Lupus cerebritis

Findings

Diffuse, predominantly white matter T2/FLAIR hyperintense signal spares only the anterior temporal lobes. Focal lesions with hypointense T1 signal, low T2 signal, are heterogeneously hypointense on gradient echo, do not show restricted diffusion, and rim enhance. Ventricles, sulci, and cisterns remain normally configured and are of normal size for patient's age. No midline shift. Normal flow-voids in the major intracranial arteries.

Differential diagnosis:
- Lupus cerebritis
- Atypical infection
- Severe progressive PRES (posterior reversible encephalopathy syndrome) with high-dose steroids

Commentary: Initial study (not showed) has findings typical of lupus encephalopathy. The focal lesions in the second study are almost certainly hemorrhage. Given the rapid progression of these abnormalities, patient's history of lupus, and the development of multiple hemorrhagic foci, lupus cerebritis is most likely, with less likely differential diagnoses including severe progressive PRES with high dose steroids or atypical infection.


Diagnosis: Lupus cerebritis (presumed)


Key points

One acute subcategory of neuropsychiatric systemic lupus erythematosus (NPSLE).

Clinical
- Neurologic disorder which can present with any neurologic sign or symptom.
Focal: Seizure
Diffuse: Psychosis

Laboratory
- May be associated with high anti-ribosomal P autoantibody.
- Role of imaging
Assessment of acute focal (stroke-like) neurologic deficits.
CNS vasculitis.
Reported in up to 7% of SLE patients.


Radiology

High T2 signal in white matter.
- Sometimes in vascular distribution.
- Involves cortical and subcortical areas.
- Particularly occipital region.
- Relative sparing of periventricular white matter relative to MS.

Atrophy: Commonly found, secondary to
- Encephalopathy
- Steroid use

- Lesions may evolve in 7-10 day course
- Also possible
Hemorrhage
Subarachnoid
Intraparenchymal
Infarct
Aneurysms also reported