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Classification according to ICD-10
I67.5 Moyamoya Syndrome
ICD-10 online (WHO version 2019)
On the left the MR angiography of an 11-year-old girl with Moyamoya. The typical, cloudy web instead of the normal anterior and middle cerebral arteries is marked. Right MRA of a healthy person for comparison.

As Moyamoya disease (of Jap. Moyamoya "foggy") refers to a disease of the brain vessels , it in to a constriction or a closure of both sides of the brain arteries is, as a rule, the internal carotid artery and the middle cerebral artery , and which results in relative anemia (stroke and transient ischemic attack ) in the brain. Many small compensatory vessels develop as bypass circuits that are intended to compensate for this reduced blood flow, but are also so fragile that they can burst and lead to cerebral hemorrhage.

The name of the disease is derived from the fact that the many thin vessels with imaging procedures ( angiography ) look like a fog.

Synonyms are: Nishimoto syndrome; English Nishimoto-Takeuchi-Kudo-Disease

The disease is rare in Europe and occurs mainly in Asia (especially Korea and Japan ). A high rate of late and incorrectly diagnosed and treated patients has been described in non-Asian patients. A 2019 scientific article names misdiagnosis rates over 60% and times to correct diagnosis of over 5 years in European patients. The incidence of the disease shows a double peak - at the age of 2 to 10 years and 30 to 40 years. While the term "Moyamoya disease" denotes the idiopathic (without recognizable cause) form, the term "Moyamoya syndrome" is used for the angiographic image in the context of other diseases such as arteriosclerosis, von Recklinghausen's neurofibromatosis, Down syndrome, radiation sequence, etc. In Asian patients, hereditary predisposition with the RNF213 gene plays a causal role, with other triggers and influencing factors also being added. In non-Asian patients, various hereditary factors have been discussed as causal.

Patients with Moyamoya disease are prone to cerebral infarction and hemorrhage . The clinical picture corresponds to these vascular diseases. In addition, headaches and movement disorders as well as a wide range of symptoms can occur. The diagnosis is made using angiography and magnetic resonance imaging and should be reserved for specialized centers. The disease can be divided into different stages from I-VI according to the Suzuki classification.

In addition to conservative treatment with ASA 100 or similar, neurosurgical bypass treatment appears to be particularly promising. An operative connection between the temporal artery ( arteria temporalis superficialis ) and a cerebral artery (arteria cerebri media) is established.


Web links

Individual evidence

  1. ^ Suzuki classification. Accessed April 4, 2020 (German).