Introduction:
An arteriovenous malformation (AVM) is an unusual intertwining of blood vessels that connects the arteries and veins, leading to disturbances in the normal blood flow and oxygen supply. Foix-Alajouanine syndrome is an arteriovenous malformation of the spinal cord. Although this disease can affect people of any age group, it is commonly seen in people who are above 50 years of age.
What Is Foix-Alajouanine Syndrome?
Foix-Alajouanine syndrome is a disorder that occurs due to the arteriovenous malformation of the spinal cord in the lumbar (lower back area of the spinal cord) and lower thoracic (the portion of the spinal cord that extends from the base of the neck to the waist area) regions. In this disorder, excess pressure is exerted on the spinal cord, resulting in spinal cord injury. Due to the arteriovenous malformation, disturbances are seen in the circulation as the veins lose their ability to return the blood to the heart. In some cases, arteries and veins involved in the arteriovenous malformation may burst, which leads to bleeding within the spinal cord. Masses of large, thick-walled veins are seen on the surface of the spinal cord, and tiny thick-walled blood vessels are seen within the spinal cord.
What Are the Causes of Foix-Alajouanine Syndrome?
The exact cause of the Foix-Alajouanine syndrome has not been found. However, researchers believe that certain factors are responsible for causing the Foix-Alajouanine syndrome. They are as follows:
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Congenital (present at birth).
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Arteriovenous fistula (the improper connection between a vein and an artery) in the outermost layer covering the brain and spinal cord.
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High blood pressure in the arteries in the outer layer covering the brain and spinal cord.
What Are the Symptoms of Foix-Alajouanine Syndrome?
The symptoms of Foix-Alajouanine syndrome may differ from one person to another. The symptoms depend on the location and severity of the spinal arteriovenous malformation. The symptoms are as follows:
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Numbness or tingling sensation in the legs.
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Difficulty in simple activities like walking and climbing stairs.
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Weakness of the body that may be present either on one side or both sides.
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Feeling of heaviness on the legs.
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Difficulty in standing for prolonged periods.
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Patient experiences unusual and abnormal sensations on touching the legs.
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The patient may have difficulty maintaining balance, leading to frequent falls.
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Pain in the lower back region accompanied by a burning sensation or cramps.
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Stiffness in the neck.
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The patient has difficulty controlling functions like urination and defecation.
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Sudden headaches.
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Lack of coordination (unsteady body movements and walking style).
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In the later stages of the disease, weakness or numbness present in the legs may spread to the arms.
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Paralysis of legs.
How to Diagnose Foix-Alajouanine Syndrome?
The diagnosis of Foix-Alajouanine syndrome can be complicated as the symptoms of this disorder is similar to many other spinal cord disorders. The following methods are employed by doctors to diagnose Foix-Alajouanine syndrome:
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Medical History And Physical Examination: The doctor makes a detailed note of the symptoms experienced by the patient. Any signs of weakness and numbness of the legs experienced by the patient are noted. The doctor may check for factors like loss of balance and difficulties in walking.
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Spinal Angiography: This is the most reliable method used in diagnosing Foix-Alajouanine syndrome. This procedure is done to obtain clear information about the blood vessels present near the spinal cord. In this procedure, a thin, small tube called a catheter is inserted into the groin (the region between the hip and thighs). A contrast agent (dye) is injected through the catheter into the blood vessels. The images of the blood vessels are taken using an X-ray machine. This test gives a precise image of the arteries and veins present near the spinal cord that helps to find out the presence of spinal arteriovenous malformations.
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Laboratory Tests: Blood tests are done to check for vitamin B12 deficiencies, the presence of infections like acquired immunodeficiency virus (AIDS), Lyme disease (a bacterial infection that causes fever, headache, and tiredness), and tumors. These are done to discover the presence of disorders with symptoms similar to the Foix-Alajouanine syndrome.
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Magnetic Resonance Imaging (MRI): Magnetic resonance imaging of the spine uses powerful magnets, a computer, and radiowaves to get detailed images of the spine and the areas surrounding the spine. This imaging technique does not reveal any findings in the initial stages of the Foix-Alajouanine syndrome. However, in the later stages of the disease, MRI reveals findings like swelling of the spinal cord. Magnetic resonance imaging with the injection of a contrast agent (dye) like gadolinium helps to reveal the arteriovenous malformations and the presence of large, coiled blood vessels in and around the spinal cord.
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Computed Tomography (CT) Scans: Computed tomography of the spine uses a computer and X-rays to obtain different images of the spinal cord and the nearby structures. A contrast agent may be used in this technique, which may provide information about the abnormalities in the spinal arteries and veins.
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Myelography: Spinal myelography is a test performed by a radiologist with the help of X-rays and contrast dye to find out the problems in the spine, surrounding nerves, and tissues. A CT scan may be taken after the injection of dye into the spinal cord to get a clear picture of the spine abnormalities. In this method, findings like blood vessels extending over their normal positions in the spinal cord and tumors present in the nerve endings of the lowest part of the spinal cord can be found.
How Is Foix-Alajouanine Treated?
The different treatment options for the Foix-Alajouanine syndrome are as follows:
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Medications: There are no specific medications to cure the Foix-Alajouanine syndrome. However, medicines are given to relieve symptoms like back pain and stiffness. In cases where blood clots are present in the arteries or veins that may block normal blood flow, medications are given to disturb the blood clot.
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Surgery: Surgery is performed to remove the arteriovenous malformation of the spinal cord. In the conventional method, the surgeon cuts the area near the spinal cord to remove the arteriovenous malformation. Surgery is done only in cases where the arteriovenous malformation is small and is easily accessible without causing any damage to the spinal cord.
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Endovascular Embolization: This is the most preferred non-invasive method. In this method, a catheter is inserted into the artery present in the leg, which in turn is connected to the artery of the spinal cord that provides blood supply to the arteriovenous malformation. A glue-like substance is injected into the catheter, which enters the spinal artery and blocks the blood supply into the arteriovenous malformation. Even though this does not completely remove the malformation, it shrinks or damages it. This method can be done before the surgical method to reduce bleeding or decrease the size of the arteriovenous malformation during surgery.
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Radiosurgery: In this method, a specific amount of radiation is specifically directed to the target areas to destroy the blood vessels involved in the arteriovenous malformation. The blood supply to the malformation is stopped, which leads to the destruction of the malformation gradually. This is done in cases of small malformations that do not have the chance of bleeding.
Conclusion:
Arteriovenous malformations can occur anywhere in the body. However, arteriovenous malformations of the brain and spinal cord are very dangerous as they can lead to severe complications. However, if treated at an early stage, the person can be free from the symptoms and lead a normal life. Hence, early diagnosis and the right treatment are the key points in treating arteriovenous malformations of the spinal cord.
