Axonal loss at time of diagnosis as biomarker for long-term disability in chronic inflammatory demyelinating polyneuropathy
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Axonal loss at time of diagnosis as biomarker for long-term disability in chronic inflammatory demyelinating polyneuropathy. / Al-Zuhairy, Ali; Jakobsen, Johannes; Moldovan, Mihai; Krarup, Christian.
In: Muscle and Nerve, Vol. 66, No. 6, 2022, p. 715-722.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - Axonal loss at time of diagnosis as biomarker for long-term disability in chronic inflammatory demyelinating polyneuropathy
AU - Al-Zuhairy, Ali
AU - Jakobsen, Johannes
AU - Moldovan, Mihai
AU - Krarup, Christian
N1 - Publisher Copyright: © 2022 The Authors. Muscle & Nerve published by Wiley Periodicals LLC.
PY - 2022
Y1 - 2022
N2 - 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.
AB - 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.
KW - axonal loss
KW - electrophysiological examination
KW - long-term follow-up
KW - prediction of CIDP
KW - prognosis
U2 - 10.1002/mus.27722
DO - 10.1002/mus.27722
M3 - Journal article
C2 - 36217677
AN - SCOPUS:85140469874
VL - 66
SP - 715
EP - 722
JO - Muscle & Nerve
JF - Muscle & Nerve
SN - 0148-639X
IS - 6
ER -
ID: 323921139