Table 2.

Patient	Age (years)	Concurrent Autoimmune Disease	Level of Suspicion for NPSLE	NPSLE Syndrome(s)	Time Interval from Initial NP Symptom Onseta	Time Interval from Last New or Worsening NP Symptom*	SLEDAI-2K Score (Without Neurologic Sub-Scores)	Pertinent Neurodiagnostic Testing	Blood, CSF NfL (pg/ml)	Blood, CSF GFAP (pg/ml)
1	15	Sjogren’s	Definite	Autonomic disorder, cranial neuropathy	<1 month	<1 month	11	MRI: Mild cerebral/cerebellar atrophy, pachymeningitis CSF: Elevated CSF protein and unique CSF OCBs	7.4 257.0	110.3 2,212.4
2	12	None	Definite	Demyelinating disorder, myelopathy	<1 month	<1 month	13	MRI: Longitudinally extensive transverse myelitis, grey-matter predominant CSF: Low glucose, elevated protein, unique CSF OCBs, and IgG index Blood: Anti-AQP4 antibody neg	65.1 8,483.0	472.8 22,218.3
3	15	Sjogren’s	Definite	Seizure disorder, moderate/severe cognitive dysfunction, mild mood disorder	1–2 years	<1 month	10	MRI: Normal CSF: Mild pleocytosis EEG: Diffuse and focal temporal slowing, bitemporal spikes	13.3	75.2
4	15	None	Definite	Aseptic meningitis, moderate/severe cognitive dysfunction, mild mood disorder	<1 month	<1 month	10	MRI: Normal CSF: Moderate pleocytosis, elevated protein	449.3 8,543.0	784.5 88,023.8
5	51	APLSb	Probable	Stroke (ischemic, hemorrhagic), mild mood disorder	6–12 months	<1 month	12	MRI: Multifocal cerebral white matter and cortical damage, with microhemorrhages. Small focus of parenchymal enhancement. MRA: Luminal irregularities of the intracranial arteries, multifocal.	34.0 1,908.1	178.7 9,443.5
6	23	APLSb	Definite	Stroke (ischemic)	6 months	6 months	2	MRI: Multifocal areas of acute infarction and microhemorrhages in the brain MRA: Luminal irregularities of the intracranial arteries, multifocal.	144.1 2,495.9	211.0 6,060.4
7	30	None	Definite	Demyelinating disorder	<1 month	<1 month	4	MRI: T2 hyperintensity in dorsal medulla CSF: Low glucose, +antineuronal antibodies Blood: Anti-AQP4 antibody neg.	29.9 1,534.6	112.4 9,800.6
9	55	Sjogren’s	Probable	Autonomic disorder, polyneuropathy, moderate/severe cognitive dysfunction	5–6 years	<1 month	2	MRI: Normal CSF: Elevated unique CSF OCBs EMG/NCS: Sensorimotor polyneuropathy	11.9 612.8	80.0 8,588.6
10	24	APLS, Sjogren’s	Probable	Aseptic meningitis, sensory neuropathy, optic neuritis	<1 month	<1 month	5	MRI: Normal (orbits not done) CSF: Elevated protein and IgG Index Blood: Anti-AQP4 neg EMG/NCS: Normal	9.0	13.1
11	37	Sjogren’s	Definite	Headache, stroke (ischemic)	<1 month	<1 month	4	MRI: Multifocal acute and subacute infarcts CSF: Mild pleocytosis, borderline IgG index	29.5	16.9
13	20	None	Probable	Headache, stroke (hemorrhagic), mild cognitive dysfunction	2–3 months	1–2 months	12	MRI: Small subarachnoid hemorrhage CSF: Elevated unique CSF OCBs	5.8	53.9
14	18	APLS	Definite	Stroke (ischemic), mononeuritis multiplex	2–3 months	<1 month	6	MRI: Multifocal acute and chronic infarcts EMG/NCS: Sensory greater than motor axonal changes, asymmetric	245.6	276.7
15	33	None	Definite	Headache, transverse myelitis	3–4 months	3–4 months	2	MRI: Longitudinally extensive, patchy T2 cord signal abnormality CSF: Elevated opening pressure, low CSF glucose, elevated CSF protein and neutrophilic pleocytosis Blood: Anti-AQP4 antibody neg	51.7	112.4

^aUntil the time of sampling.

^bDiagnosed at time of presentation by presence of strokes and positive APLAs. AQP4 = aquaporin-4; APLA = antiphospholipid antibody; APLS = antiphospholipid antibody syndrome; CSF = cerebrospinal fluid; EEG = electroencephalogram; EMG/NCS = electromyography/nerve conduction studies; GFAP = glial fibrillary acidic protein; IgG = immunoglobulin G; MRA = magnetic resonance angiography; MRI = magnetic resonance imaging; neg = negative; NfL = neurofilament light; NP = neuropsychiatric; NPSLE = neuropsychiatric systemic lupus erythematosus; OCB = oligoclonal bands; SLEDAI = systemic lupus erythematosus disease activity index.