Moyamoya disease: symptoms, causes and treatment

Our brain is a fundamental part of the bodyAs it regulates the functioning and coordination of most of the body and allows us to be who we are: controls vital constants to higher processes such as reasoning or motivation, including perception and motor skills.

But even though it is our most important organ, it could not function and even die in a short time if it did not receive a constant supply of oxygen and nutrients. These reach you through the cerebral vascular system, the neurons being supplied by various blood vessels.

However, sometimes these vessels can be injured or be affected by diseases that can cause the blood to deny part of the brain or not reach where it should be, which can have a very big impact on health. Among these problems we can find Moyamoya’s disease, Of which we will see the main features throughout this article.

    Moyamoya disease

    This is called Moyamoya disease in a rare but dangerous cerebrovascular type alteration, Characterized by progressive occlusion or stenosis of the internal carotids (usually occurs in both at the same time) and their main branches in their terminal parts inside the skull.

    This narrowing in turn causes the formation and strengthening of a large secondary network of smaller vessels which allow blood to continue to flow, forming a circuit. the shape of neuroimaging is reminiscent of cigarette smoke (This is what the term Moyamoya refers to, which in Japanese refers to smoke).

    Although in many cases it can remain silent and asymptomatic, the truth is that as there is progression of the narrowing of the carotid artery, the blood reaching it moves at high speed, which is joined. do not have sufficient capacity to carry an adequate supply of oxygen and nutrients.

    This can have serious repercussions for the subject, being able to feel dizziness and mental slowing down, discomfort, irritability, impaired vision or language, weakness or paralysis on any side of the body, Seizures and even the onset of ischemic stroke or even cerebral hemorrhage if a glass breaks (which is more common in this disorder because the secondary network is much more fragile than the carotid artery) , which can lead to loss of physical or mental abilities (depending on the affected area) or even death. The symptoms are generally more noticeable during exercise.

    Although it can appear in people of any age, the truth is that it is much more common in children between the ages of five and nine, which could lead to developmental problems and delays, or even intellectual disability. Another spike in cases was also seen in adults over 45 years old. As for sex it appears in both men and women, Although it is more predominant in the latter.

    Moyamoya’s disease was discovered by Dr Takeuchi and Dr Shimizu in 1957, and it was originally thought to be unique to the Japanese population. However, over time it has been discovered in people of different ethnicities, although it is still much more prevalent in the population of Asian descent).

    As for the prognosis, it can vary greatly depending on when it is detected and the application of treatment. Mortality associated with this disease is approximately 5% in adults and 2% in children, mainly due to stroke.

      stages

      As we have noted, Moyamoya disease is a progressive disorder that worsens over time, having established different stages or degrees depending on the stenosis. In this sense, we find six degrees.

      1st year

      Moyamoya disease is considered grade 1 when seen on its own a narrowing at the point at which the internal carotid artery branches off.

      2nd year

      A more advanced degree occurs when they begin to generate the collateral vessels or the secondary network that gives the disorder its name.

      3rd year

      At this stage of the disease, the collateral vessels begin to intensify as progressive narrowing of the internal carotid artery and middle cerebral artery, Dependent largely on collateral vascularization.

      Level 4

      The network of collateral vessels that originate in the internal carotid begins to weaken and have poorer functionality, at the same time as the flow increases and circuits are generated at the level of the external carotid (outside the skull).

      Level 5

      The network of collateral vessels grows and intensifies from the external carotid artery, while in the internal carotid artery, the collateral network is greatly reduced.

      6th year

      The internal carotid artery is completely closed and its collateral network also eventually disappears, initial secondary circuit shutdown. Blood flow becomes dependent on the external carotid and vertebral artery.

      the causes

      Moyamoya disease does not have a fully defined cause, being an idiopathic disease. Despite this the existence of a genetic influence has been observed, Having studied chromosomes such as 3, 6 and 17 and having observed that it is more common in the population of Asian origin and in people whose parents have suffered. This aspect is also taken into account as it is sometimes associated with genetic disorders.

      In addition, in some cases, it can be associated with infectious processes (in this case, it would be the syndrome, and not the disease, since it is secondary).

      treatment

      Moyamoya disease currently has no treatment to cure it or vice versa, although the symptoms can be treated and have a controlled level of stenosis or possible damage to blood vessels.

      Among other methods, surgery stands out, whereby revascularization surgery can be performed, which will improve circulation, although the vessels tend to constrict (although treatment usually slows down symptoms and their progression). It is also possible to use, although this is done in adults but not in children due to the risk of bleeding after strokes, to use very specific anticoagulants and other substances that regulate the behavior of the patient. blood.

      too much they will have to deal with the complications that may result, such as learning delays and intellectual disability, Provide guidelines and educational supports as needed. Speech therapy and / or physiotherapy can be useful for speech or movement disorders, as well as occupational therapy and psychoeducation in the family.

      Bibliographical references:

      • Acuña, AR and de Godoy, CO (2010). Moya-Moya disease. Pediatrician. (Hypothesis), 37 (1).
      • Bretón, A., Gómez, J., Ramos, R., Martín, V., Pérez, J. and Alayón, A. (1999). Moya-Moya disease. Presentation of a case and review of the literature. An.Esp.Pediatr., 50: 65-68.
      • Buller, I., Luzuriaga, C. and Soler, MG (2016). Moyamoya disease. Clinical Journal of Family Medicine, 9 (3). Albacete.
      • Takeuchi, K. and Shimizu, K. (1957). Hypogenesis of bilateral internal carotid arteries. No to Shinkei, 9: 7-43.

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