General description

Central nervous system lupus (CNS lupus), also known as neuropsychiatric SLE (NPSLE), refers to central nervous system complications caused by systemic lupus erythematosus (SLE). Common manifestations of CNS lupus include cognitive impairment, strokes due to cerebrovascular involvement, seizures, and chorea. When the spinal cord is affected, symptoms may present abruptly and progress rapidly, often leading to paraplegia, sensory deficits with a defined level, and bladder or rectal dysfunction. Approximately half of the cases of spinal cord involvement in SLE are associated with optic neuritis.

Radiographic features

Subcortical infarction

MRI reveals multiple subcortical infarctions consistent with vasculitis.

Meningitis

MRI shows thickening and contrast enhancement of the leptomeninges, consistent with abacterial meningitis. Contrast-enhanced FLAIR is particularly useful for detecting these lesions.

PRES

There are several reports suggesting that CNS lupus is associated with Posterior reversible encephalopathy syndrome (PRES). MRI typically shows T2WI and FLAIR hyperintensity in the parieto-occipital cortex and subcortical white matter, accompanied by an increased ADC value. The lesions are usually symmetric.

Calcification

CNS lupus may present with calcification in the globus pallidus, as well as in the cerebral and cerebellar white matter.

Chorea

CNS lupus with chorea is characterized by T1WI hyperintensity in the putamen and globus pallidus.

Transverse myelitis

MRI shows T2WI hyperintensity and swelling in the spinal cord, sometimes accompanied by contrast enhancement.