Chronic Inflammatory Demyelinating Polyradiculoneuropathy: From Bench to Bedside

Abstract

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is the most common treatable chronic autoimmune neuropathy. Multiple diagnostic criteria have been established, with the primary goal of identifying neurophysiologic hallmarks of acquired demyelination. Treatment modalities have expanded to include numerous immuno-modulatory therapies, although the best evidence continues to be for corticosteroids, plasma exchange, and intravenous immunoglobulins (IVIg). This review describes the pathology, epidemiology, pathogenesis, diagnosis, and treatment of CIDP.

Chronic inflammatory demyelinating polyradiculoneuropathy relapse after mexiletine withdrawal in a patient with concomitant myotonia congenita

“…[ 6 ] CIDP onset is insidious. [ 7 – 10 ] Its clinical course is slowly progressive (over at least 2 months), with relapses, and includes symmetric weakness of both muscles around the hip and shoulder, as well as of the hands and feet (both proximal and distal muscles), and sensory disturbances causing incoordination, numbness, tingling, or prickling sensations. [ 7 – 10 ] Other CIDP symptoms may include fatigue, burning sensation, pain, clumsiness, difficulty in swallowing, and double vision.…”

“…Walking may be involved when responses to various sensory stimuli are seriously impaired. [ 7 – 10 ] The immune system firstly attacks the myelin sheaths of the PNS, inducing the typical segmental demyelination and then axonal degeneration. [ 9 ] This gets slower nerve conduction velocity, reduces sensory and motor responses amplitude, and provokes conduction blocks at the neurophysiological studies.…”

Conductive hearing loss in chronic inflammatory demyelinating polyneuropathy (CIDP): A case report

“…It was later identified as an autoimmune disorder targeting myelin sheaths rather than axons themselves due to its lack of associated muscle atrophy (Dyck and Tracy, 2018). While CIDP triggers have not been identified, the pathogenesis results from segmental inflammatory infiltrates in the perivascular space of nerves (Peltier and Donofrio, 2012). Its prevalence ranges from 1 to 8.9/100,000, and is typically higher in the male population and can occur at any age (Baig et al, 2012; Hattori et al, 2001).…”

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): An Uncommon Manifestation of Systemic Lupus Erythematosus (SLE)

“…The diagnosis of CIDP is more likely when the patient has a predominance of sensory symptoms over motor symptoms. Although the cause of CIDP is unknown, there is evidence to support an autoimmune etiology with multiple immunological triggers [ 3 , 4 ]. Both the cellular and humoral components of the immune system appear to be involved in the pathogenesis of CIDP and its variants [ 3 , 4 ].…”

“…Although the cause of CIDP is unknown, there is evidence to support an autoimmune etiology with multiple immunological triggers [ 3 , 4 ]. Both the cellular and humoral components of the immune system appear to be involved in the pathogenesis of CIDP and its variants [ 3 , 4 ]. An estimated 10–20% of SLE patients show peripheral nervous system involvement and patients present with sensorimotor polyneuropathies, with less common syndromes including mononeuritis multiplex or asymmetric polyneuropathy and acute or chronic demyelinating polyneuropathy [ 5 ].…”