Moyamoya disease and syndrome: a review.

Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

Moyamoya disease and syndrome: a review / Doença e síndrome de moyamoya: uma revisão

Abstract Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

RESUMO A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

Henrique Dumont: how a traumatic brain injury contributed to the development of the airplane.

The life and death of Henrique Dumont (1832-1892) is little known, being usually remembered as Alberto Santos-Dumont's father. This report describes the history of this Brazilian engineer, also known as the King of Coffee, who achieved enormous business success and fortune in the late nineteenth century. In 1890, during the inspection of his farm, the world's largest coffee plantation at that time, he fell from a carriage, which left him a hemiplegic. This forced him to sell the farm and move to France for treatment. Before his death two years later, he gave his 18-year-old son bits of advice, and distributed his inheritance, which allowed Alberto to study in Paris and finance his experiments that would culminate in the development of the airplane. The diagnostic and therapeutic possibilities are also discussed.

Henrique Dumont: how a traumatic brain injury contributed to the development of the airplane / Henrique Dumont: como um traumatismo cranioencefálico contribuiu para o desenvolvimento do avião

ABSTRACT The life and death of Henrique Dumont (1832-1892) is little known, being usually remembered as Alberto Santos-Dumont's father. This report describes the history of this Brazilian engineer, also known as the King of Coffee, who achieved enormous business success and fortune in the late nineteenth century. In 1890, during the inspection of his farm, the world's largest coffee plantation at that time, he fell from a carriage, which left him a hemiplegic. This forced him to sell the farm and move to France for treatment. Before his death two years later, he gave his 18-year-old son bits of advice, and distributed his inheritance, which allowed Alberto to study in Paris and finance his experiments that would culminate in the development of the airplane. The diagnostic and therapeutic possibilities are also discussed.

RESUMO A vida e especialmente a morte do brasileiro Henrique Dumont (1832-1892) são pouco conhecidas, sendo geralmente lembrado como pai de Alberto Santos Dumont. Relatamos a história deste engenheiro que obteve enorme sucesso empresarial no final do século XIX, sendo denominado Rei do Café. Em 1890, durante a inspeção da sua fazenda, maior cafeicultura do mundo na época, caiu da charrete e ficou hemiplégico. Isso o fez vender a fazenda e buscar tratamento na França, trazendo o primeiro automóvel do Brasil ao retornar. Antes de morrer dois anos após o acidente, deu valorosos conselhos e distribuiu sua herança ao filho Alberto, então com dezoito anos de idade, que pôde assim estudar em Paris e financiar seus experimentos que culminariam no desenvolvimento do avião. São discutidas e contemporizadas as possibilidades diagnósticas e terapêuticas relacionadas ao déficit neurológico.

Rescue therapy with stent retrievers for thromboembolism during endovascular treatment of intracranial aneurysms.

Thromboembolism is the most frequent complication in endovascular treatment of intracranial aneurysms, causing disability and death. As stent retrievers have achieved high rates of arterial recanalization in the management of ischemic stroke, these devices were tested as rescue therapy of thromboembolism during aneurysm embolization. We retrospectively analyzed 10 consecutive patients with transprocedural arterial occlusion, treated with mechanical thrombectomy at a single center. Good angiographic recanalization was achieved in eight cases, mTICI 3, 2b and 2a in five, three and two patients, respectively, without additional complications or any deaths. Five patients showed complete recovery (mRS 0) and all patients showed improvement of disability (average mRS 1.1) over a mean follow-up period of 31 months. Eight patients had good clinical recovery, while two remained with deficits (mRS 3 and 4). The study found that the stent retriever is a valuable, rapid and effective tool for restoring blood flow, improving the safety of endovascular treatment.

Rescue therapy with stent retrievers for thromboembolism during endovascular treatment of intracranial aneurysms / Terapia de resgate com stent para tromboembolismo durante tratamento endovascular de aneurismas intracranianos

ABSTRACT Thromboembolism is the most frequent complication in endovascular treatment of intracranial aneurysms, causing disability and death. As stent retrievers have achieved high rates of arterial recanalization in the management of ischemic stroke, these devices were tested as rescue therapy of thromboembolism during aneurysm embolization. We retrospectively analyzed 10 consecutive patients with transprocedural arterial occlusion, treated with mechanical thrombectomy at a single center. Good angiographic recanalization was achieved in eight cases, mTICI 3, 2b and 2a in five, three and two patients, respectively, without additional complications or any deaths. Five patients showed complete recovery (mRS 0) and all patients showed improvement of disability (average mRS 1.1) over a mean follow-up period of 31 months. Eight patients had good clinical recovery, while two remained with deficits (mRS 3 and 4). The study found that the stent retriever is a valuable, rapid and effective tool for restoring blood flow, improving the safety of endovascular treatment.

RESUMO Tromboembolismo é a complicação mais frequente no tratamento endovascular de aneurismas cerebrais, podendo causar morte ou sequelas. Como os stent retrievers alcançaram altas taxas de recanalização arterial no tratamento do acidente vascular encefálico isquêmico, testamos esses dispositivos para tratar eventos tromboembólicos ocorridos durante a embolização de aneurismas. Foram analisados retrospectivamente 10 pacientes apresentando oclusão arterial transoperatória, tratados com trombectomia mecânica em um único centro. Obtivemos recanalização angiográfica em oito casos, mTICI 3, 2b e 2a em cinco, três e dois pacientes, respectivamente, sem complicações adicionais ou óbito. Cinco casos apresentaram recuperação completa (mRS 0) e todos os pacientes apresentaram melhora dos déficits (mRS médio 1.1) durante acompanhamento médio de 31 meses. Oito pacientes apresentaram boa recuperação clínica, enquanto dois permaneceram com déficits (mRS 3 e 4). O estudo concluiu que stents são uma ferramenta valiosa, rápida e eficaz para restaurar o fluxo sanguíneo, aumentando a segurança do tratamento endovascular.