Objectives: Clinical involvement of the nervous system is uncommon during sarcoidosis. Cerebrovascular events are rarely reported during sarcoidosis and may be confused with primary angiitis of the central nervous system. The characteristics and outcomes of cerebrovascular events during sarcoidosis have not been well-evaluated.
Patients and methods: We conducted a retrospective case series in our institution with an updated review of the literature from 1962 to 2017 to characterize the clinical presentation, imaging, histology and outcomes of stroke that were thought to be causally related to sarcoidosis.
Results: Fifty-one patients (31 men, median age at stroke diagnosis of 41 years) receiving a diagnosis of stroke and sarcoidosis, including 8 new cases from our institution and 43 from the literature review, were included. Stroke was the first manifestation of sarcoidosis in 64%. The stroke was ischemic in 69% and hemorrhagic in 31%. A total of 31% experienced a transient ischemic attack before ischemic stroke. A total of 36% of patients had multiple infarcts or hemorrhages. The median number of cardiovascular risk factors was 0. Concerning histological presentation, vascular or perivascular involvement was present in all cases. After a median follow-up of 12 months (after stroke), up to 50% of patients had developed a permanent neurological impairment, 16% had a recurrence of stroke, and 23% died.
Conclusions: Cerebrovascular events may be the first manifestation of neurosarcoidosis, and have a prognosis impact in such patients, leading to death and permanent neurological impairment.
Keywords: Brain ischemia; Cerebral venous thrombosis; Intracranial hemorrhages; Sarcoidosis; Stroke.