Short-term, high-dose intravenous methylprednisolone therapy (IVMP), which is called steroid pulse therapy, is widely used as the standard treatment for acute exacerbations of multiple sclerosis (MS), and has been shown to improve neurological symptoms. IVMP is also applied in the acute phase of neuromyelitis optica (NMO), with considerable benefit, although some patients are refractory to IVMP, and the early use of plasmapheresis should be considered in these patients. IVMP acts by inhibiting the cascade of inflammation through several different mechanisms, including reducing the inflammatory cytokines and suppressing the T cell activation. IVMP is well tolerated and relatively safe, but attention should be paid to the development of adverse events, such as psychosis, hyperglycemia and osteonecrosis.