| Domain | Measure | Value(s) | Study/citation details |
|---|---|---|---|
| Epidemiology | Frequency among sarcoidosis patients | Clinically overt neurosarcoidosis in up to 10% of sarcoidosis patients | Ungprasert et al. 2022, *Expert Review of Neurotherapeutics*; DOI: 10.1080/14737175.2022.2108705; https://doi.org/10.1080/14737175.2022.2108705 (pqac-00000002) |
| Epidemiology | Frequency among sarcoidosis patients | About 5% of sarcoidosis patients develop neurosarcoidosis | Basheer et al. 2023, *Life*; DOI: 10.3390/life14010069; https://doi.org/10.3390/life14010069 (pqac-00000001) |
| Epidemiology | Frequency among sarcoidosis patients | 5.5% (85/1532) fulfilled Stern criteria for neurosarcoidosis | Ramos-Casals et al. 2021, *Scientific Reports*; DOI: 10.1038/s41598-021-92967-6; https://doi.org/10.1038/s41598-021-92967-6 (pqac-00000000) |
| Epidemiology | Systemic sarcoidosis coexistence | 86% in local cohort; 83% in literature review had systemic sarcoidosis | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000000) |
| Clinical phenotypes | Common presenting features | Cranial neuropathies 38%, motor deficit 32%, headache 16%, pituitary dysfunction 12% | Berntsson et al. 2023, *Scientific Reports*; DOI: 10.1038/s41598-023-33631-z; https://doi.org/10.1038/s41598-023-33631-z (pqac-00000000) |
| Clinical phenotypes | CNS distribution | Brain 38%, cranial nerves 36%, meninges 3%, spinal cord 10%, peripheral nerves 14% | Ramos-Casals et al. 2021, *Scientific Reports*; DOI: 10.1038/s41598-021-92967-6; https://doi.org/10.1038/s41598-021-92967-6 (pqac-00000000) |
| Clinical phenotypes | Initial manifestations in national cohort | Cranial nerve palsies 36%, medullary symptoms 23%, seizures 21% | Dos Santos et al. 2024, *Brain and Behavior*; DOI: 10.1002/brb3.3443; https://doi.org/10.1002/brb3.3443 (pqac-00000035) |
| Clinical phenotypes | Cognitive symptoms | Cognitive failure 16.9% overall; 24.3% in group 1, 13.8% in group 2 | Dos Santos et al. 2024, *Brain and Behavior*; DOI: 10.1002/brb3.3443; https://doi.org/10.1002/brb3.3443 (pqac-00000006) |
| CSF findings | Overall CSF abnormality | 77% abnormal CSF studies | Berntsson et al. 2023, *Scientific Reports*; DOI: 10.1038/s41598-023-33631-z; https://doi.org/10.1038/s41598-023-33631-z (pqac-00000000) |
| CSF findings | Specific abnormalities | Lymphocytosis 57%, elevated protein 44%, oligoclonal bands 40%, elevated CSF ACE 28%, raised CSF CD4+/CD8+ ratio 13% | Berntsson et al. 2023, *Scientific Reports*; DOI: 10.1038/s41598-023-33631-z; https://doi.org/10.1038/s41598-023-33631-z (pqac-00000000) |
| CSF findings | Local cohort lumbar puncture | 15/22 tested; abnormal in all patients | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000000) |
| CSF findings | Cohort workup | Lumbar puncture in 54; 42/54 abnormal (78%) | Berntsson et al. 2023, *Scientific Reports*; DOI: 10.1038/s41598-023-33631-z; https://doi.org/10.1038/s41598-023-33631-z (pqac-00000017) |
| CSF findings | Meta-analytic abnormality ranges | Pleocytosis in 32–63%; increased protein in 46–76%; meta-analysis: increased WCC 58%, elevated protein 63% | Vlad et al. 2023, *Frontiers in Neurology*; DOI: 10.3389/fneur.2023.1135392; https://doi.org/10.3389/fneur.2023.1135392 (pqac-00000018) |
| Imaging findings | Brain MRI abnormalities | Brain MRI abnormal in 16/21; parenchymal lesions 63%, hypothalamic-pituitary lesions 38%, meningeal enhancement 31% | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000000) |
| Imaging findings | Pituitary involvement | Isolated pituitary gland lesions in 17% | Berntsson et al. 2023, *Scientific Reports*; DOI: 10.1038/s41598-023-33631-z; https://doi.org/10.1038/s41598-023-33631-z (pqac-00000000) |
| Imaging findings | MRI sensitivity | MRI with gadolinium sensitivity 82–97% | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000004) |
| Imaging findings | MRI positivity in cohort | Brain MRI abnormal in 61/61; spinal MRI abnormal in 15/47 (32%) | Berntsson et al. 2023, *Scientific Reports*; DOI: 10.1038/s41598-023-33631-z; https://doi.org/10.1038/s41598-023-33631-z (pqac-00000017) |
| Imaging findings | MRI positivity in NS vs MS cohort | MRI abnormalities in 88.9%; leptomeningeal enhancement 59.3%; supratentorial 70.4%; infratentorial 44.4%; spinal 22.2%; normal MRI 11.1% | Vlad et al. 2023, *Frontiers in Neurology*; DOI: 10.3389/fneur.2023.1135392; https://doi.org/10.3389/fneur.2023.1135392 (pqac-00000015, pqac-00000019) |
| Imaging findings | FDG-PET/CT yield | FDG-PET/CT abnormal in 11/22 (50%) in one cohort | Berntsson et al. 2023, *Scientific Reports*; DOI: 10.1038/s41598-023-33631-z; https://doi.org/10.1038/s41598-023-33631-z (pqac-00000017) |
| Imaging findings | FDG-PET yield | FDG-PET/CT detected abnormalities in 85% in cohort | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000004) |
| Imaging findings | FDG-PET systemic lesion detection | Sarcoidosis-typical systemic lesions/abnormalities in 15/16 (93.8%) | Vlad et al. 2023, *Frontiers in Neurology*; DOI: 10.3389/fneur.2023.1135392; https://doi.org/10.3389/fneur.2023.1135392 (pqac-00000019) |
| Biomarkers | Serum routine markers in local cohort | CRP elevated 33%, calcemia 18%, lysozyme 79%, serum ACE 16% | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000000) |
| Biomarkers | Serum/CSF sIL-2R and ACE performance | Serum sIL-2R sensitivity 88%, specificity 85%; ACE sensitivity 62%, specificity 76% | Sinha et al. 2024, *Cureus*; DOI: 10.7759/cureus.69208; https://doi.org/10.7759/cureus.69208 (pqac-00000005) |
| Biomarkers | CSF ACE performance | CSF ACE cut-off 2: sensitivity 66.7%, specificity 67.3% | Sinha et al. 2024, *Cureus*; DOI: 10.7759/cureus.69208; https://doi.org/10.7759/cureus.69208 (pqac-00000005) |
| Biomarkers | CSF ACE in cohort | 19/35 abnormal; specificity ~94–95%, sensitivity 24–55% | Berntsson et al. 2023, *Scientific Reports*; DOI: 10.1038/s41598-023-33631-z; https://doi.org/10.1038/s41598-023-33631-z (pqac-00000017) |
| Biomarkers | sIL-2R systematic review size | 6 studies; 98 neurosarcoidosis, 525 non-sarcoidosis, 118 healthy controls | Chanpura et al. 2024, *Journal of Central Nervous System Disease*; DOI: 10.1177/11795735241274186; https://doi.org/10.1177/11795735241274186 (pqac-00000021) |
| Biomarkers | CSF sIL-2R cutoff performance | Cutoff 150 pg/mL: sensitivity 61%, specificity 93%, accuracy 0.83 | Chanpura et al. 2024, *Journal of Central Nervous System Disease*; DOI: 10.1177/11795735241274186; https://doi.org/10.1177/11795735241274186 (pqac-00000020) |
| Biomarkers | sIL-2R index | AUC vs multiple sclerosis 0.724; active disease median 32.45 vs remission 7.18 (P=0.0016) | Chanpura et al. 2024, *Journal of Central Nervous System Disease*; DOI: 10.1177/11795735241274186; https://doi.org/10.1177/11795735241274186 (pqac-00000020) |
| Biomarkers | sIL-2R frequencies in NS cohort | Serum elevated 10/25 (40.0%); CSF elevated 9/21 (42.9%); serum and/or CSF elevated 16/25 (64.0%) | Vlad et al. 2023, *Frontiers in Neurology*; DOI: 10.3389/fneur.2023.1135392; https://doi.org/10.3389/fneur.2023.1135392 (pqac-00000019) |
| Biomarkers | Neopterin in NS cohort | Serum and/or CSF elevated 19/22 (86.4%) | Vlad et al. 2023, *Frontiers in Neurology*; DOI: 10.3389/fneur.2023.1135392; https://doi.org/10.3389/fneur.2023.1135392 (pqac-00000019) |
| Biomarkers | Diagnostic likelihood ratios | PLR: WCC >30/µL = 7.2; QAlb >10×10⁻³ = 66.4; absence of CSF-specific OCB = 11.5; elevated CSF lactate = 23.0; elevated sIL-2R >8.0 | Vlad et al. 2023, *Frontiers in Neurology*; DOI: 10.3389/fneur.2023.1135392; https://doi.org/10.3389/fneur.2023.1135392 (pqac-00000016) |
| Biomarkers | Best combined CSF rule | Sensitivity and specificity each >92%; PLR 12.8; NLR 0.08 | Vlad et al. 2023, *Frontiers in Neurology*; DOI: 10.3389/fneur.2023.1135392; https://doi.org/10.3389/fneur.2023.1135392 (pqac-00000016) |
| Treatment outcomes | Methotrexate usage/outcome | Most frequently used second-line therapy (>45% of cases); favorable outcome in average 50% | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000000) |
| Treatment outcomes | TNF-α antagonist use | 9% in local cohort; 27% in literature review | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000000) |
| Treatment outcomes | Overall pooled favorable outcome | 282/354 (80%) favorable; 39/354 (11%) relapse/progression; 1/354 (0.3%) died | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000024) |
| Treatment outcomes | Infliximab meta-analysis | Pooled clinical improvement 74% (95% CI 64–84%) | Chaiyanarm et al. 2024, *Annals of Clinical and Translational Neurology*; DOI: 10.1002/acn3.51968; https://doi.org/10.1002/acn3.51968 (pqac-00000023, pqac-00000025) |
| Treatment outcomes | Infliximab adverse events | 52/177 patients (29.4%) had adverse events; infections were 72.2% of events | Chaiyanarm et al. 2024, *Annals of Clinical and Translational Neurology*; DOI: 10.1002/acn3.51968; https://doi.org/10.1002/acn3.51968 (pqac-00000025, pqac-00000026) |
| Treatment outcomes | Cohort infliximab outcome | Complete remission 6 (21%), improvement 14 (50%), stable disease 7 (25%), deterioration/death 1 (4%) | Basheer et al. 2023, *Life*; DOI: 10.3390/life14010069; https://doi.org/10.3390/life14010069 (pqac-00000027) |
| Relapse/discontinuation | TNF-α antagonist discontinuation | 70/223 (31%) discontinued | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000024) |
| Relapse/discontinuation | Relapse after stopping TNF-α antagonists | 14/28 (50%) relapsed after discontinuation | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000022, pqac-00000024) |
| Relapse/discontinuation | Infliximab pooled relapse | 38% (95% CI 22–55%) | Chaiyanarm et al. 2024, *Annals of Clinical and Translational Neurology*; DOI: 10.1002/acn3.51968; https://doi.org/10.1002/acn3.51968 (pqac-00000023, pqac-00000025) |
| Relapse/discontinuation | Relapse after infliximab reduction/discontinuation | 18/30 (60%) relapsed after stopping or reducing infliximab | Chaiyanarm et al. 2024, *Annals of Clinical and Translational Neurology*; DOI: 10.1002/acn3.51968; https://doi.org/10.1002/acn3.51968 (pqac-00000026) |
| Relapse/discontinuation | Corticosteroid taper/stop | 77/110 (70%) tapered or stopped corticosteroids | Sambon et al. 2022, *Frontiers in Neurology*; DOI: 10.3389/fneur.2022.970168; https://doi.org/10.3389/fneur.2022.970168 (pqac-00000024) |
| Clinical trials | Observational disease-course study | NCT04911348; retrospective multicenter cohort; enrollment ~80; follow-up ~6 years | ClinicalTrials.gov 2021; https://clinicaltrials.gov/study/NCT04911348 (pqac-00000030) |
| Clinical trials | ACTHAR trial | NCT02920710; Phase 4; withdrawn; actual enrollment 0; primary endpoint at 12 weeks | ClinicalTrials.gov 2019; https://clinicaltrials.gov/study/NCT02920710 (pqac-00000031) |
| Clinical trials | Advanced sarcoidosis registry | NCT03769987; observational registry; enrollment 800; primary endpoint death over 2 years | ClinicalTrials.gov 2017; https://clinicaltrials.gov/study/NCT03769987 (pqac-00000032) |
| Clinical trials | Biomarker diagnostic study | NCT05056740; observational retrospective biomarker study; enrollment 300; estimated completion 2025-11-01 | ClinicalTrials.gov 2020; https://clinicaltrials.gov/study/NCT05056740 (pqac-00000033) |


*Table: This table compiles explicit quantitative findings on neurosarcoidosis across epidemiology, phenotypes, diagnostics, biomarkers, treatment outcomes, relapse, and ongoing clinical research. It is useful as a quick reference for evidence-supported values and the studies from which they were drawn.*