Clinicopathological features of inflammatory demyelinating diseases in biopsy

摘要

Inflammatory demyelinating diseases (IDD) of the central nervous system (CNS) occur throughout the world and are the leading cause of nontraumatic neurological disability in young adults. Early diagnosis of IDD allows prompt immunotherapies that minimize relapse, disabilities and mortality. Multiple sclerosis (MS) is the most frequent IDD in adults. The histological characteristics of chronic MS lesions are well known to neuropathologists; however, diagnosing an inflammatory demyelinating process in biopsy may be challenging since the specimens are frequently small and often represent only parts of a larger lesion. Furthermore, the histopathological hallmarks of acute MS differ fundamentally from the well-known characteristics of chronic demyelinated plaques observed in autopsies. Kuhlmann recently proposed a new histological classification system for MS lesions. Whether this new classification system corresponds to clinical manifestations and thus reflects the temporal multiplicity of MS and whether it can be applied to all IDD cases remain to be further studied.