A population‐based study of long‐term outcome in treated chronic inflammatory demyelinating polyneuropathy

Al‐Zuhairy, Ali; Sindrup, Søren Hein; Andersen, Henning; Jakobsen, Johannes

doi:10.1002/mus.26772

Cited by 14 publications

(29 citation statements)

References 39 publications

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“…In our study, there was a strong correlation between follow‐up demyelination, axonal loss, and poorer clinical outcome, consistent with progressive disease activity and secondary axonal loss 6,9,36 . We found a clear relationship between delay in onset of treatment and long‐term axonal, demyelinating, and clinical features, indicating that prompt initiation of treatment is needed to reduce the risk of axonal loss and for long‐term prognosis in CIDP 35 . It is of interest that long‐term demyelination and axonal loss had improved in patients not requiring continued treatment.…”

Section: Discussionsupporting

confidence: 77%

Axonal loss at time of diagnosis as biomarker for long‐term disability in chronic inflammatory demyelinating polyneuropathy

et al. 2022

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Introduction/Aims: We hypothesized that early, pretreatment axonal loss would predict long-term disability, supported by a pilot study of selected patients with chronic inflammatory demyelinating polyneuropathy (CIDP). To further test this hypothesis, we examined a larger consecutive group of CIDP patients.Methods: Needle electromyography and motor and sensory nerve conduction studies were carried out in 30 CIDP patients at pretreatment and follow-up 5 to 28 years later. Changes in amplitudes were expressed as axonal Z scores and changes in conduction as demyelination Z scores and correlated with findings of the Inflammatory Rasch-built Overall Disability Scale (I-RODS), the Neuropathy Impairment Score (NIS), and isokinetic dynamometry (IKS).Results: At follow-up, the median I-RODS score was 73, the NIS was 23, and the IKS was 56%. The median axonal Z score was unchanged at follow-up. Conversely, the corresponding demyelination Z scores improved. The initial axonal loss was correlated with the clinical outcome and was an independent predictor of outcome by multivariate regression analysis. Axonal loss at follow-up was also correlated with the clinical outcome. Only the follow-up demyelination Z score was correlated with the clinical outcomes. Furthermore, the latency until treatment initiation was predictive of all three clinical outcome scores at follow-up, and of axonal loss and demyelination at follow-up.Discussion: The present study findings indicate that pretreatment axonal loss at diagnosis in CIDP is predictive of long-term disability, neurological impairment, and strength. A delay in treatment is associated with more pronounced axonal loss and a worse clinical outcome.

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Section: Discussionsupporting

confidence: 77%

Axonal loss at time of diagnosis as biomarker for long‐term disability in chronic inflammatory demyelinating polyneuropathy

et al. 2022

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“…Long-term severe disability of CIDP patients has also been correlated to the start delay of therapy [ 37 ], as also suggested by our clinical-neurophysiological correlation analysis showing that diagnostic and therapeutic delay is positively correlated both with CMAP amplitude and MUP area of all the examined muscles.…”

Section: Discussionsupporting

confidence: 55%

Neurophysiological Hallmarks of Axonal Degeneration in CIDP Patients: A Pilot Analysis

et al. 2022

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In this work, we aim to identify sensitive neurophysiological biomarkers of axonal degeneration in CIDP patients. A total of 16 CIDP patients, fulfilling the clinical and neurophysiological criteria for typical CIDP, treated with subcutaneous immunoglobulin (ScIg) (0.4 g/kg/week) were evaluated at baseline (before ScIg treatment) and after long-term treatment with ScIg (24 months) by clinical assessment scales, nerve conduction studies (NCS) and electromyography (EMG). Conventional and non-conventional neurophysiological parameters: motor unit potential (MUP) analysis, MUP thickness and size index (SI)] and interference pattern (IP) features were evaluated after long-term treatment (24 months) and compared with a population of 16 healthy controls (HC). An increase of distal motor latency (DML) and reduced compound motor action potential (CMAP) amplitude and area in CIDP patients suggest axonal damage of motor fibers, together with a significant increase of MUP amplitude, duration and area. Analysis of non-conventional MUP parameters shows no difference for MUP thickness; however, in CIDP patients, SI is increased and IP area and amplitude values are lower than HC. Despite clinical and neurophysiological improvement after ScIg treatment, neurophysiological analysis revealed axonal degeneration of motor fibers and motor unit remodeling. Correlation analysis shows that the axonal degeneration process is related to the diagnostic and therapeutic delay. MUP area and SI parameters can detect early signs of axonal degeneration, and their introduction in clinical practice may help to identify patients with the worst outcome.

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“…CIDP is a heterogeneous disorder with marked variability in treatment response 29 . With effective treatment, long‐term disability is generally limited, whereas poor outcome is tied to delay of therapy 85 . An ongoing multicenter, prospective study aims to better define the natural history of CIDP 86 .…”

Section: Outcome Measuresmentioning

confidence: 99%

Chronic inflammatory demyelinating polyradiculoneuropathy—Diagnostic pitfalls and treatment approach

2020

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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by progressive weakness and sensory loss, often affecting patientsʼ ability to walk and perform activities of daily living independently. With the lack of a diagnostic biomarker, the diagnosis relies on clinical suspicion, clinical findings, and the demonstration of demyelinating changes on electrodiagnostic (EDx) testing and nerve pathology. As a result, patients can often be misdiagnosed with CIDP and unnecessarily treated with immunotherapy. Interpreting the EDx testing and cerebrospinal fluid findings in light of the clinical phenotype, recognizing atypical forms of CIDP, and screening for CIDP mimickers are the mainstays of the approach to patients suspected of having CIDP, and are detailed in this review. We also review the currently available treatment options, including intravenous immunoglobulin (IVIg), corticosteroids (CCS), and plasma exchange (PE), and discuss how to approach treatment‐refractory cases. Finally, we emphasize the need to adopt objective outcome measures to monitor treatment response.

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A population‐based study of long‐term outcome in treated chronic inflammatory demyelinating polyneuropathy

Cited by 14 publications

References 39 publications

Axonal loss at time of diagnosis as biomarker for long‐term disability in chronic inflammatory demyelinating polyneuropathy

Axonal loss at time of diagnosis as biomarker for long‐term disability in chronic inflammatory demyelinating polyneuropathy

Neurophysiological Hallmarks of Axonal Degeneration in CIDP Patients: A Pilot Analysis

Chronic inflammatory demyelinating polyradiculoneuropathy—Diagnostic pitfalls and treatment approach

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