Uncommon Causes of Stroke
Atrial fibrillation, hardening of the arteries and high blood pressure are the causes of most strokes. But there are less common causes as well, particularly vascular and hematologic disorders:
Vascular disorders
Noninflammatory blood vessel disorders
Fibromuscular dysplasia (FMD) is a disease that is not inflammatory and does not cause atherosclerosis. It causes abnormal cell development in the artery wall that may lead to stiffening, blockage, aneurysm or tearing of the artery. FMD most often affects the renal (kidney) and carotid arteries.
Vasospasm happens in about a quarter of people who experience subarachnoid hemorrhage (a bleed between the brain and thin tissue covering the brain), typically occurring 3-10 days later. It is a contraction of blood vessels and can reduce blood flow to the brain.
Reversible cerebral vasoconstriction syndrome describes several conditions that share a common set of symptoms. It is diagnosed when a patient experiences thunderclap headaches or severe recurring headaches, along with test results showing at least two arteries in the brain that are constricting but return to normal within three months. Brain imaging via CT scan or MRI is required for diagnosis.
Radiation-induced vasculopathy may happen after radiation treatment for cancer speeds up the development of age-related conditions like atherosclerosis.
Moyamoya disease is a disease in which the carotid arteries progressively narrow and potentially close, resulting in ischemic or hemorrhagic stroke.
Fabry disease is an inherited disease caused by a buildup of a particular type of fat in the body's cells.
Inflammatory blood vessel disorders
Primary angiitis of the central nervous system (PACNS) is a rare inflammatory blood vessel disease that only affects the brain and spinal cord. Symptoms may include headache, seizure, stroke and degenerative brain disease.
Giant cell arteritis (GCA) causes inflammation of the arteries. It usually happens in the scalp, neck and arms. It causes arteries to narrow, reducing blood flow.
Cerebral vasculitis related to infection – A variety of infectious diseases may cause inflammation of cerebral blood vessels. A few examples include:
- Bacterial infections, such as tuberculosis and syphilis
- Viral infections, such as Epstein-Barr
- Fungal infections, such as Candida and Aspergillus
Cerebral vasculitis related to neoplasms – Diseases of the arteries may rarely complicate systemic tumors. Meningitis caused by cancer or lymphoma may affect the small and middle-sized intracranial arteries.
Hematological disorders
Ischemic stroke may be associated with hereditary and acquired blood clotting disorders. These are uncommon.
Sickle cell disease (SCD) (also called sickle cell anemia) is a genetic disorder almost exclusively affecting people of African or Black American ancestry. It causes abnormal hemoglobin, the substance in red blood cells that carries oxygen to all the body’s organs. About 300,000 babies in the U.S. are born with SCD each year. All newborns in the U.S. are screened for it at birth with a simple heel prick blood test.
Clotting and coagulation system disorders – These disorders lead to blood clots in certain parts of the body, such as the deep veins in the leg.
Certain gene mutations may cause blood to clot more easily. Clotting tendencies are also associated with oral contraceptives, systemic inflammatory disorders and malignancies.
Antiphospholipid antibody syndrome (APS) is an autoimmune disorder that can cause blood clots.
Miscellaneous disorders
Migraine is associated with abnormalities of platelet, coagulation and blood vessel inner lining function. Migraine with aura is associated with a greater risk of ischemic and hemorrhagic stroke in women, especially under age 55. We need more research to recommend specific ways to treat migraine while lowering stroke risk. However, stroke risk and migraine frequency are associated. Therefore, guidelines recommend treatments that lower stroke risk and reduce how often migraines happen. Quitting smoking is strongly recommended for women with migraine headaches with aura.
Cerebral venous thrombosis – CVT is a rare cause of stroke. In children, it’s related to infections —sinusitis, tonsillitis or pharyngitis — and requires immediate antibiotics. In adults, most cases are related to contraceptives, severe dehydration, malignancy and conditions that cause blood cells to grow abnormally in the bone marrow.
