Immune-Mediated Neuropathies: Pathophysiology and Management

Abstract

Dysfunction of the immune system can result in damage of the peripheral nervous system. The immunological mechanisms, which include macrophage infiltration, inflammation and proliferation of Schwann cells, result in variable degrees of demyelination and axonal degeneration. Aetiology is diverse and, in some cases, may be precipitated by infection. Various animal models have contributed and helped to elucidate the pathophysiological mechanisms in acute and chronic inflammatory polyradiculoneuropathies (Guillain–Barre Syndrome and chronic inflammatory demyelinating polyradiculoneuropathy, respectively). The presence of specific anti-glycoconjugate antibodies indicates an underlying process of molecular mimicry and sometimes assists in the classification of these disorders, which often merely supports the clinical diagnosis. Now, the electrophysiological presence of conduction blocks is another important factor in characterizing another subgroup of treatable motor neuropathies (multifocal motor neuropathy with conduction block), which is distinct from Lewis–Sumner syndrome (multifocal acquired demyelinating sensory and motor neuropathy) in its response to treatment modalities as well as electrophysiological features. Furthermore, paraneoplastic neuropathies are also immune-mediated and are the result of an immune reaction to tumour cells that express onconeural antigens and mimic molecules expressed on the surface of neurons. The detection of specific paraneoplastic antibodies often assists the clinician in the investigation of an underlying, sometimes specific, malignancy. This review aims to discuss the immunological and pathophysiological mechanisms that are thought to be crucial in the aetiology of dysimmune neuropathies as well as their individual electrophysiological characteristics, their laboratory features and existing treatment options. Here, we aim to present a balance of discussion from these diverse angles that may be helpful in categorizing disease and establishing prognosis.

Only anti-GM4 antibody positivity in a Chinese girl with overlapping MFS/GBS: a case report

“…GBS is an inflammatory polyneuropathy characterized by progressive flaccid ascending and symmetric muscle weakness associated with motor features and the presence or absence of sensory symptoms [ 2 ]. GBS is subclassified into classic forms, which present with acute flaccid paralysis of all four limbs, and localized forms, including pharyngeal–cervical–brachial weakness, acute pharyngeal weakness, paraparetic GBS, and bifacial weakness with paresthesias.…”

Identifying circRNA–miRNA–mRNA Regulatory Networks in Chemotherapy-Induced Peripheral Neuropathy

“…Consistently, we found that, in CIPN rats, there was a meaningful correlation between overlapping mRNAs and many immune cells. According to the immune infiltration analysis of hub genes, Cdh1 mainly correlates with plasmacytoid dendritic cells and macrophages, which can modulate the function of nociceptor sensory neurons and the development of neuropathies [ 59 , 60 ]. Fas and P2ry2 are negatively correlated with monocytes, Satb2 is positively correlated with Type 2 T helper cells, and Zfhx2 is closely related to T cells CD4 memory resting, suggesting their importance in CIPN and immunity.…”

An updated review on Guillain-Barré syndrome: Challenges in infection prevention and control in low- and middle-income countries

“…Guillain-Barré syndrome (GBS) is an inflammatory polyneuropathy distinguished by a gradual onset of symmetrical, flaccid hypoxia in the ascending extremities, accompanied by motor symptoms with or without sensory manifestations. 1 It is a severe inflammatory peripheral radiculopathy and neuropathy with a reported global incidence of about 100,000 new cases per year. It is characterized by progressive limb weakness, sensory deficits, cranial nerve involvement, tendon areflexia, and cerebrospinal fluid (CSF) albuminocytological dissociation.…”