Moyamoya disease is a very rare disease. This intriguing name hides a progressive and incurable disease of the cerebral vessels, in which the lumen of the vessels forming the Willis circle and the vessels that nourish them is closed. What are the causes of moyamoya syndrome? What are the symptoms?

The termmoyamoyacomes from Japan (Japanese も や も や 病) and literally means foggy, cloudy, fuzzy, or billows of smoke. In Poland, the disease is often referred to as a cigarette. This is not a metaphor, but also a medical term, because in the radiograph of the brain, the newly formed abnormal collateral vessels look like puffs of smoke.

The disease was first described in Japan in 1960. In Poland, about 40 people struggle with moyamoya, but vascular surgeons believe that this is incomplete data.

To understand this disease, it is important to understand why proper blood flow to the brain is crucial for our functioning. So how does the blood circulate in our head and neck.

The carotid artery is a large artery whose pulse can be felt by placing your fingers on either side of your neck. It originates from the aorta as the common carotid artery and divides near the larynx into external and internal carotid arteries.

The external carotid arteries supply blood to the face and scalp. The inner ones carry blood to the front and side areas of the brain.

Blood is transported to the brain through two paired arteries, the internal carotid arteries and the vertebral arteries. The internal carotid arteries supply the frontal arteries, and the vertebral arteries supply the posterior areas of the brain. As they pass through the skull, the right and left vertebral arteries connect to form a single basal artery.

The basilar artery and the internal carotid arteries communicate with each other in a ring at the base of the brain, which is called the Willis Wheel.

The blood is carried to the face and scalp through paired external carotid arteries. Behind the jaw, the external carotid artery is divided into facial, maxillary, superficial temporal and occipital arteries.

What is the essence of moyamoya disease?

Moyamoya disease is a chronic and progressive stenosis of the internal carotid arteries at the base of the brain, where it is divided into the middle andfront. The walls of the arteries thicken, which translates into a decrease in their internal diameter, and thus worse blood flow.

Progressive narrowing can lead to the complete obstruction of the artery and a stroke. To rotate before ischemia, the brain creates collateral blood vessels to supply oxygen-rich blood to those parts of the brain that do not receive it.

These tiny collateral vessels, visible on the angiogram, have a hazy, transparent appearance, what the Japanese called moyamoya, i.e. clouds of smoke. Side dishes are more delicate than normal items. They can burst and bleed into the brain.

Causes of moyamoya disease

They are not known. It is known, however, that the peak incidence occurs in the first, third and fourth decades of life, so children and young adults usually suffer from the disease. Asia has the highest number of cases of the disease, but it also occurs in Europe. The disease affects women slightly more often.

Moyamoy's disease: symptoms

Symptoms can appear suddenly, even in a previously he althy person. The symptoms differ depending on the age at which the disease appeared.

Symptoms of moyamoy in children are:

  • hemiplegia
  • dizziness
  • headache
  • convulsions
  • involuntary movements
  • mental retardation

This is just a dry encyclopedic description of the symptoms accompanying the disease. In reality, however, patients suffer from an unimaginable headache that provokes vomiting, causes loss of consciousness, loss of ability to speak, suffering difficult to understand.

In adults, apart from the symptoms already mentioned, there are also strokes, subarachnoid hemorrhages and symptoms of TIA, i.e. transient ischemic attack.

In the medical literature there are also cases of familial occurrence of the disease (about 10%). Then we are talking about the genetic basis of the disease, a multifactorial autosomal type of inheritance.

Many cases of moyamoya disease coexisting with other serious conditions have also been reported. These include:

  • Graves' disease
  • leptospirosis
  • tuberculosis
  • aplastic anemia
  • Fanconi's anemia
  • Aperta team
  • Down syndrome
  • Marfan's syndrome
  • tuberous sclerosis
  • Hirschsprung's disease
  • atherosclerosis
  • aortic coarctation
  • hypertension

The disease may appear after vascular damage after radiotherapy, due to the presence of lupus anticoagulant, cranial trauma or a tumor in the areaTurkish saddle.

Moyamoya develops in stages that can be captured in subsequent angiographic examinations. It begins with the narrowing of the internal carotid arteries. Once initiated, the process of blocking the arteries continues. No known drugs can reverse the blockage.

Moyamoy's disease: treatment

No drugs have been developed to stop the progression of the disease. Surgery is usually recommended in patients with recurrent or progressive TIAs or strokes.

There are several different types of treatments available, all of which are designed to help prevent subsequent strokes by restoring (revascularizing) blood flow to the affected areas of the brain.

Procedures that can be divided into two groups include direct or indirect connection between blood vessels.

Generally, direct bypass procedures are performed on adults and older children, while indirect procedures are preferred for children under the age of 10.

Moyamoy's disease: prognosis

The prognosis for moyamoya patients is difficult to predict as the natural history of the disease is not well known.

Moyamoya may progress slowly with occasional TIA or strokes, or it may progress rapidly.

The overall prognosis of patients with moyamoya disease depends on how quickly it occurs and how much the vessels become blocked. It is also important how the patient's organism copes with the formation of collateral circulation.

The fate of patients also depends on how quickly they undergo surgery to protect them from a stroke.

In patients who have suffered a stroke, it is also extremely important to introduce physical therapy, speech therapy and occupational therapy to therapy to help them regain function and cope with disability.

Read also:

  • Stroke rehabilitation - principles of medical care
About the authorAnna Jarosz A journalist who has been involved in popularizing he alth education for over 40 years. Winner of many competitions for journalists dealing with medicine and he alth. She received, among others The "Golden OTIS" Trust Award in the "Media and He alth" category, St. Kamil awarded on the occasion of the World Day of the Sick, twice the "Crystal Pen" in the national competition for journalists promoting he alth, and many awards and distinctions in competitions for the "Medical Journalist of the Year" organized by the Polish Association of Journalists for He alth.

Category:

Genetic diseases