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. 2022 Nov 7;12(11):1510.
doi: 10.3390/brainsci12111510.

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

Dario Ricciardi  1 , Federica Amitrano  2 , Armando Coccia  2 , Vincenzo Todisco  1 , Francesca Trojsi  1 , Gioacchino Tedeschi  1 , Giovanni Cirillo  3
Affiliations

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

Dario Ricciardi et al. Brain Sci. .

Abstract

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.

Keywords: EMG; axonal degeneration; chronic inflammatory demyelinating polyneuropathy; motor unit analysis; subcutaneous immunoglobulin.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Exemplificative interference pattern analysis from BB muscle: the signal window between the two green vertical lines is selected by visual analysis by an expert technician. IP area is then computed on each 300 ms window. The window with the highest IP area is selected for the analysis.
Figure 2
Figure 2
(A–C) Analysis of nerve conduction parameters (DML and CMAP amplitude and area) in CIDP patients after ScIg treatment and HCs (CIDP patients vs. HCs). Figure legend: HCs: healthy controls; CIDP: chronic inflammatory demyelinating polyneuropathy; ScIg: subcutaneous immunoglobulin; MC: right musculocutaneous; Ul: left ulnar; Fem: left femoral; DP: right deep fibular; DML: distal motor latency; CMAP: compound muscle action potential.
Figure 3
Figure 3
(A) Size index and (B,C) interference pattern analysis (IPA) in CIDP patients after ScIg treatment and HCs. Figure legend: HCs: healthy controls; CIDP: chronic inflammatory demyelinating poly-neuropathy; ScIg: subcutaneous immunoglobulin; BB: brachial biceps; FDI: first dorsal interosseous; VL: vastus lateralis; EDB: extensor digitorum brevis; MUP: motor unit potential; IP: interference pattern; RMS: root mean square.
Figure 4
Figure 4
ROC curves for MUP area and size index (CIDP patients vs. HCs). Figure legend: BB: brachial biceps; FDI: first dorsal interosseous; VL: vastus lateralis; EDB: extensor digitorum brevis; MUP: motor unit potential; AUC: area under the curve.
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