To describe a rare case of a patient with neurosarcoidosis complicated by systemic vasculitis of medium-large vessels.

Sarcoidosis is an inflammatory systemic disease characterized by the presence of noncaseating granulomas in any organ, when it affects the nervous system is called neurosarcoidosis. Systemic vasculitis is an unusual complication of sarcoidosis but it has not been previously reported in neurosarcoidosis.

A 62-year-old woman with a past medical history of hypertension, diabetes mellitus, coronary artery disease, neurosarcoidosis, s/p craniotomy (right cavernous sinus mass resection) was transferred to the hospital due to worsening headaches, generalized weakness, vomiting, and decreased oral intake for the last two weeks. On admission repeat brain MRI showed interval worsening of the known inflammatory right cavernous noncaseating granuloma, now extending into the right tentorial leaflet, right middle cranial fossa, and pituitary region, repeat vasculitis panel was negative, the patient was started on IV steroids and mycophenolate. However, the hospital stay was complicated by a syncopal episode, CT Head was normal but EKG showed a new T wave inversion with troponin elevation. Later on, the patient had worsening mentation and left-sided hemiparesis, CT head showed acute hypodensity in the right MCA territory and CTA showed bilateral stenosis of the distal right M1 and left M1segments of the MCAs, the patient was out of the window for tPA and was not a candidate for mechanical thrombectomy, she was started on aspirin. TTE and TEE did not show any intracardiac shunting/thrombi. Later on, she developed signs of ischemia in her right 1st and 2nd toes, aortogram showed partial and complete obstructions of both superficial femoral, anterior tibial, and peroneal arteries, and vascular surgery performed RLE SFA stent placement and recommended clopidogrel. IV cyclophosphamide was added with no further thromboembolic events.

Vasculitis although uncommon can also occur in patients with neurosarcoidosis.