
Sarcoidosis is a multisystem inflammatory disease that can affect almost every organ in the body. It is characterised by the presence of non-caseating granulomas in the lymph nodes and lungs and can cause inflammatory changes in small vessels. While cardiac and neurological involvement is rare in sarcoidosis, it can lead to strokes, which are a leading cause of death in developed countries. Strokes associated with sarcoidosis can be either hemorrhagic or ischemic and are generally more common in males. In this article, we will explore the link between sarcoidosis and strokes, the risk factors involved, and the impact on patients' daily lives.
Characteristics | Values |
---|---|
Sarcoidosis type | Neurosarcoidosis, Cardiac Sarcoidosis |
Sarcoidosis presentation | Often asymptomatic before stroke |
Stroke type | Ischemic, Hemorrhagic |
Stroke location | Anterior cerebral circulation |
Patient age | Often young, but not always |
Patient sex | Male predominance |
Pre-existing diagnosis | Often no pre-existing diagnosis of sarcoidosis |
Symptoms | Headache, weakness, confusion, disorientation, dizziness, abnormal sensations of movement, vision problems, facial palsy, loss of senses of smell and taste, psychiatric disturbances, numbness, tingling, loss of movement |
Treatment | Tissue plasminogen activator (TPA), Corticosteroids, Immunosuppression, Thrombolysis, Thrombectomy, Antiplatelets, Statins |
What You'll Learn
Sarcoidosis can cause stroke through granulomatous vasculitis
Sarcoidosis is a multisystem inflammatory disease that affects small vessels. It is a chronic granulomatous condition that can affect almost every organ, causing inflammatory changes in small vessels. Granulomatous vasculitis is a common pattern characterised by vasculocentric destructive mononuclear inflammation associated with well-formed granulomas, multinucleated giant cells, or both.
Cardiac and neurological involvement is rare in sarcoidosis, with only 5% of patients presenting with neurosarcoidosis. However, cardiac involvement in sarcoidosis is not uncommon, and it can lead to restrictive cardiomyopathy, causing a paroxysmal irregular rhythm of the heart, which may result in a cardioembolic stroke.
In a reported case, a 28-year-old male presented with speech difficulty and right-sided weakness. Investigations revealed an abnormal configuration of the P wave in the 12-lead echocardiograph (ECG) and mediastinal widening on chest x-ray. Further imaging confirmed bilateral enlargement with restrictive cardiomyopathy and mediastinal lymphadenopathy, raising suspicion of sarcoidosis. A cardiac positron emission tomography (PET) scan confirmed the diagnosis by identifying a non-caseating granuloma.
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Sarcoidosis-associated strokes can be either hemorrhagic or ischemic
Sarcoidosis is a multi-systemic inflammatory disease defined by the presence of non-caseating granulomas in lymph nodes, lungs, and other organ systems. Affecting the nervous system in about 5% of patients, neurological symptoms are correlated with a poorer prognosis. Stroke in sarcoidosis is a rare but disabling occurrence.
In rare cases, ischemic stroke may be caused by a vasculopathic process associated with underlying rheumatologic or inflammatory disease. Several possible pathologic mechanisms include direct invasion of medium and small arteries and arterioles by granulomatous inflammation as seen on autopsy, cardiovascular embolism, and large-vessel inflammation.
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Sarcoidosis can affect the heart and cause cardioembolic strokes
Sarcoidosis is a multisystem inflammatory disease that can affect multiple organs, including the heart. Cardiac sarcoidosis (CS) refers to granulomatous inflammation that affects the heart, either in isolation or as part of systemic sarcoidosis. This condition can interfere with the normal functioning of the heart and lead to serious complications, including cardioembolic strokes.
CS occurs when tiny collections of immune cells, known as granulomas, form in the heart tissue. This can result in heart rhythm abnormalities, also known as arrhythmias, such as ventricular tachycardia or heart block. It can also lead to cardiomyopathy or heart failure. The signature lesions of CS are noncaseating granulomas, particularly within the interventricular septum and inferior left ventricle. While granulomas can be found in epicardial, myocardial, and endocardial tissue, the subepicardium and myocardium are most commonly involved, while the endocardium is typically spared.
The diagnosis of CS is often challenging due to its variable clinical presentation and the lack of a single definitive diagnostic test. Patients may present with symptoms such as palpitations, presyncope, and syncope related to arrhythmias, as well as exertional dyspnea, orthopnea, or paroxysmal nocturnal dyspnea due to cardiomyopathy. Cardiac magnetic resonance imaging (CMR) and fluorodeoxyglucose F 18 positron emission tomography (FDG-PET) are valuable tools for detecting structural abnormalities and inflammation associated with CS.
The treatment of CS aims to slow disease progression, reduce the risk of sudden cardiac death, treat heart failure, and manage arrhythmias. Immunosuppressive therapy, such as corticosteroids, is recommended to reduce inflammation and fibrosis. Additionally, cardiac-specific procedures, such as implantable cardioverter-defibrillators (ICD) and heart transplants, may be necessary in some cases.
While the exact cause of sarcoidosis is unknown, it is believed to result from an abnormal response of the body's immune system, along with potential environmental and genetic factors. CS can develop in individuals of all ethnicities and ages, but it is more common in patients aged 20-60 and those of African American or Northern European ethnicity.
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Neurosarcoidosis can cause stroke and is treatable
Neurosarcoidosis, a form of sarcoidosis, is a chronic disease that affects the central nervous system, encompassing the brain, spinal cord, and optic nerve. It is characterised by inflammation that can cause the destruction of the coating (myelin) surrounding nerve fibres. This damage disrupts the normal flow of messages from the central nervous system, resulting in a reduction or loss of body function. Neurosarcoidosis can affect the cranial and facial nerves, the hypothalamus, and the pituitary gland. Symptoms vary depending on the affected areas and can be similar to those of multiple sclerosis.
Neurosarcoidosis is a rare but important cause of stroke, as it is treatable. While it typically affects young, relatively healthy people, it has also been observed in elderly patients. The condition can lead to cerebral vasculitis, causing headaches, seizures, confusion, and dementia. Additionally, neurosarcoidosis can result in stroke through direct invasion of medium and small arteries by granulomatous inflammation, cardiovascular embolism, or large-vessel inflammation.
The diagnosis of neurosarcoidosis is challenging and requires a high index of clinical suspicion. It is often identified through brain imaging, such as MRI, MRA, or CTA, and lumbar puncture to evaluate cerebrospinal fluid inflammation. Treatment options include immunosuppressive therapy, such as high-dose steroids, and physical and occupational therapy to help patients adjust to daily life.
In summary, neurosarcoidosis is a rare but treatable cause of stroke that requires prompt recognition and management. It can affect people of all ages and presents with a wide range of neurological symptoms. Early diagnosis and appropriate treatment are crucial for preventing recurrent strokes and improving patient outcomes.
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Sarcoidosis is a rare cause of stroke
The exact mechanism by which sarcoidosis causes stroke is not fully understood. However, it is believed that the inflammation and granuloma formation associated with sarcoidosis can lead to vasculitis, or inflammation of the blood vessels. This can result in a blockage or rupture of the blood vessels supplying the brain, leading to an ischemic or haemorrhagic stroke, respectively.
The occurrence of stroke in patients with sarcoidosis is rare but disabling. In a systematic review of reported cases, stroke was found to be more common in males with sarcoidosis and typically occurred in the absence of traditional risk factors for vascular disease. The majority of patients with sarcoidosis-associated strokes did not have a pre-existing diagnosis of sarcoidosis, with stroke being the presenting symptom in 65% of cases. This highlights the importance of considering sarcoidosis as a potential cause of stroke, even in patients without a known history of the disease.
The diagnosis of sarcoidosis-associated stroke can be challenging and requires a high index of clinical suspicion. Brain imaging, such as magnetic resonance imaging (MRI) and computed tomography (CT) scans, may reveal findings suggestive of neurosarcoidosis, such as leptomeningeal enhancement and granulomas. However, brain biopsy may be necessary to confirm the presence of granulomatous vasculitis, as this is not always evident on imaging.
The treatment of sarcoidosis-associated stroke involves managing the underlying sarcoidosis and preventing further strokes. Immunosuppressive therapies, such as corticosteroids, are often used to reduce inflammation and slow the progression of the disease. Additionally, antiplatelet or anticoagulant therapies may be prescribed to prevent blood clots and reduce the risk of recurrent stroke.
In summary, sarcoidosis is a rare but important cause of stroke. It can affect the heart and blood vessels supplying the brain, leading to ischemic or haemorrhagic stroke. The diagnosis of sarcoidosis-associated stroke can be challenging, and brain imaging and biopsy may be necessary to confirm the presence of neurosarcoidosis. Early recognition and treatment of sarcoidosis-associated stroke are crucial to prevent recurrent strokes and improve patient outcomes.
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Frequently asked questions
Sarcoidosis is a multisystem inflammatory disease that involves small vessels. It is a chronic granulomatous condition that affects almost every organ and causes inflammatory changes in small vessels.
A stroke is a sudden interruption in the blood supply of the brain, usually because blood vessels are blocked or burst.
Yes, although rare, sarcoidosis can cause a stroke.