A 43-year-old African-American female with 13-year history of systemic lupus erythematosus (SLE) presents with one year of progressive leg weakness and incontinence. Prior treatment with hydroxychloroquine and prednisone. Neurologic exam remarkable for bilateral leg weakness, right greater than left, brisk patellar reflexes, absent ankle jerks, and bilateral Babinski signs. Sensory exam reduced to light touch in the right lower extremity.

Lumbar puncture performed and revealed the following: WBC=25. RBC=250. Lymphocytes= 89. Monocytes=11. Protein=69. Glucose=29. Results were interpreted by rheumatology and ID as an inflammatory process.

CT brain demonstrated curvilinear calcifications bilaterally in basal ganglia and jagged calcifications in the temporal lobe, cerebellum, and brainstem (Figures 1 and 2). Brain MRI revealed chronic small vessel disease with lacunar infarcts, superficial siderosis of the left frontal lobe, and multifocal intracranial calcifications.

The differential diagnosis for intracranial calcifications includes neurocysticercosis, congenital cytomegalovirus, toxoplasmosis, Fahr disease, Primary Familial Brain Calcification, SLE and other rare etiologies. After an extensive workup including VDRL CSF screen, HIV and hepatitis panels, Quantiferon gold assay, family history review for neurodegenerative or genetic disorders and evaluation of PTH and thyroid hormone for metabolic causes of Fahr syndrome was negative, it became clear that the cause of the calcifications was likely SLE.

Furthermore, MRI revealed atrophy of the cervical, thoracic, and lumbar spine. The progressive myelopathy was then attributed to SLE, B12 and copper deficiency.

Hospitalists should be aware that CNS calcifications can occur in various brain regions including basal ganglia and cerebellum in up to 30% of patients with lupus. Calcifications are better visualized on CT than MRI.