Anterior periventricular linear lesions in optic-spinal multiple sclerosis: A combined neuroimaging and neuropathological study

There are two distinct subtypes of multiple sclerosis (MS) in Asians, optic-spinal (OSMS) and conventional (CMS). In OSMS, severe spinal cord lesions are characteristic while brain lesions are scant. We sought to clarify atypical brain lesions in OSMS by neuroimaging and pathological studies. For brain MRI, 124 consecutive Japanese patients with clinically definite MS based on Poser criteria were enrolled, 57 with OSMS and 67 with CMS. Ten autopsied cases (seven OSMS and three CMS) were studied pathologically. Although the frequency of brain lesions fulfilling Barkhof criteria was significantly higher in CMS than in OSMS, periventricular linear lesions along with the anterior portion of the corpus callosum and the lateral ventricles were significantly more common in OSMS than in CMS. Pathologically, periventricular lesions in CMS extended deeply into the white matter, while those in OSMS were confined to periventricular areas. T cell infiltration in lesions was prominent in CMS but not in OSMS. Although severe axonal loss and cavity formation were commonly seen in periventricular and spinal cord lesions in OSMS, lymphocytic infiltrates and vessel wall thickening were observed only in the latter. Thus, we suggested that anterior periventricular linear lesions without ovoid ones are characteristic of OSMS.