Moyamoya syndrome causing stroke in young women with type 1 diabetes

Abstract

Context Moyamoya syndrome is an idiopathic brain vasculopathy characterized by stenosis of major intracranial arteries. It often presents in patients with type 1 diabetes or thyroid disease and may have an autoimmune etiology. Moyamoya-related stroke poses a diagnostic challenge as initial symptoms and deficits vary greatly from classic ischemic stroke to encephalopathy, psychiatric, or seizure disorder. Case Description We report 4 patients with type 1 diabetes and other autoimmune diseases who developed moyamoya-related stroke at a young age. Despite having long-term diabetes, these patients exhibited no evidence of dyslipidemia or other typical risk factors for atherosclerosis which might contribute to premature stroke. Three of the four patients underwent revascularization surgery while one patient received conservative management. All patients had improved neurologic function after treatment, some with residual deficits. Conclusion We highlight the importance of recognizing moyamoya syndrome in patients with pre-existing autoimmune diseases such as type 1 diabetes, as prompt diagnosis and treatment can have major impact on patient outcome and quality of life.

rAAV8-733-Mediated Gene Transfer of CHIP/Stub-1 Prevents Hippocampal Neuronal Death in Experimental Brain Ischemia

“…In addition, our data suggest that an rAAV8-733-CHIP vector qualifies as a potential candidate for neuroprotective gene therapy for chronic ischemic conditions in the brain, such as brain obstructive chronic vascular diseases. 34 …”

Od akutne psihoze do Moyamoya sindroma

“…Od Moyamoya bolesti obolevaju mlađe osobe, često još u detinjstvu, a odnos žene : muškarci su 1.8 : 1. Starosna dob pri postavljanju dijagnoze kreće se od 6 meseci do 67 godina, ali je najveći pik obolevanja u prvoj deceniji, sa dva manja pika u 3. i 4. deceniji života (1,4).…”

“…Dodatni dokaz o potencijalnoj ulozi autoimunog procesa u Moyamoya oboljenju je prisustvo cirkulišućih antitela u serumu obolelih, pre svega antiendotelijalna antitela, koja mogu potencijalno razjasniti kako autoimuni inflamatorni proces može rezultovati oštećenjem endotela. Ostala opisana antitela su CAMK2A, CD79A, i EFNA3, ali i tiroidna autoantitela koja najverovatnije ukršteno reaguju sa antigenima cerebralnih arterija, prouzrokujući autoimuni arteritis (1,2,4,5). Dodatno, u bolesnika sa Moyamoya oboljenjem opisan je i značajno viši nivo lipidnih parametara u odnosu na opštu populaciju.…”

“…Razlog za to ostaje nejasan, iako je poznata povezanost dislipidemije i okluzivne vaskularne bolesti. Svakako, ateroskleroza nastala u uslovima hiperlipidemije se ne može u potpunosti isključiti kao potencijalni uzrok Moyamoya oboljenja (1,4,5).…”

“…Stopa mortaliteta iznosi oko 10% u odraslih i 4.3% u dece. Oko 50-60% obolelih osoba ima kognitivno slabljenje, kao posledica multiinfarkta mozga (1,5,6).…”