Moyamoya syndrome is a group of occlusive diseases characterized by progressive stenosis or occlusion of the internal carotid arteries and their large branch vessels bilaterally, and the formation of an abnormal neovascular network at the base of the skull. The name “smoke” comes from the fact that the cerebral angiogram shows a fuzzy mesh-like shadow at the base of the brain due to abnormal capillary hyperplasia, like a puff of smoke from smoking.
The clinical manifestations are mainly divided into two categories: hemorrhage and ischemia, with a bimodal distribution of the age of onset between 5 and 40 years old. The essence of the disease is occlusion of the arterial trunk at the base of the brain with compensatory vascular proliferation.
Symptoms and signs of smoker’s disease
The onset of smoker’s disease is more common in children and adolescents, and often begins as a stroke, which can manifest as cerebral thrombosis or as cerebral hemorrhage and subarachnoid hemorrhage. Patients may present with varying degrees of hemiparesis or sequential paralysis on the right and left sides, which may be accompanied by aphasia, choking on water, dysphagia, mental retardation, dementia, seizures, headaches, and transient ischemic attacks.
Infarcts or hemorrhagic changes are usually seen on CT scans of the head. Infarcts are often multiple, with frontal, temporal, parietal, occipital, basal ganglia regions, and thalamus being the most common, and frontal lobe atrophy may be combined in half of the patients. The hemorrhages can be lobar hemorrhages, basal ganglia hemorrhages, or subarachnoid hemorrhages, and most of the cerebral hemorrhages caused by hypertension are located in the basal ganglia region. Patients with cerebral hemorrhage may also have concurrent findings of infarct foci and/or cerebral atrophy.
Cerebral angiography may reveal stenosis or non-visualization of the beginning of the internal carotid artery, the beginning of the anterior and middle cerebral arteries, and a large number of tiny vascular clusters in the basal ganglia region like smoke exhaled from a cigarette. In addition, a compensatory branch of the collateral circulation can be formed in the brain. As the disease progresses, the number of compensatory anastomosing branches gradually decreases or shrinks.
Experts say: early detection of symptoms of smog and early treatment can effectively prevent the deterioration of the disease.
Common preventive measures for smog disease
1, pay attention to rest and functional exercise of paralyzed limbs.
2, strengthen nutrition, give high protein and high vitamin diet.
3,In case of subarachnoid hemorrhage, the patient should not be moved, and avoid coughing, sneezing and holding the breath to defecate and other actions that increase the pressure in the chest and abdominal cavity.
4,Flooding should be prevented, rodents should be exterminated, poultry should be kept in captivity to prevent contamination of water sources, and drinking water should be disinfected.
5,Educate children not to drink raw water, do not play in infected water, bathing, etc., to avoid leptospirosis.
Warm tip: early detection, early diagnosis and early treatment of smog is the key, through early surgical treatment, more than 80% of patients can return to normal life. Internationally, it has been reported in the literature that smog can be treated with intracranial and extracranial vascular anastomosis, combined brain-muscle-vascular surgery and other surgical procedures to reconstruct blood flow and improve prognosis. Patients are also advised to choose a regular hospital for treatment.