CNS Involvement in Chronic Inflammatory Demyelinating Polyneuropathy

Ingwersen, Jens; Graf, Jonas; Kluge, Julia; Weise, Margit; Dietrich, Michael; Lee, John-Ih; Harmel, Jens; Hartung, Hans‐Peter; Ruck, Tobias; Meuth, Sven G.; Albrecht, Philipp; Aktaş, Orhan; Ringelstein, Marius

doi:10.1212/nxi.0000000000001099

Cited by 8 publications

(10 citation statements)

References 46 publications

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“…APOSTEL reporting recommendations were applied for SD-OCT methodology and results 13 . The methods are well-established and have also been used and described elsewhere 14 – 16 . For macular volume scans, 61 vertical scans centered on the fovea (30° × 25°, high-speed scanning mode) were captured.…”

Section: Methodsmentioning

confidence: 99%

Real-world evidence of ocrelizumab-treated relapsing multiple sclerosis cohort shows changes in progression independent of relapse activity mirroring phase 3 trials

Ingwersen,

Masanneck,

Pawlitzki

et al. 2023

Sci Rep

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Ocrelizumab is a B cell-depleting drug widely used in relapsing–remitting multiple sclerosis (RRMS) and primary-progressive MS. In RRMS, it is becoming increasingly apparent that accumulation of disability not only manifests as relapse-associated worsening (RAW) but also as progression independent of relapse activity (PIRA) throughout the disease course. This study’s objective was to investigate the role of PIRA in RRMS patients treated with ocrelizumab. We performed a single-center, retrospective, cross-sectional study of clinical data acquired at a German tertiary multiple sclerosis referral center from 2018 to 2022. All patients with RRMS treated with ocrelizumab for at least six months and complete datasets were analyzed. Confirmed disability accumulation (CDA) was defined as a ≥ 12-week confirmed increase from the previous expanded disability status scale (EDSS) score of ≥ 1.0 if the previous EDSS was ≤ 5.5 or a ≥ 0.5-point increase if the previous EDSS was > 5.5. PIRA was defined as CDA without relapse since the last EDSS measurement and at least for the preceding 12 weeks. RAW was defined as CDA in an interval of EDSS measurements with ≥ 1 relapses. Cox proportional hazard models were used to analyze the probability of developing PIRA depending on various factors, including disease duration, previous disease-modifying treatments (DMTs), and optical coherence tomography-assessed retinal degeneration parameters. 97 patients were included in the analysis. Mean follow-up time was 29 months (range 6 to 51 months). 23.5% developed CDA under ocrelizumab therapy (n = 23). Of those, the majority developed PIRA (87.0% of CDA, n = 20) rather than RAW (13.0% of CDA, n = 3). An exploratory investigation using Cox proportional hazards ratios revealed two possible factors associated with an increased probability of developing PIRA: a shorter disease duration prior to ocrelizumab (p = 0.02) and a lower number of previous DMTs prior to ocrelizumab (p = 0.04). Our data show that in ocrelizumab-treated RRMS patients, the main driver of disability accumulation is PIRA rather than RAW. Furthermore, these real-world data show remarkable consistency with data from phase 3 randomized controlled trials of ocrelizumab in RRMS, which may increase confidence in translating results from tightly controlled RCTs into the real-world clinical setting.

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Section: Methodsmentioning

confidence: 99%

Real-world evidence of ocrelizumab-treated relapsing multiple sclerosis cohort shows changes in progression independent of relapse activity mirroring phase 3 trials

Ingwersen,

Masanneck,

Pawlitzki

et al. 2023

Sci Rep

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“…Visual evoked potential studies have revealed electrophysiologic evidence of subclinical optic nerve dysfunction in those with longstanding CIDP (2,4). A recent optical coherence tomography study found subtle retinal ganglion cell/inner plexiform layer neurodegeneration in patients with CIDP compared with healthy controls (18). The precise mechanism of optic nerve dysfunction in CIDP remains unclear.…”

Section: Discussionmentioning

confidence: 99%

Vision Loss as a Presenting Feature of Chronic Inflammatory Demyelinating Polyneuropathy: A Case Series

Kruszewski¹,

Tauqeer²,

Meer³

et al. 2022

Journal of Neuro-Ophthalmology

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Background:Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated, and clinically heterogeneous demyelinating disease affecting the nerve roots and peripheral nerves. We report a series of 4 patients who presented with early and progressive vision loss in the context of new-onset CIDP: 3 due to papilledema and 1 due to optic neuropathy without papilledema.Methods:This was a retrospective case series of 4 patients with vision loss as a presenting feature of CIDP evaluated at the Hospital of the University of Pennsylvania from January 2016 to August 2021. Demographic, clinical, diagnostic, and treatment data were collected via retrospective medical record review.Results:Case 1 was a 51-year-old man with 2 months of progressive bilateral papilledema associated with reduced visual acuity (count fingers at 1 foot in each eye) and severely constricted visual fields. Case 2 was a 36-year-old man with 4 months of worsening headaches, reduced visual acuity (count fingers at 1 foot in each eye), severely constricted visual fields, and papilledema. Case 3 was a 39-year-old man with papilledema causing progressive vision loss (20/80 in both eyes), headaches, and relapsing limb sensorimotor deficits. Case 4 was a 19-year-old man with 3 months of progressive bilateral visual decline (20/400 in the right eye, 20/600 in the left eye), central scotoma, and optic disc pallor consistent with optic neuropathy without papilledema. All 4 patients met clinical and electrodiagnostic criteria of CIDP. Cases 3 and 4 each tested positive for serum neurofascin-155 IgG4 antibodies. All patients were managed with immunomodulatory therapy. Cases 1 and 2 also each required surgical intervention with bilateral optic nerve sheath fenestration and cerebrospinal fluid (CSF) shunting procedures.Conclusion:Vision loss from optic neuropathy with or without papilledema has rarely been reported in CIDP, and typically has been described in the context of longstanding disease. Our cases highlight how CIDP can present with early vision loss that may be profound and challenging to manage if diagnosis is delayed. CIDP should be considered in any patient with new progressive vision loss when associated with peripheral sensorimotor symptoms and elevated CSF protein. The small subgroup of CIDP patients with neurofascin-155 antibodies may be at particular risk of optic nerve involvement.

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“…However, signs of central nervous system (CNS) affection, such as fatigue, 3 cognitive 4 and visual abnormalities, 5 , 6 were reported to accompany typical sensorimotor CIDP symptoms. In this regard, it is notable that optical coherence tomography showed reduced volumes of the retinal ganglion cell (RGC) layer in patients with CIDP.…”

Section: Introductionmentioning

confidence: 99%

Central involvement in peripheral disease: melanopsin pathway impairment in chronic inflammatory demyelinating polyneuropathy

Steiner,

Klostermann

2024

Brain Communications

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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) compromises functions of the peripheral nervous system (PNS). Recently, however, symptoms such as cognitive deficits, visual dysfunction, and circadian disorders were reported, compatible with additional involvement of the central nervous system (CNS) in CIDP. Against this background, we were interested in the functional state of melanopsin-expressing retinal ganglion cells (mRGCs) as a potential biomarker for sleep-wake abnormalities and CNS involvement in CIDP. Based on a chromatic pupillometry protocol, we examined the integrity of the melanopsin system in a prospective case-control study in 20 persons with CIDP compared to 20 controls without CIDP. The results were referred to clinical measures of disease severity and sleep behavior. CIDP patients had a significantly reduced melanopsin-mediated post-illumination pupil response (PIPR) compared to healthy controls (25% vs. 36%; p<0.01). This reduction correlated with disease severity (r= 0.478, p<0.05). Further, CIDP patients reported diminished sleep quality (p<0.05), however, there was no significant correlation with the melanopsin-mediated PIPR.The results demonstrate an impairment of mRGC function related to CIDP. Since the PIPR reduction correlated with disease severity, it could be an easily available biomarker for CNS affection in CIDP, a condition defined as PNS disorder.

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CNS Involvement in Chronic Inflammatory Demyelinating Polyneuropathy

Cited by 8 publications

References 46 publications

Real-world evidence of ocrelizumab-treated relapsing multiple sclerosis cohort shows changes in progression independent of relapse activity mirroring phase 3 trials

Real-world evidence of ocrelizumab-treated relapsing multiple sclerosis cohort shows changes in progression independent of relapse activity mirroring phase 3 trials

Vision Loss as a Presenting Feature of Chronic Inflammatory Demyelinating Polyneuropathy: A Case Series

Central involvement in peripheral disease: melanopsin pathway impairment in chronic inflammatory demyelinating polyneuropathy

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