Diffuse intracranial calcification, deep medullary vein engorgement, and symmetric white matter involvement in a patient with systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a chronic relapsing‐remitting autoimmune disease with multiple autoantibodies causing damage to different target organs. Central nervous system is commonly in‐ volved, with a myriad of clinical and radiologic features always com‐ plicating the diagnosis. In this study, we report an atypical case of SLE with brain involvement showing a variety of unique and novel neuroimaging findings. A 53‐year‐old woman presented with slurred speech, deep voice, and gait instability for over 1 year. She had a 13‐year history of SLE and had been treated with corticosteroid. Neurological examination revealed a female with the moon face, dysarthria, bradykinesia, ri‐ gidity, hyperreflexia of the upper extremities, and abnormal tandem gait. Routine laboratory examinations including parathyroid hor‐ mone and serum calcium were normal except for immunologic tests, which showed antinuclear antibodies titer 1:80 (++), anti‐ double‐stranded deoxyribonucleic acid antibodies 563.8 IU/mL (reference range < 100 IU/mL), antiribonucleic protein antibodies (++), anti‐Sjögren's‐syndrome‐related antigen A antibodies (+++), and anti‐Ro52 antibodies (+++). Cerebrospinal fluid assay revealed no abnormalities. Computed tomography (CT) scan of the chest, ultrasounds of the lymph nodes, and abdomen were normal. Brain magnetic resonance imaging revealed patchy lesions in centrum se‐ miovale and periventricular regions, with prolonged signals on T1 and T2‐weighted images and normal signals on diffusion‐weighted images, suggesting leukoencephalopathy; additional lesions bilater‐ ally in the basal ganglia, thalamus, and cerebellar dentate nucleus showed shortened signals on T1 and T2 images, as well as low sig‐ nals on susceptibility weighted imaging (SWI), with medullary veins slightly dilated (Figure 1). Brain magnetic resonance angiography was normal. Head CT demonstrated multiple patchy and punctate lesions in the bilateral lenticular nucleus and cerebellar dentate nucleus, as well as radiating lesions along the periventricular area (Figure 2). Those lesions have a symmetric distribution with a CT unit of around 300 HU, which were consistent with calcification. Targeted next‐generation sequencing was unrevealing, ruling out hereditary leukoencephalopathy, and intracranial calcification‐re‐ lated hereditary diseases. A final diagnosis of central nervous system lupus (CNSL) was made. After consulting with the rheumatologist, the dosage of