Moyamoya disease is a progressive and rare type of blood vessel problem caused primarily because of blocked arteries at the base region of one’s brain also known as basal ganglia. The name Moyamoya indicates puff of smoke in Japan and its structure is more or less similar to tangled vessels, which form to compensate for the formed blockage.

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Can You Die From Moyamoya Disease?

Can You Die From Moyamoya Disease?

Moyamoya disease is a progressive one, as mentioned earlier here. Because of this, we have to discuss its prognosis based on two different conditions i.e. without surgical treatment and with surgical treatment.

Without Treatment

Moyamoya disease in case of without surgical treatment may prove to be fatal because of intracerebral hemorrhage. Because of this, without surgery, a large number of affected individuals experienced strokes on a recurrent basis and graduation deterioration in the cognitive function. Moreover, if we talk about research studies related to long-term follow-up of many untreated patients, we have found about 50% to 66% people have reported poor outcome and progressive type of neurologic deficits.

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Overall, the mortality rate because of Moyamoya disease is 10 percent in case of adults, while 4% approximately in kids.

With Surgery

Even though no one can guarantee about the numbers of years you will live with your Moyamoya disease after your surgical treatment, surgeries have proved to be beneficial in avoiding strokes and thereby, increasing the numbers of years. Reason for this is that surgery related to Moyamoya disease prevents neurologic deterioration due to strokes. For this, doctors recommend for two different types of surgeries, which include indirect and direct revascularization. (1)

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Direct Revascularization

In case of Direct Revascularization, doctors use a branch of an STA i.e. scalp artery to perform direct anastomosis to a branch belongs to MCA i.e. brain artery on the brain’s outer surface. The procedure benefits many patients by providing them with an immediate improvement in the supply of blood towards their brain.

The main objective behind STA-MCA bypass and laying of STA directly on the surface of the patient’s brain is to achieve an indirect revascularization process. Based on STA enlargement and increase in indirect revascularization, the flow of blood will continue to improve in a period of few months.

If you have a look at the outcome of direct revascularization surgery in patients, you will find about 95 percent increase in the graft patency with excellent yet long-term outcomes. In addition, a large number of patients recovered successfully in only 2 weeks to 6 weeks period after their surgery.

Indirect Revascularization

In case a child or adult is ineligible for direct i.e., STA-MCA bypass surgery, doctors recommend for indirect surgical methods used primarily to provide relatively higher amount of blood flow to Moyamoya patients’ brains. These include the following-

EMS i.e. Encephalo Myo Synangiosis: In case of EMS indirect surgery, doctors dissect the temporalis muscle present in the temple areas of one’s forehead and later on, place it on the brain surface with the help of an opening present in the skull.

EDAS i.e. Encephalo Duro Arterio Synangiosis: In this surgery, doctors use a specific branch of one’s superficial temporal artery laid directly on the brain surface without any requirement of a direct anastomosis. The main logic behind this surgery is that doing it results in the growth of new arteries in the brain to provide enough flow of oxygen and blood both. The surgery revealed relatively better results in case of children as compared to adults.

Both of these mentioned indirect revascularization surgery requires a patient to wait for 3 months to 6 months period for the development of new blood supply from their transposed muscles.

Key Facts on Moyamoya Disease

  • Moyamoya disease often affects children, but it may even affect adults.
  • Affected individuals remain at relatively higher risk related to strokes, blood clots and TIAs i.e. Transient Ischemic Attacks.
  • The mentioned problems accompanied frequently due to muscle weaknesses and seizures, along with paralytic attack on a particular side of the patient’s body.
  • Moyamoya disease affected individuals even have speech deficits, disturbed consciousness, cognitive and sensory impairments, vision problems and involuntary movements.
  • Moyamoya is a type of inherited disease, as it tends to run in other members of the family.

Conclusion

Based on aforementioned facts, we should say that chances of a patient’s death increases if he does not undergo with surgical treatment in a timely way.

References:

  1. https://www.ninds.nih.gov/Disorders/All-Disorders/Moyamoya-Disease-Information-Page

Also Read:

Sheetal DeCaria MD

Written, Edited or Reviewed By:

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Last Modified On: April 16, 2019

This article does not provide medical advice. See disclaimer

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