The patient developed paroxysmal headache without obvious cause more than 20 days ago, accompanied by slight blurred vision. The patient had intermittent epilepsy for 4 times since the age of 6, and the duration was not long. After taking carbamazepine and other auxiliary neurotrophic drugs, the medication was basically controlled and stopped.
Patchy in the right frontal, temporal, occipital and other white matter of the brain, long T1T2 signal in point shape, FLAIR mostly high signal, DWI speckle high signal. T2WI showed that the airflow signal of the middle cerebral artery in both sides of the brain basically disappeared and the lumen became narrow. MRA: occlusion of the terminal, anterior and middle cerebral arteries of the right internal carotid artery; The left middle cerebral artery is also narrow and partially blocked. Abnormal vascular network (Moyamoya[smog]) formed at the bottom of the brain.
Diagnosis: moyamoya disease.
(1) basic pathology
Moyamoya disease (Moyamoya disease) is a group of cerebrovascular diseases with unknown causes, characterized by the narrowing or occlusion of the internal carotid artery, the compensatory dilation of blood vessels at the bottom of the brain, and the abnormal capillary network of hyperplasia.
The smooth muscle cells in the middle of the end of the internal carotid artery were damaged and proliferated until the vascular wall atrophied. Abnormal vascular network is formed due to the distal stenosis or occlusion of the bilateral internal carotid artery, the compensatory dilated collateral circulation and the obvious dilatation of the perforated artery.
(2) major clinical information
The main manifestations are cerebral ischemia and intracranial hemorrhage.
In childhood (less than 15 years old), the main manifestations were ischemic changes in the internal carotid system, and the incidence rate was about 90%.
More than half of the adult group showed symptoms of subarachnoid hemorrhage.
(3) imaging diagnosis and pathological basis
The main basis for the diagnosis of moyamoya disease is to directly display the rich and disorderly collateral circulation network of occluded bilateral internal carotid artery and brain floor.
MRI angiography has obvious advantages in showing abnormal cerebral basal ganglia arterial ring. The main abnormality is the asymmetric distribution of middle cerebral arteries on both sides of the brain, and the vascular diameter is uneven or even absent. Irregular and twisted networks of vessels can be seen in basal cistern and basal ganglia. MR T1WI showed basal ganglia dotted and striated low signal shadows, all of which were compensatory vascular collateral circulation network.
More in cortex and subcortical areas on both sides, multiple cerebral infarctions, and concurrent extensive brain atrophy, ventricular expansion and brain stem vascular stenosis and occlusion edge area of the brain blood deficiency, but normal cerebellum and brain stem. These changes are an important basis for a wide range of vascular lesions in the brain.
Intracranial hemorrhage is a common complication of moyamoya disease.
Vascular malformations, extensive cerebral parenchymal lesions, 'triple signs' of intracranial hemorrhage and a history of clinical recurrence can be used to confirm the diagnosis of moyamoya disease.
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