Patient no/sex/age | Diagnosis | Disease duration (y) | SLEDAI*score | Actual CNS symptoms | Treatment | PET findings (hypometabolism >1 SD below controls) | MRI findings |
Severe neurospychiatric symptoms | |||||||
1/f/20 | SLE | 8 | 29 | headache, depression, memory impairment | S,A | parieto-occipital B, occipito-frontal B, ↑ metabolism caudate nucleus B | Normal |
2/m/54 | SLE | 1 | 0 | severe impairment of memory and concentration, amaurosis fugax attacks, oscillopsia | none | parieto-occipital B, parietal R | Mild atrophy |
3/f/22 | SLE | 1 | 15 | psychomotorous unrest, obsessive-compulsive neurosis, temporal lobe epilepsy due to neurocytoma right (state after brain surgery) | S | parieto-occipital L, parietal R>L, frontal B, basal ganglia R, temporal R (surgery) | Mild atrophy, defect temporal R (surgery) |
4/f/53 | SLE, TTP, sAPL | 11 | 13 | stroke on the right, vertigo, hypogeusia | S,bC (n=2), PL | parieto-occipital R, parietal R, basal ganglia R, cerebellar L (diaschisis) | Multiple infarcts parieto-occipital R, temporal R, cerebellar R, multiple SFL-R>L (10 mm), moderate atrophy |
5/f/46 | SLE | 0.5 | 17 | vertigo, gait disturbance, headache, depression | S,bC (n=1) | parieto-occipital R, praecentral R, latero-frontal L, occipital L, cerebellar B | Multiple infarcts parieto-occipital,frontal -R, multiple SFL-L (2 mm), moderate atrophy |
6/f/36 | SLE | 0.3 | 18 | headache, vertigo, relapsing diplopia | S | parieto-occipital B | Normal |
7/f/37 | SLE | 10 | 21 | memory impairment, common migraine, depression, paraesthesia | S,I | parieto-occipital B | Multiple SFL-R,L (5 mm) |
8/f/71 | SLE | 2 | 37 | hypomanic syndrome, impairment of attention, mental aberration | S,bC (n=1) | parieto-occipital B | Normal |
9/f/39 | SLE, sAPL | 3 | 5 | diplopia | none | parieto-occipital B, parietal B | Mild atrophy |
10/f/31 | SLE | 1 | 23 | vertigo, disorder of eye movement, acute confusional state | S,I | parieto-occipital B | Normal |
11/f/29 | SLE | 5 | 0 | athymia, history of infarction 4 years ago | S,H | patchy uptake pattern, parieto-occipital B, parietal B, temporal B | Multiple infarcts temporal R, multiple SFL frontal, temporo-occipital L |
12/f/32 | SLE | 1 | 10 | visual disturbance | none | parieto-occipital B, frontal R, anterofrontal R | Multiple confluent SFL fronto-parietal R>L |
Mild neuropsychiatric symptoms | |||||||
13/m/30 | SLE | 0.1 | 29 | headache | S,bC (n=1) | parieto-occipital B | Normal |
14/f/20 | SLE | 0.3 | 32 | headache | none | parieto-occipital B, temporal B, cerebellar B | Multiple SFL-L (4 mm) |
15/m/35 | SLE | 0.3 | 22 | headache, impairment of concentration | S | parieto-occipital B, cerebellar B | ND |
16/f/31 | SLE | 11 | 4 | reactive depression, anxiety, vertigo | S,oC | parieto-occipital B, parietal B | Normal |
17/f/41 | SLE | 7 | 1 | impairment of concentration | S,oC | parieto-occipital B | Mild cerebellar atrophy |
18/f/27 | SLE | 1.8 | 6 | memory impairment | S,A | parieto-occipital L>R | Normal |
19/f/35 | SLE | 4 | 14 | common migraine | S | parieto-occipital B | Normal |
20/f/34 | SLE | 2.7 | 23 | headache | S,bC (n=6) | parieto-occipital B, parietal B | Normal |
21/f/32 | SLE | 3 | 6 | impairment of attention, tinnitus | S | parieto-occipital B | Normal |
22/f/47 | SLE | 8 | 8 | common migraine, scintilating scotoma, impairment of memory | S | parieto-occipital B | Multiple SFL-R>L (2 mm) |
23/f/42 | SLE | 15 | 6 | impairment of memory | S,H | parieto-occipital R, parietal B | Normal |
No neuropsychiatric symptoms | |||||||
24/f/53 | SLE | 28 | 0 | none | S | parieto-occipital L | Normal |
25/f/18 | SLE | 5 | 0 | none, history of impairment of concentration 3 years ago | H | cerebellar B | Few SFL-L (2 mm) |
26/m/70 | SLE | 19 | 4 | none | S | normal | ND |
27/f/45 | SLE | 6 | 11 | none | S | normal | Normal |
28/f/37 | SLE | 4 | 8 | none | S,oC | normal | Normal |
S = corticosteroids, bC = bolus cyclophosphamide (number of boli), oC = oral cyclophosphamide, A = azathioprine, H = hydroxychloroquine/chloroquine, PL = plasmapheresis, I = intravenous immunoglobulins, L = left, R = right, B = both, PET = positron emission tomography, MRI = magnetic resonance imaging, ND = not done, SFL = a small focal area of increased signal on T2 weighted images present in white matter, TTP = thrombocytic thrombocytopenic purpura, SLE = systemic lupus erythematosus, sAPL=secondary anti-phospholipid antibody syndrome. *SLEDAI score (maximum possible score: 105).