Any pathophysiological process leading to blood supply disorder of brain tissue may lead to stroke. The most common cause is cerebral artery disease, such as atherosclerosis, inflammation and artery dissection. In addition, the thrombus in the heart or extracranial vessels may transfer to the cerebral vessels and lead to cerebral ischemia.
The patient was 19 years old and lacked traditional stroke and young stroke related risk factors such as atherosclerosis, hypertension, diabetes, atrial fibrillation, heart disease, patent foramen ovale and positive-anticardiolipin antibody et al. He just had a family history of Wilson's disease.
The mechanism of Wilson's disease with massive cerebral infarction is not clear, and may be related to the following reasons: When the accumulation of Cu in the body exceeds the load capacity of various organelles containing copper protein in the tissues, unbound or loosely bound copper can induce oxidative stress and/or exert its toxicity through directly inhibited protein function6. The production of reactive oxygen species (Ros) far outpaces the removal of intracellular antioxidant defenses, the accumulation of free radicals leads to oxidative stress, which leads to damage or even apoptosis of brain cells, in turn leads to neurologic deficits. After oxidative stress occurs, ROS also triggers the release of Metalloproteinase-9 (MMP-9) through neurons, glial cells and endothelial cells, thereby digesting the endothelial basal layer and damaging the blood-brain barrier (BBB). The destruction of BBB can directly change the physiological neuron environment, and then cause neuroinflammation, brain edema, and aggravate the ischemic injury of brain tissue7. Patients with reduced platelets can significantly activate adhesion molecules and damage endothelial cells, which in turn activates neutrophils, macrophages, and microglia, which are expressed as increased levels of serum intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1), both of which can directly damage endothelial cells and cause local brain tissue ischemia8. FIB increases, and FIB promotes the accumulation of Vascular smooth muscle cells and lipids, and contributes to the formation of arteriosclerosis plaque, which increases the formation and development of thrombus9. Patients with low ALB, the liver is activated by a compensatory increase in albumin, which is involved in the conversion of Arachidonic acid to thromboxane A2(TXA2) in the blood. Normally, albumin binds to AA, and when albumin is reduced, the combined AA reduced, and free AA is further converted to TXA2, which enhances platelet aggregation and promotes thrombosis. High-sensitivity C-reactive protein is a marker of inflammation and a direct factor inducing arterial thrombosis10.The increase of hs-CRP can activate the complement system to act on endothelial cells to produce oxidative stress reaction, induce monocyte to produce procoagulant activity, promote the formation and development of thrombus in artery. In this patient, there were over deposition of Cu, thrombocytopenia, decrease of ALB ,increase of FIB and hs-CRP, decrease of ALB, it may lead to massive cerebral infarction through the above mechanism.