Skip to main content

Table 1 Historical SLE cases in the literature with diffuse leukoencephalopathy

From: Leukoencephalopathy and cerebral edema as the presenting manifestations of SLE in an ANA-negative adolescent female: a case report and review of literature

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