encephalomyelitis, neuromyelitis optica, and multiple sclerosis
نویسندگان
چکیده
Purpose of review Advanced immunopathological techniques hold promise for more precise diagnosis of idiopathic demyelinating diseases of the central nervous system. We review recent progress in differentiating and understanding the disease mechanisms of acute disseminated encephalomyelitis, neuromyelitis optica, and classical multiple sclerosis. Recent findings Four distinct immunopathological patterns have been described in multiple sclerosis patients, potentially implicating different inflammatory, demyelinating, and apoptotic mechanisms. A specific serum biomarker, neuromyelitis optica immunoglobulin G, is strongly associated with neuromyelitis optica and identifies patients with severe optic nerve and spinal cord lesions with specific pathological features such as eosinophilic and neutrophilic inflammatory infiltrates, necrosis, vascular hyalinization, and extensive vasculocentric immunoglobulin and complement deposition. This biomarker targets the water channel aquaporin-4, which is lost in neuromyelitis optica lesions. Acute disseminated encephalomyelitis still has no validated clinical diagnostic criteria but its perivenous pathological findings distinguish it from multiple sclerosis and neuromyelitis optica. Summary The clinically heterogeneous group of idiopathic inflammatory demyelinating diseases of the central nervous system is characterized by several immunopathological patterns that suggest the involvement of diverse pathogenic effector mechanisms. Future advances in experimental pathology, immunology, molecular genetics, and neuroimaging, as well as the discovery of specific biomarkers, will more precisely define these disorders and lead to better targeted therapies.
منابع مشابه
Epidemiology, immunopathogenesis and management of pediatric central nervous system inflammatory demyelinating conditions.
PURPOSE OF REVIEW Pediatric inflammatory demyelinating central nervous system diseases comprise monofocal and potentially monophasic disorders like optic neuritis and transverse myelitis, the multifocal, self-limiting disorder of acute disseminated encephalomyelitis, and multifocal chronic diseases like relapsing neuromyelitis optica and multiple sclerosis. This review discusses characteristics...
متن کاملHistopathology and clinical course of MOG-antibody-associated encephalomyelitis
We present histological, MRI, and clinical features of an adult patient with relapsing encephalomyelitis and antibodies against myelin oligodendrocyte glycoprotein (MOG). Furthermore, we report molecular details of the recognized epitope that is specific for human MOG. A brain biopsy revealed multiple sclerosis (MS)-type II pathology. Some features overlapped with both MS and neuromyelitis opti...
متن کاملAcute disseminated encephalomyelitis: current understanding and controversies.
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...
متن کاملEfficacy and Safety of Rituximab in patients with Neuromyelitis Optica Spectrum Disorders: A prospective observation in Iranian Cases.
متن کامل
Acute cervical myelitis
Acute myelitis represents a major subset of acute non-compressive myelopathies. Acute non-compressive myelopathies include vascular, infectious, autoimmune, inflammatory, neoplastic and metastatic disorders. Acute myelitis can occur as an isolated inflammatory disorder, or as part of a multifocal CNS demyelinating disease such as acute disseminated encephalomyelitis, multiple sclerosis, neuromy...
متن کامل