Table 1.

Historical SLE cases in the literature with diffuse leukoencephalopathy

Patient (ref)	Age/Sex at presentation	Initial Presentation	Previous diagnosis of SLE	Previous neurological involvement	Neuroimaging	Positive ANA?	Other Ab Results Reported	Treatment	Outcome
1 [8]	38yo/F	Severe headache, syncope	YES	NO	CT: diffuse cerebral edema MRI: diffuse white matter hyperintensities	YES (1:2560)	-anti-dsDNA	3 day pulse-dose steroids→ oral prednisone, plaquenil	Herniation → death
2 [9]	11yo/F	Malar rash, photosensitivity, prolonged fever, hemolysis, generalized convulsions, unconsciousness	NO	N/A	MRI: high signal intensity in b/l basal ganglia and thalami, hyperintensities in deep white matter, pons, b/l caudate heads, putamens, thalami	YES	+anti-dsDNA +anti-ssDNA +anti-RNP +anti-Smith +anti-SSA	3 day pulse-dose steroids, IV 500 mg/day methylprednisolone	Return to baseline 1 year after insult
3 [8, 10]	14yo/F	HA 1 mo, progressive vomiting 1 week, abducens palsy 5 days	YES	NO	CT: Diffuse white matter hypodensity without ventricular dilatation. MRI: diffuse white matter hyperintensities	YES (1:320)	Unknown	3 day pulse-dose steroids w/steroid taper, ranitidine, plaquenil 200 mg.	No further recurrence, stable neurologically
4 [11]	35yo/F	Headache, mild Papilledema, skin eruption, fever	NO	N/A	MRI: diffuse hyperintense white matter lesions	YES	+anti-dsDNA	Unknown	Unknown
5 [12]	49yo/F	5wk constant HA, AMS, somnolence	YES	YES	CT: diffuse cerebral edema, small SAH MRI: diffuse sulcal hyperintensity	YES	+anti-dsDNA	Mannitol, 7 day high-dose steroids, IVIG, steroid taper	4 weeks from discharge, no recurrence
6 [13]	28yo/F	fever, malaise, facial edema, diplopia	NO	N/A	MRI: asymmetrical, multifocal high signal intensity lesions in subcortical white matter Gadnolinium: leptomeningial enhancement	Unknown	Unknown	3 days high-dose steroid pulse	Unclear
7 [14]	7yo/F	4 days ataxia, diplopia, morning vomiting; 1 yr hx of HA, recurrent vomiting, cognitive dysfunction	NO	N/A	CT: bilateral widening of the horizontal sulcus of cerebellum MRI: multiple cortico-subcortical lesions in both cerebral hemispheres with increased signal intensity.	YES (1:5120)	+anti-dsDNA -anti-RNP -anti-Smith -anti-Ro -anti-La -anti-mitochondrial	steroid pulse monthly, Cyclophosphamide monthly, continuous oral prednisolone	stabilization w/residual ataxia, dysmetria, psychomotor slowing.
8 [15]	32 yo/F	Nausea, vomiting, diplopia	NO	N/A	CT: diffuse cerebral edemaMRI: bilateral symmetric diffuse FLAIR hyperintensities Cerebral angiogram: no vasculitis	YES (1:1280)	+anti-dsDNA +anti-Smith	IV steroid pulse, Plasmapheresis, Cyclophosphamide, Acetazolamide, Mannitol, Hypertonic saline, hypothermia	Recalcitrant cerebral edema, sepsis, multi-organ failure ➔ death
9 [15]	29 yo/F	Loss of consciousness	YES	Unknown	CT: diffuse cerebral edema Cerebral angiogram: negative for vasculitis	Unknown	Unknown	Hydroxychloroquine, mycophenolate, IV methylprednisolone, IVIG	Recalcitrant cerebral edema ➔ death by neurologic criteria
10 [8, 16]	56 yo/F	Generalized macular rash, raynaud’s phenomenon, diarrhea, steady neurologic decline, dysphagia, pleural effusions, lymphopenia	NO	N/A	CT: normal MRI: extensive, confluent hyperintensity of the cerebral and cerebellar white matter	NO	+anti-dsDNA -anti-ENA -anti-Smith -anti-RNP -anti-La -anti-Ro	80 mg oral prednisone daily	Improvement of speech, swallowing. 1 year later ➔ mild hypophonia, some memory trouble
11 [8, 17]	35 yo/F	erythematous rash, polyarthropathy, Headache, photophobia,memory impairment	NO	N/A	CT: diffuse, uniform low attenuation in the white matter.	YES (1:320)	+anti-dsDNA	Oral prednisone, azathioprine	Improvement with oral prednisone
12 [8, 18]	41 yo/M	HA, vertigo, proteinuria, anemia, papilledema, retinal bleeding	YES	Unknown	Brain CT: diffuse brain edema MRI: diffuse white matter hyperintensities	YES	+anti-Smith	IV methylprednisolone, osmotic diuretics	Improvement in symptoms