Motor chronic inflammatory demyelinating polyneuropathy (CIDP) in 17 patients: Clinical characteristics, electrophysiological study, and response to treatment

Abstract

Background and Aims: Motor chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare poorly described subtype of CIDP. We aimed to study the clinical and electrophysiological characteristics and as response to treatment. Methods: From a prospective database of CIDP patients, we included patients with definite or probable CIDP with motor signs and without sensory signs/symptoms at diagnosis. Patients were considered to have pure motor CIDP (PM-CIDP) if sensory conductions were normal or to have motor predominant CIDP (MPred-CIDP) if ≥2 sensory nerve action potential amplitude were abnormal. Results: Among the 700 patients with CIDP, 17 (2%) were included (PM-CIDP n=7, MPred-CIDP n=10); 71% were male, median age at onset was 48 years (range:13-76 years), 47% had an associated inflammatory or infectious disease or neoplasia. At the more severe disease stage, 94% of patients had upper and lower limb weakness, with distal and proximal weakness in 4 limbs for 56% of them. Three-quarters (75%) responded to intravenous immunoglobulins (IVIg) and 4/5 patients to corticosteroids including 3/3 patients with MPred-CIDP. The most frequent conduction abnormalities were conduction blocks (CB, 82%) and F-wave abnormalities (88%). During follow-up, 4/10 MPred-CIDP patients developed mild sensory symptoms; none with PM-CIDP did so. Patients with PM-CIDP had poorer outcome (median ONLS:4, range:2-5) compared to MPred-CIDP (2, range:0-4;p=0.03) at last follow-up. Conclusions: The present study found a progressive clinical course in the majority of patients with motor CIDP as well as frequent associated diseases, CB, and F-wave abnormalities. Corticosteroids might be considered as a therapeutic option in resistant IVIg patients with MPred-CIDP.

European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force—Second revision

“…If sensory nerve conduction is abnormal in clinically motor CIDP, 55 the diagnosis is motor‐predominant CIDP. Patients with motor CIDP may deteriorate after corticosteroids (PICO 8) 36,52,55,56 Sensory CIDP is usually characterized by gait ataxia, impairment of vibration and position sense and changes in cutaneous sensation 35,57,58 .…”

“…Low to moderate certainty evidence suggests that there are fewer side‐effects and a faster response with pulsed high‐dose corticosteroid compared with daily oral corticosteroid treatment. Some patients with CIDP may deteriorate after corticosteroid treatment, especially those with motor CIDP 36,52,55 . Therefore, corticosteroids are not recommended as first‐line treatment in these patients 105 …”

“…• Chest X-ray corticosteroid treatment, especially those with motor CIDP. 36,52,55 Therefore, corticosteroids are not recommended as first-line treatment in these patients.…”

“…Patients with motor CIDP may deteriorate after corticosteroids (PICO 8). 36,52,55,56 • Sensory CIDP is usually characterized by gait ataxia, impairment of vibration and position sense and changes in cutaneous sensation. 35,57,58 By definition, muscle weakness is not present.…”

European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force—Second revision

“…This is in contrast to both typical CIDP, where sensation is abnormal, and multifocal motor neuropathy (MMN), where the pattern of weakness is asymmetric and mainly affecting the upper limbs [54]. If sensory nerve conduction is abnormal in clinically motor CIDP [55], the diagnosis is motor‐predominant CIDP. Patients with motor CIDP may deteriorate after corticosteroids (PICO 8) [36,52,55,56].…”