Collagen diseases are often complicated by cerebrovascular disorders; however, these rarely occur in RA [4]. The rate of occurrence of cerebral vasculitis in patients with RA is 1–8% [5].
The most frequent neurological complications with RA are atlantoaxial subluxation, polymyositis, mononeuritis multiplex, peripheral neuropathy, rheumatoid nodules within the central or peripheral nervous system, and RV [6].They were initially described in seropositive patients with long history of active disease, with subcutaneous nodules and extra-articular manifestations [7].
Our case was seronegative and had a long history of active RA, but no previous extra-articular manifestations.
The neurological manifestations of cerebral RV include hemiparesis, focal onset epilepsy, cranial nerve palsy, visual field impairment, altered consciousness, and cognitive impairment [8, 9].
The neurological manifestations in our case were headache and receptive aphasia due to the occurrence of intracerebral hemorrhage in absence of other risk factors for hemorrhage.
Two previous studies evaluated various autoimmune diseases and the risk of ICH. Both studies found that the danger of ICH is higher in patients with RA, with the highest risk occurring during the first year of diagnosis [10]. In a meta-analysis of 23 studies, it was found that patients with RA have a risk of hemorrhagic stroke 1.68 times higher than that in normal individuals [11].
Moyamoya disease is a rare neurovascular disorder of unknown etiology characterized by progressive stenosis or occlusion of the supraclinoid portion of both internal carotid arteries and development of collateral vessels. The classical presentations of the disease in children are transient ischemic attacks and seizures, whereas in adults, cerebral ischemia and cerebral hemorrhage are the commonest manifestations [12].
Moyamoya syndrome or secondary moyamoya disease has similar angiographic findings as primary moyamoya disease. Moyamoya syndrome can be seen in patients with cerebral vasculitis, fibromuscular dysplasia, sickle cell disease, and advanced intracranial atherosclerosis. The incidence of moyamoya syndrome in patients with RA is unclear due to lack of sufficient data [13].
Our case showed moyamoya syndrome angiographic pattern with DSA, denting chronic intracranial vasculitis. Also, intracerebral hemorrhage was the presenting manifestation as reported in the literature.