Demyelinating diseases are characterized by a loss of oligodendrocytes leading to axonal degeneration and impaired brain function. Current strategies used for the treatment of demyelinating disease such as multiple sclerosis largely rely on modulation of the immune system. Only limited treatment options are available for treating the later stages of the disease, and these treatments require reg...

Down syndrome (DS) is one of the most common survivable chromosomal disorders, and is well known to be associated with multiple autoimmune diseases. Multiple sclerosis (MS) is a chronic inflammatory autoimmune demyelinating disease of the central nervous system. An association of DS and other autoimmune disease has been previously reported, and we report one case of DS in coexistence with MS, d...

Cerebrospinal fluid (CSF) analysis very frequently makes the difference to the diagnosis, not only in relation to infections but also in other diseases of the nervous system such as inflammatory, demyelinating, neoplastic and degenerative diseases. The authors review some practical and important features of CSF analysis in infectious diseases of the nervous system, with regard to acute bacteria...

We discuss two of the most common of the acquired inflammatory neuropathies: Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy, as well as their variants. We review their clinical presentation, electrophysiologic findings, and management, highlighting knowledge gained from the recent literature. Unfortunately, although treatments exist for both Guillain-Barre syndrom...

Death receptors (DRs) are members of the tumor necrosis factor receptor (TNF-R) superfamily that are characterised by the presence of a conserved intracellular death domain and are able to trigger a signalling pathway leading to apoptosis. Strong evidence suggests that DRs contribute to the pathology of tissue destructive diseases, including multiple sclerosis (MS), the most common inflammatory...

Acute disseminated encephalomyelitis (ADEM) is an uncommon monophasic idiopathic inflammatory demyelinating disease. Available diagnostic criteria do not reliably distinguish it from first presentations of relapsing diseases such as multiple sclerosis (MS) and neuromyelitis optica (NMO). In this article, we review current concepts about ADEM and what distinguishes it from other idiopathic infla...

Neuromyelitis optica (NMO) is a rare and severe inflammatory disease of the central nervous system (CNS), which constitutes up to 5% of pediatric aquired demyelinating diseases. The optic nerves and the spinal cord are the most affected sites. The discovery of an autoantibody called NMO-IgG, which targets aquaporin-4, the main water channel in the CNS, gave a new direction to understanding the ...

In the past few years several studies have consistently reported the presence of high-titer serum IgG antibodies to conformational epitopes of the myelin oligodendrocyte glycoprotein (MOG) in predominantly pediatric patients with acquired demyelinating diseases. Moreover, MOG antibodies seem not only to distinguish patients with clinically isolated syndromes or multiple sclerosis (MS) from mono...

Myelinating cells wrap axons with multi-layered myelin sheaths for rapid impulse propagation. Dysfunctions of oligodendrocytes or Schwann cells are often associated with neuroinflammation, as observed in animal models of leukodystrophies and peripheral neuropathies, respectively. The neuroinflammatory response modulates the pathological changes, including demyelination and axonal injury, but al...

Treatments with anti-TNF alpha (ATA) are now commonly used in certain inflammatory rheumatic diseases such as spondyloarthritis, rheumatoid arthritis or psoriatic arthritis, and inflammatory bowel disease (IBD). Among the different therapeutic classes available, there are, firstly, chimeric or humanized monoclonal antibody such as infliximab (Remicade) and adalimumab (Humira) and, secondly, eta...