Inflammatory demyelinating diseases of the central nervous system

Abstract

Inflammatory demyelinating diseases are a heterogeneous group of disorders, which occur against the background of an acute or chronic inflammatory process. The pathologic hallmark of multiple sclerosis (MS) is the presence of focal demyelinated lesions with partial axonal preservation and reactive astrogliosis. Demyelinated plaques are present in the white as well as gray matter, such as the cerebral or cerebellar cortex and brainstem nuclei. Activity of the disease process is reflected by the presence of lesions with ongoing myelin destruction. Axonal and neuronal destruction in the lesions is a major substrate for permanent neurologic deficit in MS patients. The MS pathology is qualitatively similar in different disease stages, such as relapsing remitting MS or secondary or primary progressive MS, but the prevalence of different lesion types differs quantitatively. Acute MS and Balo's type of concentric sclerosis appear to be variants of classic MS. In contrast, neuromyelitis optica (NMO) and spectrum disorders (NMOSD) are inflammatory diseases with primary injury of astrocytes, mediated by aquaporin-4 antibodies. Finally, we discuss the histopathology of other inflammatory demyelinating diseases such as acute disseminated encephalomyelitis and myelin oligodendrocyte glycoprotein antibody-associated demyelination. Knowledge of the heterogenous immunopathology in demyelinating diseases is important, to understand the clinical presentation and disease course and to find the optimal treatment for an individual patient.

A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies

“…In line with previously published neuropathology of MOG-Abs-associated demyelination, our case did show well-demarcated partly confluent, partly perivenous plaques, relative preservation of axons, and numerous macrophages and complement deposition, compatible with demyelinating features of MS pattern II (14). In addition, some areas showed superimposed ischemic tissue damage with necrosis, loss of astrocytes and axons, and infiltration of neutrophilic granulocytes, most likely due to reduced perfusion in the context of the severe brain edema.…”

Pathology of inflammatory diseases of the nervous system: Human disease versus animal models

“…Inflammatory diseases of the central nervous system comprise a broad spectrum of infection related or putative autoimmune disorders, characterized by the presence of inflammatory infiltrates in the brain and spinal cord and immune mediated tissue injury (Höftberger & Lassmann, , ; Kuhlmann, Lassmann, & Brück, ; Table ). In general, the inflammatory process involves mechanisms of adaptive immunity, reflected by the abundant presence of T‐ and B‐lymphocytes and their local activation, proliferation, and clonal expansion within the lesions.…”

Secondary Hypogammaglobulinemia After Rituximab for Neuromyelitis Optica: A Case Report

“…NMO, also known as Devic disease, is an autoimmune demyelinating disease of the central nervous system that selectively affects the spinal cord and optic nerve. B-cell-mediated humoral immunity has been implicated in the pathogenesis of the disease [ 1 ], leading to resultant primary injury of astrocytes arbitrated by the formation of aquaporin-4 antibodies [ 2 ].…”