Moya-Moya like vasculitis secondary to sarcoidosis: a rare case report of concomitant ischemic infracts and sub-arachnoid hemorrhage

Abstract

Abstract Background : Sarcoidosis is a rare idiopathic systemic inflammatory disease that mainly affects the lymph nodes, lungs, skin and eyes. Nervous system involvement is only seen in 5% of cases of sarcoidosis. Cerebral vasculitis, is particularly uncommon in patients with sarcoidosis, with serious outcome. Case presentation : We reported the case of a 55 years-old female who developed a severe hemicranial headache with nausea and vomiting. She had a history of erythema nodosum. The clinical examination showed left arm weakness and central facial paralysis, along with brisk tendon reflexes and arthralgia. Brain MRI showed a moderate subarachnoid hemorrhage, multiple acute lacunar infracts, and a large ischemic stroke over the right superficial sylvian territory; and MR angiography revealed a Moya-Moya like vasculopathy pattern and the occlusion of intracranial internal carotid arteries. Extensive laboratory workup including infectious serologies and vasculitis screen was negative; and Angiotensin-converting enzyme levels were normal. Findings in the CT chest and abdomen scan, and Broncho alveolar lavage confirmed the diagnosis of sarcoidosis. Conclusions : Cerebrovascular manifestation are uncommon in sarcoidosis. Multiple factors seem to be involved in the pathogenesis of sarcoidosis related vasculopathy. The diagnosis should be considered when recurrent infracts and hemorrhage are encountered in a patient having systemic symptoms of sarcoidosis.