Clinical manifestations of smoker’s disease (Moyamoya disease)

  The clinical presentation of smoker’s disease differs significantly in pediatric and adult patients. The vast majority of pediatric patients present primarily with transient ischemic attack (TIA) or cerebral infarction, while approximately 50% of adult patients present primarily with cerebral hemorrhage and another 50% with transient cerebral ischemia or cerebral infarction.  I. Transient cerebral ischemia and cerebral infarction Smoking disease often leads to cerebral ischemia in the internal carotid artery supply area of the brain, especially in the frontal lobe. As a result, most patients will exhibit frontal lobe signs and symptoms, such as dysarthria, aphasia, or hemiparesis. Smoker’s disease can also have other atypical symptoms such as syncope, lower extremity bradykinesia, visual symptoms or involuntary movements, which are more likely to occur in pediatric cases. Some pediatric cases develop mental retardation due to frontal lobe cerebral ischemia or infarction. A few adult cases may have cognitive impairment, such as memory loss, irritability, or anxiety. Patients with these symptoms are often misdiagnosed with psychiatric disorders such as schizophrenia, depression, or personality disorders.  Cerebral ischemic attacks in pediatric patients are often triggered by hyperventilation, such as crying or playing a wind instrument. Therefore, if a sudden weakness or numbness of one limb occurs in children and improves for a short period of time, this should be considered as a possibility of smog, and a specialist examination such as magnetic resonance angiography (MRA) should be done at the hospital as soon as possible.  Intracranial hemorrhage About 50% of adult smoky patients will have brain hemorrhage. There are two main causes of hemorrhage: rupture of a dilated, brittle Moyamoya vessel or rupture of a cystic aneurysm in the ring of Willis. In the former case, ruptured Moyamoya vessels may be caused by prolonged hemodynamic stress on the vessel wall, primarily in the basal ganglia, thalamus, or periventricular regions, often in combination with ventricular hemorrhage. In some patients, cerebral angiography may reveal aneurysm formation on the Moyamoya vessels. In the latter case, ruptured bleeding from a saccular aneurysm in the ring of Willis occurs most often in the bifurcation of the basilar artery or in the connection between the basilar and superior cerebellar arteries. In patients with smoke, the vertebrobasilar system plays an important role in the blood supply of the collateral circulation. Therefore, hemodynamic stress on the canal wall often leads to aneurysm formation in the vertebrobasilar system, and aneurysm rupture can lead to subarachnoid hemorrhage. There is increasing evidence that adult smoky patients can develop subarachnoid hemorrhage on the surface of the brain even in the absence of an aneurysm. In rare cases, adult smokers can also bleed from the rupture of a dilated Moyamoya vessel on the surface of the brain. Pregnancy and childbirth may increase the risk of ischemic or hemorrhagic stroke in female patients, whether treated conservatively with medication or surgically.  Other neurological symptoms 1. Headache is one of the serious symptoms of smog, especially in pediatric patients, where this symptom is more common. Seol et al. analyzed 204 cases of childhood smog and found that 25% of the cases had headache, and the headache persisted for more than 12 months after simple patching surgery.  2, epilepsy and involuntary movements are also one of the important clinical manifestations of smog disease, and involuntary movements are mostly seen in children cases.