diagnosis resulted in significantly higher response rate than late treatment (79% versus 50 %, p = 0.04, χ2-test, n = 46) and in lower disability in long-term follow up (INCAT-ODSS 3.8 versus 5.8, p = 0.02, t-test, n = 48). Patients with Lewis-Sumner syndrome ( n = 9) and autoantibody mediated neuropathies ( n = 13) had excellent response rates after treatment with RTX (90–100%). In contrast, typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) showed a response rate of 64% in CYP, 64% in RTX, and 75% in BTZ. Conclusion: Treatment with CYP, RTX, or BTZ was effective in this cohort of CIN refractory to first-line treatment. Our data increase evidence for an early use of these therapies. High efficacy of RTX in Lewis-Sumner syndrome in contrast to typical CIDP suggests a distinct pathophysiology.

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“…This good response to RTX is in accordance with the literature. 6 , 8 , 35 – 40 Therefore, RTX should be considered for patients with autoantibody related disease.…”

Section: Discussionmentioning

confidence: 99%

Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study

Motte

Fisse

Köse

et al. 2021

Ther Adv Neurol Disord

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“…Only few data are available concerning imaging of paranodopathies, but the pattern seems to be similar to that seen in typical CIDP, probably more often affecting cranial nerves [ 78 , 79 ].…”

Section: Immune-mediated Neuropathiesmentioning

confidence: 98%

Nerve Ultrasound as Helpful Tool in Polyneuropathies

et al. 2021

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Background: Polyneuropathies (PNP) are a broad field of diseases affecting millions of people. While the symptoms presented are mostly similar, underlying causes are abundant. Thus, early identification of treatable causes is often difficult. Besides clinical data and basic laboratory findings, nerve conduction studies are crucial for etiological classification, yet limited. Besides Magnetic Resonance Imaging (MRI), high-resolution nerve ultrasound (HRUS) has become a noninvasive, fast, economic and available tool to help distinguish different types of nerve alterations in neuropathies. Methods: We aim to describe typical ultrasound findings in PNP and patterns of morphological changes in hereditary, immune-mediated, diabetic, metabolic and neurodegenerative PNP. Literature research was performed in PubMed using the terms ‘nerve ultrasound’, neuromuscular ultrasound, high-resolution nerve ultrasound, peripheral nerves, nerve enlargement, demyelinating, hereditary, polyneuropathies, hypertrophy’. Results: Plenty of studies over the past 20 years investigated the value of nerve ultrasound in different neuropathies. Next to nerve enlargement, patterns of nerve enlargement, echointensity, vascularization and elastography have been evaluated for diagnostic terms. Furthermore, different scores have been developed to distinguish different etiologies of PNP. Conclusions: Where morphological alterations of the nerves reflect underlying pathologies, early nerve ultrasound might enable a timely start of available treatment and also facilitate follow up of therapy success.

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“…CCM was also useful to detect CIDP in those individuals with and without coexisting diabetes, indicating that the increase in dendritic cells is driven by peripheral nerve inflammation. Indeed, recent longitudinal studies have shown that increased dendritic cell infiltration may predict worsening outcomes in CIDP [ 24 ] and infiltrates may be reduced in response to immune therapy [ 25 ].…”

Section: Discussionmentioning

confidence: 99%

“…In a prospective study of 17 individuals with CIDP who were followed over 18 months, the presence of > 30 cells/mm 2 at baseline identified clinical progression with a sensitivity and specificity of 100% [ 24 ]. Moreover, in one person with anti-neurofascin-155 neuropathy, treatment with rituximab was associated with a reduction in the serum antibody titer with a clinical and electrophysiological improvement and reduction of corneal inflammatory cell infiltrates [ 25 ].…”

Section: Introductionmentioning

confidence: 99%

Corneal confocal microscopy differentiates inflammatory from diabetic neuropathy

Fleischer

Lee

Erdlenbruch

et al. 2021

J Neuroinflammation

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Background Immune-mediated neuropathies, such as chronic inflammatory demyelinating polyneuropathy (CIDP) are treatable neuropathies. Among individuals with diabetic neuropathy, it remains a challenge to identify those individuals who develop CIDP. Corneal confocal microscopy (CCM) has been shown to detect corneal nerve fiber loss and cellular infiltrates in the sub-basal layer of the cornea. The objective of the study was to determine whether CCM can distinguish diabetic neuropathy from CIDP and whether CCM can detect CIDP in persons with coexisting diabetes. Methods In this multicenter, case-control study, participants with CIDP (n = 55) with (n = 10) and without (n = 45) diabetes; participants with diabetes (n = 58) with (n = 28) and without (n = 30) diabetic neuropathy, and healthy controls (n = 58) underwent CCM. Corneal nerve fiber density (CNFD), corneal nerve fiber length (CNFL), corneal nerve branch density (CNBD), and dendritic and non-dendritic cell density, with or without nerve fiber contact were quantified. Results Dendritic cell density in proximity to corneal nerve fibers was significantly higher in participants with CIDP with and without diabetes compared to participants with diabetic neuropathy and controls. CNFD, CNFL, and CNBD were equally reduced in participants with CIDP, diabetic neuropathy, and CIDP with diabetes. Conclusions An increase in dendritic cell density identifies persons with CIDP. CCM may, therefore, be useful to differentiate inflammatory from non-inflammatory diabetic neuropathy.

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Longitudinal study on nerve ultrasound and corneal confocal microscopy in NF155 paranodopathy

Cited by 16 publications

References 33 publications

Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study

Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study

Nerve Ultrasound as Helpful Tool in Polyneuropathies

Corneal confocal microscopy differentiates inflammatory from diabetic neuropathy

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