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[A case of multiple cerebral arterial thrombosis due to congenital protein C deficiency]
Insights
Protein C deficiency, a risk factor for blood clots, may cause cerebral thrombosis. This rare arterial thrombosis is linked to reduced Protein C levels, challenging previous understandings of its embolic mechanism.
Area of Science:
- Neurology
- Hematology
- Genetics
Background:
- Protein C deficiency is a known risk factor for thrombotic diseases, primarily venous thrombosis.
- Arterial thrombosis is a rare manifestation of Protein C deficiency, typically attributed to embolic mechanisms.
Observation:
- A 49-year-old man presented with right hemiparesis and motor aphasia.
- Cerebral angiography revealed occlusion of the left distal internal carotid artery and anterior cerebral arteries, with left internal carotid artery stenosis.
- The patient and his brother exhibited approximately 50% of normal Protein C activity and antigen levels, with no other stroke risk factors.
Findings:
- A diagnosis of atherothrombotic cerebral infarction was established based on clinical presentation and angiographic findings.
- This case suggests a potential direct causal link between Protein C deficiency and the development of cerebral thrombosis.
- Atherothrombotic infarction in this context is considered extremely rare.
Implications:
- This case challenges the prevailing view that Protein C deficiency-related arterial ischemic stroke occurs solely via embolic mechanisms.
- It highlights the possibility of Protein C deficiency directly contributing to cerebral thrombosis, expanding the understanding of its thrombotic potential.
- Further research is warranted to elucidate the mechanisms by which Protein C deficiency may lead to atherothrombotic cerebral infarction.
Abstract:
We report a 49-year-old man who had right hemiparesis and motor aphasia. A computed tomography revealed hypodense areas in the left frontal subcortex. A cerebral angiography demonstrated occlusion of the left distal internal carotid artery and both anterior cerebral arteries, as well as stenosis of the left internal carotid artery at the cervical portion. The second angiogram obtained a month later showed no changes. The diagnosis of atherothrombotic cerebral infarction was established on the basis of clinical profile and angiographic findings. Protein C activity and antigen levels were reduced to approximately one half of the normal level in the patient and his brother. The patient had no other risk factors for stroke. Protein C deficiency has been considered one of the risk factors for thrombotic diseases. Venous thrombosis is the most common clinical manifestation, whereas arterial thrombosis is relatively rare. It is generally believed that arterial ischemic stroke associated with protein C deficiency occurs with embolic mechanism, and atherothrombotic infarction is extremely rare. This is the first report suggesting the possibility that protein C deficiency can cause cerebral thrombosis.