Table 5.

Cases of cerebral vasculitis in neurosarcoidosis from our hospital records included in this study

Age/Sex	Neurologic manifestation	Imaging	Diagnostic work-up	Follow-up and Immunosuppressive treatment
No.1 36 yo female	Sensory symptoms Gait disturbance	2017 CT: acute infarcts of the brainstem and the right occipital lobe DSA: multiple microaneurysms of the pontine perforators 2018 MRI: new infarcts of the brainstem and the right occipital lobe, leptomeningeal contrast-enhancement cerebral as well spinal 2019 MRI: infarct of the left occipital lobe, new leptomeningeal contrast-enhancement	CSF: lymphocytic pleocytosis (68 /µl), protein ↑, OCB typ IV, CSF lysozyme and neopterin ↑ Antineural antibodies neg., laboratory screening for systemic vasculitis neg Event recorder since 07/2017: no atrial fibrillation HRCT of the thorax: Biliary and pulmonary lymphadenopathy Bronchoscopy + BAL + lymph node biopsy 2018 confirmed sarcoidosis CVRF: obesity, hypercholesterolemia	2017 + 2018 + 2019 Recurring ischemic strokes 2018 → high-dose IV GC, followed by oral GC tapering  + RTX + MTX for further GC tapering 2019 Persisting CSF pleocytosis in follow-up lumbar puncture and new leptomeningeal contrast-enhancement in MRI despite treatment with oral GC + RTX  → High-dose IV GC + IFX 2020 Improving CSF und MRI results under RTX, IFX and low-dose oral GC 2021 Persisting CSF pleocytosis, steady MRI results  → MTX reintroduced 2022 CSF unremarkable, steady MRI + DSA results
No. 2 44 yo male	Uveitis anterior Left-sided hemiparesthesia Motor impairment of the left hand	01/2023 MRI: acute right-sided thalamic infarct 02/2023 DSA: bilateral caliber irregularities of MCA, ACA and PCA 02/2020 Spinal MRI: unremarkable	CSF lymphocytic pleocytosis (142/ul), protein ↑, OCB typ IV, CSF lysozyme and neopterin ↑ Antineural antibodies neg., laboratory screening for systemic vasculitis neg HRCT of the thorax and abdomen: no sign of systemic sarcoidosis CVRF: smoking	01/2023 Uveitis anterior 01/2023 thalamic stroke  → Oral GC tapering + IFX 02/2023 Improving CSF pleocytosis (32/µl) 08/2023 Frontoparietal brain edema in MRI, progressive angiographic abnormalities and increasing CSF pleocytosis (67/µl) despite oral GC + IFX  → RTX 04/2024 MRI regression of the brain edema, CSF unremarkable
No. 3 60 yo female	Transitory left-sided hemianopsia Gait disturbance Headache Cognitive impairments	01/2020 MRI: leptomeningeal contrast-enhancement, lacunary post-ischemic abnormalities of the right corona radiata 01/2020 DSA: Occlusion of proximal right MCA and left PCA, caliber irregularities of the left MCA, moyamoya-like 01/2020 Spinal MRI: unremarkable	CSF lymphocytic/ monocytic pleocytosis (14/µl), protein slightly ↑, OCB typ IV, CSF lysozyme and neopterin ↑, Antineural antibodies neg., laboratory screening for systemic vasculitis neg., dsDNA-antibodies slightly ↑, Lupus anticoagulant and anti-cardiolipin-antibodies neg., Serum sIL-2R ↑ HRCT of the thorax: Billiary and pulmonary lymphadenopathy Hepatic tissue biopsy 2011 confirmed sarcoidosis CVRF: hypercholesterolemia	01/2020 cognitive impairments, headache and gait disturbance  → High-dose IV GC, followed by oral GC tapering 02/2020 TIA of the right PCA  → IFX + oral GC → improving CSF and steady MRI results 06/2022 focal epileptic seizures → anti-epileptic medication
No. 4 48 yo female	Vertigo Headache Left-sided hemihypesthesia Dysarthria Paraparesis Bladder dysfunction	10/2021 MRI: acute lacunary stroke of the Medulla Oblongata, old postischemic lesions of brain stem and semioval center 11/2021 spinal MRI: spinal lesion Th 10–12 with contrast-enhancement DSA: microaneurysm of the pontine perforators	CSF lymphocytic/ monocytic pleocytosis (11/µl), protein slightly ↑, OCB typ IV, CSF neopterin ↑, Antineural antibodies neg., MOG- AQP4-antibodies neg., Serum sIL-2R ↑ HRCT of the thorax + ultrasound of the abdomen: no sign of systemic sarcoidosis CVRF: smoking	05/2021 TIA of the brain stem 10/2021 ischemic stroke of the medulla oblongata 11/2021 transverse myelitis Th 10–12  → High-dose IV GC + 4 courses of plasmapheresis followed by oral GC tapering + IFX

ACA anterior cerebral artery, BAL bronchoalveolar lavage, CSF cerebrospinal fluid, CT computed tomography, CVRF cardiovascular risk factors, CYC Cyclophosphamide, DSA digital subtraction angiography, GC glucocorticoids, HRCT high-resolution computed tomography, IFX Infliximab, IV intravenous, MCA middle cerebral artery, MRI magnetic resonance imaging, MTX Methotrexate, OCB oligoclonal bands, PCA posterior cerebral artery, RTX Rituximab, sIL-2R soluble interleukin-2 receptor, TIA transitory ischemic attack