Table 3. Neuroimaging Findings and Histopathology.

Subject	Terminal NPSLE Event	MRI to Death Interval	Cause of Death	Neuroimaging	Brain Histopathology
1	Acute ischemic stroke associated with accelerated SLE	2 weeks	-acute stroke superimposed on chronic multifocal disease secondary to active SLE, -tonsillar herniation, -Libman-Sacks -endocarditis, -antiphospholipid antibody syndrome.	-moderate cortical atrophy -severe ventricular dilation -severe multifocal white matter abnormalities -chronic diffuse white matter abnormalities -multiple old and new infarcts	Brain 1145 gms -left cerebral cortex soft and edematous -multiple new and old cerebral infarctions -microinfarcts -severe calcific atherosclerosis of the internal carotid arteries -left internal carotid occluded by fresh thrombus -post-infarctive cyst in right superior temporal lobe -arteries with multiple platelet and fibrin microemboli -left frontal lobe undergoing coagulation necrosis secondary to acute cerebral infarction.
2	Acute confusional state, Fahr's disease, seizure disorder (associated with accelerated SLE)	1 month	-Fahr's disease -active SLE -seizure disorder -diffuse ischemic encephalopathy, -antiphospholipid antibody syndrome -sepsis	-moderate cortical atrophy -moderate ventricular dilation -extensive focal white matter abnormalities -multiple calcifications -increased T2 in basal cisterns, periventricular white matter, and centrum semiovale.	Brain 1540 gms -extensive calcinosis -varying degrees of blood vessel calcification -small round oval calcospherites surrounding the small capillaries; homogeneous rings of calcium in larger vessels, many vessels completely occluded -vessels show evidence of chronic thrombosis and recanalization -small acute and chronic microinfarcts
3	Acute confusional state, seizure disorder (associated with accelerated SLE)	4 days	-active SLE -diffuse ischemic encephalopathy -cerebral edema with tonsillar herniation -peripheral vasculitis -Libman-Sacks endocarditis -antiphospholipid syndrome -sepsis	-moderate cortical atrophy and ventricular dilation -minimal focal white matter abnormalities -few punctate hypointense lesions -late brain edema -tonsillar herniation	Brain 1160 gms -cerebral edema -hemorrhagic necrosis and herniation of the right cerebellar tonsil -multifocal areas of loss of neurons with gliosis and microglial proliferation -myelinated fibers reduced in numbers -hemorrhages in basal ganglia -few arterioles occluded with fibrin -cerebellum with moderate loss of Purkinje cells -microinfarctions.
4	Chronic multifocal disease with dementia	1 month	-respiratory arrest -overdose with oxycodone -diffuse ischemic encephalopathy -active SLE -Libman-Sacks endocarditis -antiphospholipid syndrome	-severe cortical atrophy -severe ventricular dilation -multiple cortical infarcts -multiple small focal lesions -chronic frontoparietal diffuse white matter abnormalities.	Brain 1080 gms -dropout of neurons of variable distribution -multiple infarcts of various ages -vessels with chronic remodeling changes suggesting recurrent thrombosis and recanalization.
5	Acute confusional state and seizure disorder, (associated with accelerated SLE)	2 weeks	-active SLE -diffuse ischemic encephalopathy -cerebral edema with tonsillar herniation -Libman -Sacks endocarditis, -bacterial endocarditis -sepsis.	-moderate cortical atrophy and ventricular dilation -small focal lesions -few deep white matter lesions -late cerebral edema	Brain 1280 gms -gyri diffusely flattened with loss of normal sulci -diffuse cerebral edema -transtentorial and uncal herniation -diffuse softening of temporal lobes -multiple small petechial hemorrhages -extensive ischemic change with laminar necrosis -neuronal loss -ischemic red neurons with foamy histiocytes -scattered diffuse fibrin and platelet thrombi -leptomeninges with mononuclear inflammatory cells -multiple microinfarctions.
6	Seizure disorder (epilepsy)	1 year	-seizure disorder diffuse ischemic encephalopathy antiphospholipid syndrome -noncompliance with antiseizure drugs	-moderate cortical atrophy and ventricular dilation -focal white matter abnormalities -multiple resolved -cortical infarcts -cortical cysts.	Brain 1080 gms -three areas of atrophy of cerebral cortex with parenchymal loss -small cysts, macrophages and reactive astrocytosis, focal area of neuronal loss in hippocampus, and multiple resolved cerebral infarctions.
7	Seizure disorder (associated with accelerated SLE)	2 months	-seizure disorder diffuse ischemic encephalopathy	-minimal cortical atrophy -minimal ventricular dilation, -moderate focal white matter abnormalities	Brain 1400 gm -brain largely unremarkable with a few areas of vague focal reductions in neuron and axon numbers in a patchy distribution and foci of multifocal eosinophilic degeneration
8	Seizure disorder (epilepsy), headache, and acute confusional state.	6 months	-heart block with cardiopulmonary arrest -myocarditis -diffuse ischemic encephalopathy	-moderate cortical atrophy -severe ventricular dilation -minimal focal white matter abnormalities -focal cerebellar atrophy -resolved intracerebral hemorrhage with cyst formation.	Brain 900 gms -right posterior hemisphere indented over a 5 cm area -posterior horn of right lateral ventricle cystically dilated -cyst lined with cortical tissue with gliosis and hemosiderin -laden macrophages -single focus of cortical atrophy in cerebellum with complete drop-out of Purkinje cells and disruption of the granular cell layer
9	Acute confusional state	2 weeks	-systemic lupus erythematosus -pneumonia sepsis.	-minimal cortical atrophy -minimal ventricular -dilation -few focal white matter abnormalities	Brain 1250 gms -diffuse ischemic changes -no obvious focal lesions
10	Acute confusional state	5 months	-atherosclerosis acute myocardial infarction -ischemic bowel -sepsis	-minimal cortical atrophy -minimal ventricular dilation -septa in lateral ventricles -moderate focal white matter abnormalities	Brain 1250 gms -atherosclerosis of the basilar, carotid, and anterior cerebral arteries -choroid plexus cysts in lateral ventricle -few small areas of resolved infarction.
11	Acute confusional state, and lupus headache.	3 weeks	-systemic lupus erythematosus -multiple cerebral infarctions -sepsis.	-severe cortical atrophy -ventricular dilation -acute focal white matter lesions -chronic diffuse white matter abnormalities -cerebral edema.	Brain 950 gms -frontal cortical atrophy -small lesions (<0.5 cm) consistent with acute infarcts in frontal lobe cortex, caudate nucleus, and right parietal white matter with peripheral zones of edema and necrotic core with macrophages, gliosis, swollen axons -moderate gliosis of the thalami -diffuse loss of Purkinje cells.
12	Headache. seizure disorder, and acute confusional state.	1 week	-intracranial hemorrhage -cerebral edema with tonsillar herniation -diffuse ischemic encephalopathy -active systemic lupus erythematosus hypertensive encephalopathy -tonsillar herniation.	-no cortical atrophy -no ventricular dilation -minimal focal white matter abnormalities -acute intracerebral hemorrhage -cerebral edema.	Brain 1230 gms -brain grossly edematous -right to left subfalcian herniation -cerebellar tonsillar herniation -subarachnoid hemorrhage -large intraparenchymal hemorrhages in the right corpus striatum and in the left thalamus -mild mononuclear perivascular inflammation -perivascular siderophages -perihematoma satellite hemorrhages.
13	Acute confusional state, seizure disorder, and headache.	6 months	-pneumonia -sepsis -diffuse ischemic -encephalopathy active systemic lupus -erythematosus.	-minimal cortical atrophy -no ventricular dilation -focal white matter changes	Brain 1380 gms -histopathology unremarkable except for generalized bland ischemic changes.
14	Status epilepticus Brain death	1 week	-systemic lupus erythematosus -status epilepticus -cerebral edema -diffuse ischemic encephalopathy	-moderate cortical atrophy -minimal dilation -minimal focal white matter changes -acute -leukoencephalopathy in putamen, pons, cerebellum -generalized cerebral edema	Brain 1100 gms -cerebra edema -multifocal areas of loss of neurons with gliosis and microglial proliferation -myelinated fibers reduced in number -hemorrhages in basal ganglia -bland ischemic cellular injury