Uncommon Causes of Stroke

Updated:Apr 1,2014
(An online extra for the Stroke Connection magazine Spring 2014 article, Uncommon Causes, available in e-Zine or PDF format)

The vast majority of strokes are caused by problems such as atrial fibrillation, hardening of the arteries and high blood pressure. But there are many less common causes of stroke as well. Following is a list of some of the rarer causes of stroke:

VASCULAR DISORDERS

NONINFLAMMATORY BLOOD VESSEL DISORDERS

Fibromuscular Dysplasia – a disease that causes abnormal cell development in the artery wall. As a result, areas of narrowing, aneurysms or dissections may occur. If narrowing or a tear causes a decrease in blood flow through the artery, symptoms may result. Renal (kidney) and carotid arteries are most commonly affected. (For more information see our Update on FMD, or visit the Fibromuscular Dysplasia Society of America.)

Vasospasm after Subarachnoid Hemorrhage – With vasospasm a blood vessel spasms or contracts causing less blood flow . Cerebral (brain) vasospasm may happen after an operation for a bleed that occurs between the brain and the thin tissue covering the brain (subarachnoid hemorrhage). This type of vasospasm increases the risk of an ischemic stroke. Vasospasm typically occurs 4-10 days after subarachnoid hemorrhage.

Reversible Cerebral Vasoconstriction Syndromes – A condition in which the arteries of the brain develop vasospasm (a blood vessel spasms causing less blood flow) without a clear cause, such as hemorrhage or trauma. It includes several connected disorders linked with problems of brain blood vessel dilation and constriction, leading to constriction of medium and large arteries, such as the circle of Willis. This can cause sudden-onset, debilitating headaches.

Radiation-induced Vasculopathy – Radiation treatment for cancers of the head and neck may lead to delayed toxicity to the nervous system. Brain cells may die as a result, but damage to blood vessels is thought to be how that develops.

Moyamoya Disease – A rare disease of the carotid arteries in which they progressively narrow and potentially close, resulting in either ischemic or hemorrhagic stroke. A network of blood vessels form within the brain's blood circulation in order to compensate for the lack of blood flow, but the new vessels cannot manage the amount of blood flow handled by the closed down artery. (For more information, see Stroke Connection Winter 2014.)

Fabry Disease – A rare X-linked genetic disease that causes lipid deposits in the inner lining of blood vessels and results in progressive disease of the blood vessels of the brain, heart, skin and kidneys.

INFLAMMATORY BLOOD VESSEL DISORDERS

Isolated Angiitis of the Central Nervous System – A rare form of inflammatory blood vessels disease that affects only the brain and spinal cord; no other blood vessels appear to be affected. Symptoms may include headache, seizure, stroke and degenerative brain disease.
 
Temporal (Giant Cell) Arteritis – A systemic inflammatory disorder of the blood vessels, most commonly involving large and medium arteries of the head, usually the branches of the external carotid artery. The most serious complication of GCA is permanent blindness. It is twice as common in women, but still rare.

Cerebral Vasculitis related to Infection – There are a number of infectious diseases that cause inflammation of cerebral blood vessels, including meningovascular syphilis, tuberculous meningitis, other bacterial meningitis, fungal meningoencephalitides, neurocysticercosis, varicella-zoster virus encephalitis, human immunodeficiency virus (HIV) and hepatitis C virus. Generally treating the underlying cause reduces the cerebrovascular inflammation and risk of stroke.

Cerebral Vasculitis related to Toxins – Toxins involved in inflammation of the cerebral blood vessels include cocaine, amphetamines, heroin, lysergic acid diethylamide (LSD) and inhaled volatile solvents (glue sniffing). No specific therapy has been shown to improve the problem with the blood vessels, but the drug(s) should definitely be stopped immediately.

Cerebral Vasculitis related to Neoplasms – Diseases of the arteries may rarely complicate the course of systemic tumors. Meningitis (serious inflammation of the meninges – the thin, membrane covering the brain and the spinal cord) caused by cancer or lymphoma may affect the small and middle-sized intracranial arteries.

HEMATOLOGICAL DISORDERS
Ischemic stroke may be associated with a number of hereditary and acquired blood clotting disorders, including abnormalities of red cell or platelet function, coagulation factors or endogenous fibrinolysis. All of these affect how the blood clots or does not clot as it should. According to Dr. Kasner, these disorders are uncommon, but are over-represented among young stroke patients and should be considered when no alternative stroke cause is identified.

PROTHROMBOTIC (CLOTTING) DISORDERS

Disorders of the Coagulation System – Certain gene mutations (changes in the gene) may cause blood to clot more easily, but they are not typically the cause of ischemic strokes. Clotting tendencies are also found in association with oral contraceptives, systemic inflammatory disorders and malignancies.

Antiphospholipid Antibody Syndrome – Antiphospholipid antibodies are associated with immune-mediated illnesses, syphilis, and stroke. It is thought to result from a hypercoagulable disorder – meaning the person has increased risk for developing blood clots. The mechanism by which they cause clots is uncertain, but they appear to interfere with naturally occurring anticoagulants, protein and platelet stability.

Sickle Cell Disease – A group of inherited blood disorders characterized by chronic anemia, painful events and various complications, including stroke, due to associated tissue and organ damage. Sickle cell disease causes a progressive nonatherosclerotic large vessel narrowing that may develop into a pattern that looks like moyamoya disease. Blood thickens during a sickle crisis and reduces blood flow to tissue or may block blood flow to the brain. Anemia that often accompanies the disorder can cause arteries to dilate and increase cerebral blood flow, which may in turn increase the risk of intracerebral hemorrhage. For more information visit Sickle Cell Disease Association of America.

MISCELLANEOUS DISORDERS

Migraine-Related Stroke – There is evidence that patients with migraine, particularly migraine with aura, have an increased risk of stroke. The mechanism for this is unclear, although migraine is associated with abnormalities of platelet, coagulation and blood vessel inner lining function, and that may contribute to an increased risk of stroke.

Cerebral Venous Thrombosis – CVT is a rare cause of stroke. In children it is related to infection, such as sinusitis, tonsillitis or pharyngitis, and requires early administration of appropriate antibiotics. In adults, most cases are related to contraceptives, severe dehydration, malignancy, myeloproliferative disorders (conditions that cause blood cells to grow abnormally in the bone marrow), hypercoagulable states (increasing the risk for developing blood clots) and inflammatory bowel disease. The systemic disorder must be treated in order to maximally treat the CVT and prevent recurrent events. Many adult cases are related to pregnancy and are treated after the delivery of the child. The cause remains unknown in about 20 percent of cases.

Regarding migraine with aura and CVT from the American Heart Association/American Stroke Association Feb 2014 Guidelines for the prevention of stroke in women: A statement for healthcare professionals:
  • Stroke from central venous and sinus thrombosis (CVT) is remarkably more common in women, and has been related to hormonal factors (contraceptive use, pregnancy) and thrombophilia. The guidelines provide recommendations for screening, testing for prothrombotic conditions, and anticoagulation for women with CVT and a history of CVT.
  • Migraine with aura is associated with an increased risk for ischemic and hemorrhagic stroke in women, especially in women under the age of 55 years.  Data are insufficient to recommend specific strategies to treat migraine while lowering stroke risk. However, since an association exists between migraine frequency and stroke risk, guidelines support treatments to reduce migraine frequency. Quitting smoking is strongly recommended for women with migraine headaches with aura.


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