Moyamoya disease with Sjogren disease and autoimmune thyroiditis presenting with left intracranial hemorrhage after messenger RNA-1273 vaccination

Abstract

Rationale:The new vaccines are emergently authorized and currently approved for use to protect against the coronavirus disease 2019 (COVID-19) pandemic and serious adverse events are uncommon. Moyamoya disease (MMD) with autoimmune disease is a rare entity and usually presents with intracranial hemorrhage in adults.Patient concerns:We reported a 40-year-old female patient with Sjogren disease and autoimmune thyroiditis, who had received the second dose of Moderna (mRNA-1273) vaccination. Three days later, she presented with left intraventricular and intracerebral hemorrhage as a complication.Diagnosis:After a series of diagnostic workups, left intracranial hemorrhage was associated with MMD.Interventions:Emergent external ventricular drainage and subsequent stereotactic evacuation of hematoma with insertion of intracranial pressure monitoring were performed.Outcomes:Under the care of the neurocritical care team, her physical condition improved gradually. The neurological sequelae was noted by defects of cognitive function, apraxia, agnosia, and impaired executive function. She was discharged after eight weeks with a follow-up in the vascular neurology clinic planning for performing revascularization.Lessons:To the best of our knowledge, no similar case has been reported before, and this is the first case of MMD complicated with intracerebral and intraventricular hemorrhage after mRNA-1273 vaccination. It is noticeable to assess the vaccine safety surveillance and raise the alertness about moyamoya in patients with autoimmune diseases during the COVID-19 pandemic. Further studies for risk evaluation of COVID-19 vaccines in patients with autoimmune diseases might be required in the future.

Clinical value of the systemic immune-inflammation index in moyamoya disease

“…Although little is known about the biological function of RNF213 or its related pathogenesis in moyamoya disease, the identification of RNF213 as an immunosensor reveals a clear molecular link between MMD and infection ( 31 , 36 , 37 ). In recent years, increasing reports have been made about the coexistence of moyamoya disease and immune diseases, such as Graves' disease and systemic lupus erythematosus ( 38 – 41 ). In the United States and China, patients with moyamoya disease combined with autoimmune diseases have been reported to have a higher prevalence than the general population, but the exact reason is unclear ( 42 , 43 ).…”

Neurological Adverse Reactions to SARS-CoV-2 Vaccines

“…There was only moderate thrombocytopenia but PF-4 antibodies were elevated [22]. In a 40-yearold female with Moyamoya disease, Sjögren syndrome, and autoimmune thyroiditis, application of the second MOV dose was complicated by left ICH and intraventricular bleeding 3 days after SC2V [23]. The patient benefited from an external ventricular drainage and subsequent stereotactic evacuation of the hematoma [23].…”

“…In a 40-yearold female with Moyamoya disease, Sjögren syndrome, and autoimmune thyroiditis, application of the second MOV dose was complicated by left ICH and intraventricular bleeding 3 days after SC2V [23]. The patient benefited from an external ventricular drainage and subsequent stereotactic evacuation of the hematoma [23]. A 46-year-old female developed ICH due to vasculitis following the first BPV dose [24].…”

What Should We Do after the COVID-19 Vaccination? Vaccine-Associated Diseases and Precautionary Measures against Adverse Reactions

“…Moreover, IL-6 is also involved in producing anti-aquaporin-4 antibodies, and it has been reported that IL-6 has a higher level in NMOSD [72]. Intracerebral hemorrhages after vaccination due to central venous sinus thrombosis [68], vasculitis [61], and Moyamoya disease with Sjogren disease [73] have been reported. In cases of cerebral hemorrhage after COVID-19 vaccination, thrombosis, vasculitis, and autoimmune diseases should also be considered.…”