Table 1: (a) Clinical, imaging, EMG, and EEG characteristics of individual APS− NPSLE patients recorded at the time of the initial NP manifestation. (b) Clinical, imaging, EMG, and EEG characteristics of individual APS+ NPSLE patients recorded at the time of the initial NP manifestation.

(a)

ID Age of the patient at the time of NP event (years) Interval between NP event and SLE diagnosis (years) Case definition Brain CT scan/brain MRI/EMG 1* 16 −2 Generalized seizure disorder, acute psychosis, and severe hemicraneal headache MRI: generalized cortical atrophy 2 11 0 Generalized seizure disorder Not available 3* 30 0 Generalized seizure disorder MRI: multiple focal deep white matter lesions hyperintense in T2-weighted images and FLAIR 4* 17 0 Generalized seizure disorder MRI: no abnormality 5* 42 −1 Generalized seizure disorder, severe headache, major depression MRI: subcortical high signal bifrontal, bilateral deep cortical, and corona radiata focal lesions in T2-weighted images. 6* 23 0 Generalized seizure disorder and ischemic stroke MRI: right cerebral atrophy. Left thalamic calcified lesion. 7 49 0 Ischemic stroke CT scan: ischemic infarct left hippocampus 8 31 1 Ischemic stroke CT scan: left lenticular haemorrhagic infarct 9 50 2 Ischemic stroke MRI: right ischemic parieto-temporal infarct 10 23 0 Peripheral neuropathy EMG: motor-sensitive neuropathy 11 17 1 Peripheral neuropathy EMG: motor-sensitive neuropathy 12* 45 0 Major depression, severe hemicraneal headache MRI: multiple focal deep white matter high signal on T2-weighted images and FLAIR. Small focal lesions in the subcortical fronto parietal white matter, predominantly on the right. 13* 29 2 Major depression with suicidal ideation, cluster headache MRI: multiple small focal subcortical white matter and right paramedian pontine high-signal lesions on T2-weighted images and FLAIR.

*Interictal EEG was performed in these patients. An epileptiform focus was detected in 3 (patients 3, 4, and 6) with no abnormality in the other 4 patients (patients 1, 5, 12, and 13). Nephritis was present in patients 3, 12, and 13 at the time of the NP event.

(b)

ID Age of the patient at the time of NP event (years) Interval between NP event and SLE diagnosis (years) ACR clinical entity APS defining event Brain CT scan/brain MRI/EMG 14 29 5 Ischemic stroke with hemorrhagic transformation Cerebral thrombosis MRI: subcortical right parietal ischemic infarct with hemorrhagic transformation 15 34 17 Ischemic stroke Peripheral artery thrombosis MRI: right parietal ischemic infarct 16 43 0 Ischemic stroke and 7 days later Guillain Barré Syndrome Cerebral thrombosis MRI: oval-shaped left subcortical paraventricular high-signal lesions on T2- and FLAIR-weighted images with a signal change in diffusion sequence and a left corona hemorrhagic infarct; EMG: motor-sensitive polyneuropathy 17 34 1 Peripheral neuropathy Coronary thrombosis EMG: motor-sensitive neuropathy 18 33 16 Peripheral neuropathy Repeated dialysis access thrombosis EMG: motor-sensitive neuropathy 19 38 −2 Major depression; ischemic optic neuropathy, Retinal artery thrombosis MRI: no abnormality 20* 43 14 Major depression Limb deep vein thrombosis and obstetric loss MRI: multiple focal subcortical lesions hyperintense in T2 21 28 0 Migrainous headaches and acute confusional state Cerebral thrombosis MRI: no abnormality 22* 35 14 Severe headaches and cognitive dysfunction Obstetric loss MRI: multiple focal deep white matter lesions hyperintense in T2 on T2-weighted images and FLAIR.

*Interictal EEG was performed in 2 patients (patients 20 and 22). Left temporal subclinical rhythmic electrographic discharges of adults—SREDA was detected in patient 22.