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[An autopsy case of moyamoya disease (author's transl)]
Abstract:
An autopsy case of severely progressed "Moya Moya" disease was presented. A 38-year-old male was admitted because of unconsciousness. On examination, he showed spider-like extremities, suggesting Marfan's syndrome. In the past history, he experienced acute rt. hemiparesis at 6 years old and generalized convulsion, lt. hemiparesis and aphasia at 7 years old. After these episodes, he spent an uneventful life until 38 years old, when he was found unconscious in his room by the family. Lt. CAG showed severe stenosis at the siphon of lt. ICA and fine vascular networks in the basal ganglia and many transdural collateral arteries. Three days later, he died and an autopsy was performed. On histological examination, proliferation of the intima, elongated elastica interna and lost of the media were identified in the stenotic arteries. In the vessels of other organs, especially of the heart, proliferated intima and lost of the media were also noticed. From these findings, "Moya Moya" disease was thought to be one of the manifestations of the systemic vascular diseases. The autopsy cases of "Moya Moya" disease in Japan were reviewed. According to the review, most of these autopsy cases died from intracerebral bleeding attack. Recently, EC-IC bypass operations have been performed in order to improve ischemic condition of "Moya Moya" disease. According to the finding of the review, however, the financial efficiency of such by-pass operations for "Moya Moya" disease must be elucidated after long-term follow-up study.
Insights
Moya Moya disease, a rare cerebrovascular condition, can manifest systemically, affecting organs beyond the brain. Autopsy findings reveal widespread vascular abnormalities, necessitating further research into treatment efficacy.
Area of Science:
- Neurology
- Pathology
- Vascular Biology
Background:
- Moya Moya disease is a rare, progressive cerebrovascular disorder.
- Systemic vascular involvement in Moya Moya disease is not fully understood.
Observation:
- A 38-year-old male with a history of neurological episodes presented with unconsciousness.
- Physical examination suggested Marfan's syndrome; cerebral angiography revealed severe internal carotid artery stenosis and collateral networks.
- Autopsy showed intimal proliferation and medial loss in stenotic arteries and other organs, including the heart.
Findings:
- Histological examination confirmed widespread vascular abnormalities consistent with systemic vasculopathy.
- Moya Moya disease may represent a manifestation of a broader systemic vascular disease.
- Review of Japanese autopsy cases indicated intracerebral hemorrhage as a common cause of mortality.
Implications:
- The systemic nature of Moya Moya disease requires consideration in diagnosis and management.
- Long-term follow-up studies are crucial to evaluate the financial efficiency and clinical benefit of treatments like EC-IC bypass surgery.