Rare and serious types of central nervous system demeylinating diseases and MS types

Abstract

Multiple sclerosis (MS) is an inflammatory condition causing demyelination and axonal loss in the central nervous system (brain and spinal cord). The primary brain insult (“sclerosis”) and its clinical features are “dissemination in space”, affecting different anatomical sites, and “dissemination in time”, appearing episodically over time. The common types of MS includes-Relapsing and remitting type (RRMS)-Secondary progressive MS (SPMS)-Primary progressive MS ( PPMS) The sub-division is based more on clinical characteristics and not specific biological pathophysiology. Nonetheless, they provide an organized framework for diagnosis and long-term management. Multiple Sclerosis was initially thought to be a rare condition in countries close to the equator such as India. However that has changed now and we do see cases of Multiple Sclerosis in this part of the world. And occasionally we do also come across patients with evidence of demyelination in the CNS which is resistant to treatment and has a rapidly progressive course which even leads to death. These rare types of MS includes Marburg variety and tumefactive MS. We have three such patients who presented with rapidly progressive neurological deficits and showed such a rampant course, in spite of various anti-inflammatory agents used including Rituximab. This presentation is to highlight the fact that demyelinating disease of the CNS could be quite dangerous from the onset and rare types of MS should be considered early in such cases.