No abstract

White matter diseases include a wide spectrum of disorders that have in common impairment of normal myelination, either by secondary destruction of previously myelinated structures (demyelinating processes) or by primary abnormalities of myelin formation (dysmyelinating processes). The pathogenesis of many white matter diseases remains poorly understood. Demyelinating disorders are the object of this review and will be further divided into autoimmune, infectious, vascular, and toxic-metabolic processes. Autoimmune processes include multiple sclerosis and related diseases: tumefactive demyelinating lesions, Balo concentric sclerosis, Marburg and Schilder variants, neuromyelitis optica (Devic disease), acute disseminated encephalomyelitis, and acute hemorrhagic leukoencephalopathy (Hurst disease). Infectious processes include Lyme disease (neuroborreliosis), progressive multifocal leukoencephalopathy, and human immunodeficiency virus (HIV) encephalopathy. Vascular processes include different types of small-vessel disease: arteriolosclerosis, cerebral amyloid angiopathy, cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), primary angiitis of the central nervous system, Susac syndrome, and neurolupus. Toxic-metabolic processes include osmotic myelinolysis, methotrexate leukoencephalopathy, and posterior reversible encephalopathy syndrome. The imaging spectrum can vary widely from small multifocal white matter lesions to confluent or extensive white matter involvement. Understanding the pathologic substrate is fundamental for understanding the radiologic manifestations, and a systematic approach to the radiologic findings, in correlation with clinical and laboratory data, is crucial for narrowing the differential diagnosis. (©)RSNA, 2016.

No abstract

Neuropsychiatric lupus is a major diagnostic challenge, and a main cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Magnetic resonance imaging (MRI) is, by far, the main tool for assessing the brain in this disease. Conventional and advanced MRI techniques are used to help establishing the diagnosis, to rule out alternative diagnoses, and recently, to monitor the evolution of the disease. This review explores the neuroimaging findings in SLE, including the recent advances in new MRI methods.

Objectives The objective of this study was to determine whether advanced MRI could provide biomarkers for diagnosis and prognosis in neuropsychiatric systemic lupus erythematosus (NPSLE). Methods Our prospective study included 28 systemic lupus erythematosus (SLE) patients with primary central NPSLE, 22 patients without NPSLE and 20 healthy controls. We used visual scales to evaluate atrophy and white matter hyperintensities, voxel-based morphometry and Freesurfer to measure brain volume, plus diffusion-tensor imaging (DTI) to assess white matter (WM) and gray matter (GM) damage. We compared the groups and correlated MRI abnormalities with clinical data. Results NPSLE patients had less GM and WM than controls ( p = 0.042) in the fronto-temporal regions and corpus callosum. They also had increased diffusivities in the temporal lobe WM ( p < 0.010) and reduced fractional anisotropy in the right frontal lobe WM ( p = 0.018). High clinical scores, longstanding disease, and low serum C3 were associated with atrophy, lower fractional anisotropy and higher diffusivity in the fronto-temporal lobes. Antimalarial treatment correlated negatively with atrophy in the frontal cortex and thalamus; it was also associated with lower diffusivity in the fronto-temporal WM clusters. Conclusions Atrophy and microstructural damage in fronto-temporal WM and GM in NPSLE correlate with severity, activity and the time from disease onset. Antimalarial treatment seems to give some brain-protective effects.