Systemic lupus erythematosus | Depression (39%), anxiety (13%), psychosis (5–7%), mood disorder with mixed features (4%) | Memory impairment (43%), inattention (26%), visuospatial processing (26%), decreased psychomotor speed (26%), encephalopathy (7%) | Cutaneous manifestations (e.g., malar rash), oral ulcers, alopecia, synovitis, serositis, renal injury (e.g., proteinuria), low cell counts (anemia, leukopenia, and/or thrombocytopenia), seizures, neuropathy (cranial or peripheral), myelitis, fatigue, vision loss (e.g., due to optic neuritis, retinopathy, vitreous hemorrhage), arterial strokes |
Sjögren’s syndrome | Hypochondriasis (91%), depression (48%), behavior/personality changes (33%), somatization (33%), dissociation (23%), anxiety/panic (12.5%), hypomania followed by depression (5%), hypomania (2.5%), paranoia (2%) | Aphasia (5%), encephalopathy (2.4%) | Peripheral neuropathy, stroke- or multiple sclerosis-like symptoms, sicca (including dry mouth, dry eyes), enlarged parotid glands |
Behçet’s disease | Behavior/personality changes (30%; of these, one-third with apathy and two-third with disinhibition), anxiety (24%), mixed psychiatric disorder (21%), depression (21%), mania (6%), hypersomnia (3%), psychosis (3%) | Memory impairment (88%, learning and recall more impaired than recognition, both visual and verbal impairment), inattention 60%, executive dysfunction (52%), visuospatial dysfunction (9%), abstraction (8%), aphasia (<5% including 1% anomia) | Oral/GI and genital ulcers, erythema nodosum, arthritis/arthralgia, uveitis, venous and arterial thrombi, ophthalmoparesis, cranial neuropathy, cerebellar dysfunction, extrapyramidal dysfunction, myelopathy, hemiparesis, hemisensory loss, seizures |
Susac’s syndrome | Emotional disturbance (16%), behavior/personality changes (12–15%), apathy (12%), psychosis (10%) | Acute encephalopathy (76%), cognitive impairment (48%, usually loss of memory, attention, and executive function), decreased level of consciousness (9%) | Branch retinal arterial occlusion (BRAO), hearing loss |
CNS vasculitis | Behavior/personality changes (up to 95% in small vessel pediatric cohort, otherwise N/A) | Aphasia (28–84%, including language production and/or comprehension), decreased level of consciousness (47%), amnestic syndrome (9%) | Prodromal period lasting months, headache, focal neurologic signs (e.g., hemiparesis or ... |