Moyamoya disease in children

Introduction to pediatric moyamoya disease Moyamoya disease, also known as Moyamoya disease or spontaneous basilar artery occlusion, is a kind of slowing of the intima of the arteries at the end of the bilateral internal carotid artery and the anterior and middle cerebral artery. The arterial lumen is gradually narrowed and occluded. A disease characterized by compensatory dilatation of the perforating artery. The source of the "smog" is a reticular shadow that appears in the bottom of the brain due to abnormal capillary proliferation during cerebral angiography, like a smoked one. Stock smoke, hence the name. Moyamoya is the pronunciation of Japanese "". The essence of the disease is the occlusion of the main artery of the brain with compensatory vascular proliferation. basic knowledge The proportion of the disease: the disease is rare, the incidence rate of children is about 0.0001% - 0.0002% Susceptible people: children Mode of infection: non-infectious Complications: Somatosensory Disorders Epilepsy

Causes of pediatric moyamoya disease

(1) Causes of the disease

The cause of this disease is unknown. At present, the disease is divided into congenital and acquired acquired diseases. Congenital moyamoya disease has many genetic factors. Acquired moyamoya disease is caused by a variety of causes, such as sickle cell anemia, neurofibromatosis, tuberous sclerosis, glycogen storage disease, connective tissue disease, aortic coarctation, Apert syndrome after radiation therapy And various infections of the central nervous system, such as tuberculous meningitis, purulent meningitis, leptospirosis, syphilis, etc. In recent years, the etiology of this disease emphasizes ethnic genetic factors, Alva reports that the disease occurs in the same family. Single-oval twins account for 7%, and familial moyamoya disease accounts for about 10% in Japan. Recent studies on familial moyamoya disease suggest that the disease's genes are located on chromosomes 3, 7 and 17 (17q25).

(two) pathogenesis

The basic lesions of this disease are chronic progressive stenosis and occlusion of the cerebral artery ring caused by a variety of reasons, and gradually form a collateral circulation, which is caused by the expanded Henbnei artery, the bean vein artery, the anterior and posterior choroidal artery, and the posterior cerebral artery to the ventricle. Branching, basal ganglia and dilated capillaries at the internal capsule are a compensatory phenomenon. It is not a change in the characteristics of a disease. Angiography is an abnormal vascular network. If the disease progresses slowly and the collateral circulation is abundant, it does not occur. Obvious clinical symptoms; such as rapid progression of vascular occlusion, resulting in the failure to form collateral compensatory, that is, a series of symptoms of internal carotid artery ischemia, such as hemiplegia, aphasia, etc., due to abnormal blood vessel wall, showing hyperplasia, and In the high-pressure area of the proximal occlusion, it is subjected to large blood pressure and blood flow, so it can be broken to form subarachnoid hemorrhage.

Pediatric moyamoya disease prevention

Active prevention and treatment and various infectious diseases, such as brain, purulent, syphilis, leptospirosis, etc., and pay attention to the prevention and treatment of hereditary diseases.

Pediatric moyamoya disease complications Complications somatosensory disorder epilepsy

Other symptoms of the disease include sensory disturbances, seizures, blindness, involuntary movements, mental retardation, and mental disorders. Bleeding type of moyamoya disease, usually like a normal person, once the onset, will suddenly have a severe headache, followed by nausea, vomiting, and soon will be coma. Going to the hospital for a checkup is cerebral hemorrhage, and further examination is due to cerebral hemorrhage caused by moyamoya disease. This part of the patient's condition is very serious, severely in a coma, even after active treatment, the patient is awake and has severe neurological dysfunction.

Pediatric moyamoya symptoms common symptoms meningeal irritation weakness intracranial hemorrhage

The disease can occur in all age groups, the age of children with good age is below 10 years old, the clinical manifestations can be divided into two types of ischemic and hemorrhagic type, children with ischemic type, adult patients with intracranial hemorrhage, Karasawa 104 cases of children with moyamoya disease were reported. The most common clinical manifestation was hemiplegia. Among them, transient ischemic attack (TIA) accounted for 57 cases, persistent hemiplegia occurred in 44 cases, and cortical dysfunction was most common in 21 cases of aphasia. It is worth noting that TIA high frequency occurs in the first 4 years after the first onset of the disease, but it is rare in the future. Some children or headaches may occur. Headaches may occur several months or a year before hemiplegia, and individual cases have also been seen. The first symptom of the child is subarachnoid hemorrhage, which is characterized by severe headache, obvious neck resistance and meningeal irritation. The first symptom in adults is more common in subarachnoid hemorrhage, followed by dyskinesia. Beijing Children's Hospital reports moyamoya disease. For example, in 12 cases (63%) of patients over 5 years old, 10 cases (53%) with paroxysmal limb weakness or hemiplegia as the first symptom in the 19 cases, and 17 cases of hemiplegia in the main clinical manifestations, including alternating hemiplegia 7 cases 11 cases of sports aphasia, 10 cases of headache, clinical indications such as limb weakness or hemiplegia, sports aphasia or headache as the main manifestation of children, should consider the possibility of this disease, should do head magnetic resonance angiography as soon as possible ( MRA) or cerebral angiography for early diagnosis and further examination can help identify the cause.

Pediatric smoke sickness check

There was no abnormality in routine laboratory tests at the time of onset. In addition to children with subarachnoid hemorrhage in cerebrospinal fluid examination, the cerebrospinal fluid of some children may have a slight increase in protein, and the number of cells and biochemistry are normal. And examination of cerebrospinal fluid virus immunological antibodies.

EEG examination

The change is non-specific and a slow wave can appear on the lesion side, which helps to determine the lesion side.

2. Neuroimaging

The diagnosis of moyamoya disease is mainly based on neuroimaging diagnosis. Therefore, brain magnetic resonance angiography (MRA) or digital subtraction angiography (DSA) is an important basis for the diagnosis of pediatric moyamoya disease. MRA is a new vascular imaging technology. It better reflects the location and extent of intracranial vascular lesions, and because of its non-invasive, fast, no need for contrast agents, easy to repeat, etc., it may become an important and common technical method in pediatric cerebrovascular diseases, through MRA or DSA examination, This disease can show the end of the internal carotid artery siphon and the anterior or middle cerebral artery stenosis or occlusion, abnormal vascular network formation at the bottom of the brain, most involving bilateral, can also be seen in unilateral involvement, in 1 to 2 years One-sided involvement can progress to bilateral damage.

Diagnosis and diagnosis of pediatric moyamoya disease

diagnosis

The clinical manifestations are limb hemiplegia, motor aphasia and headache, and auxiliary examination of cerebral blood vessels with smoke-like image changes, which supports the diagnosis of this disease.

Differential diagnosis

Different from other cerebrovascular diseases, such as intracranial hemorrhage, cerebrovascular embolism.

1. Cerebrovascular hemorrhage is most common in craniocerebral trauma, birth injury, sometimes thrombocytopenic purpura, aplastic anemia, hemophilia, leukemia, brain tumors, and acquired acquired late vitamin K deficiency. Can be complicated by cerebrovascular hemorrhage, clinical onset, convulsions, coma, convulsions, neck stiffness and symptoms of intracranial hypertension (such as severe headache, frequent jet vomiting, optic disc edema, slow pulse, high blood pressure, etc.), waist The cerebrospinal fluid is evenly red, the pressure is increased, and the red blood cells are contained. After a few hours, there are wrinkled red blood cells, which are easy to be complicated by hippocampus, and the cerebellar tonsils can cause rapid death.

2. Cerebral vascular embolism (cerebrovascular embolism) Infective endocarditis, rheumatic heart disease mitral stenosis with atrial fibrillation, cardiac wall thrombus can fall off causing cerebral embolism, occasional air embolism, complex open fracture Fat plugs, emboli of thrombophlebitis can also cause cerebral embolism. The clinical onset is sudden, sometimes only occurs within a few seconds. Because of easy to break into the middle cerebral artery, there are three partial signs, cerebrospinal fluid is normal, or there are slight red blood cells. Increased, acute stage may have mild disturbance of consciousness, but the period is shorter, the intracranial hypertension is mild, the distal part of the cerebral embolism has secondary cerebral edema and subsequent brain softening, lacunar infarction (lacunar cerebral infarction) And the cavity is easy to leave partiality, dementia.

3. Cerebral thrombosis (cerebrovascular thrombosis) tetralogy of Fallot, polycythemia vera, thrombocytopenia, diffuse intravascular coagulation and other blood viscous, can be complicated by cerebral artery thrombosis, clinical onset is relatively slow, prone to Light and moderate internal capsule triple deviation, the consciousness is not affected, the cerebrospinal fluid is normal and there is no red blood cells.

4. Cerebral arterial intimitis In the past, tuberculous meningitis, bacterial meningitis, leptospirosis and cerebral endarteritis are rare, and connective tissue disease (especially systemic lupus erythematosus) is rare. After cryptococcal meningitis and non-specific viral infection, sometimes middle cerebral artery endarteritis is present, and hemiplegia syndrome occurs. The latter is mild, most of them return to normal after 1 to 2 months, causing leptospirosis Alternating hemiplegia or even bilateral paralysis can occur.

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