Sneddons syndrome


Sneddon's syndrome

Description, Causes and Risk Factors:

Sneddon's syndrome is a noninflammatory arteriopathy in which livedo reticularis is associated with cerebrovascular disease. It is slowly progressive and may go undiagnosed for some time. It may be seen in patients with an autoimmune disorder, e.g. antiphospholipid syndrome or systemic lupus erythematosus (SLE).

Sneddon's Syndrome is a rare, though underdiagnosed, vascular disease that affects women more often than men. Its primary symptoms are “Livedo reticularis” (a bluish-purple, net-like mottling on the skin that often gets worse in the cold) and transient neurological episodes that can be very severe. Some patients with Sneddon's have TIA's (“transient neurological attacks”) which they will sometimes describe as “episodes” or “spells”, during which they might have confusion, speech difficulties, dizziness, collapse, weakness, numbness or tingling on one side of the body, drooping on one side of the face, or vision problems. Some Sneddon's patients have full strokes. Many Sneddon's patients have problems with memory or concentration and these sometimes develop into dementia even in younger patients.

Sneddon's syndrome is not a homogeneous disease entity. Patients should be classified as "primary Sneddon's syndrome" if no etiologic factor can be detected. On clinical grounds, this from differs from several varieties of "secondary Sneddon's syndrome" which occurs mainly as part of an autoimmune disorder or in a thrombophilic state.

The exact cause of Sneddon syndrome is yet to be discovered. But there have been theories as to the disease being caused by immunological, genetic, and environmental factors. It has been observed that a family member who is a carrier of this disease can possibly pass it on to his offspring. But then again, the disease won't show in the child unless it is triggered by certain circumstances.

Sadly, little is known about exactly how Sneddon's Syndrome develops and progresses in the body. Because it has been known to occur among siblings or other family members most researchers believe Sneddon's Syndrome is genetic. Some researchers have suggested that it is a lifelong deterioration of the linings of the blood vessels (or a proliferation and drifting of the material that lines the vessels), and in this sense Sneddon's is a progressive disease. Once the vessels have deteriorated to a certain point the patient will develop clotting abnormalities in the blood and this is Sneddon's primary threat. Some researchers believe that when clotting begins to occur Sneddon's also causes vascular spasms in the brain similar to those we see evidenced in the skin as livedo reticularis.

Research: Researchers have studied the relationship between the histocompatibility class I and II antigens and Sneddon's syndrome (SS) in a Spanish patient with SS and her relatives (13 available members of an extensive 3-generation pedigree with diverse autoimmune hypercoagulation abnormalities). The patient and her father were diagnosed with a primary antiphospholipid antibody syndrome and were HLA-A30-B13-Bw6. In addition, a HLA-Bw6-DQ1 association was present in all the members of this kindred. These data suggest that the combination of the histocompatibility class I and II antigens in this family may be a marker for predisposition to SS.

Sneddon's has been reported to occur with a frequency of four cases per million people. It is generally believed, however, that this figure is not a real measure of how often the disease occurs, but rather of how rare it is for doctors to be familiar enough with the disease to pursue it as a diagnostic possibility. That is to say that there are a great many people who currently suffer from Sneddon's Syndrome but have been given a diagnosis of “anxiety”, no diagnosis, or an actual misdiagnosis.

Symptoms:

The patient with Sneddon's syndrome will experience chronic headaches. They would also feel dizzy from time to time and it is associated with the sense of being disoriented with his surroundings. Most patients will also develop hypertension. The patient's blood pressure will be abnormally high and it can't be associated to anything the he did or had eaten.

Several heart diseases can also be considered as a symptom of the Sneddon syndrome. And since this disease affects the brain, patients will also have episodes of stroke, which could range from mild to severe. Doctors usually refer mild strokes to be mini-strokes. Skin lesions, also known as infarcts, may also develop in some patients. This is because the disease affects the central nervous system (CNS). The overall reduction in the body's blood flow is the direct reason for these lesions.

And along with skin problems and other overt symptoms, patients will also observe that their mental capacity is reduced and their memory becomes weak over time. These symptoms are referred to as the neurological effects of this disease. Difficulty in memorizing and remembering names are the best examples of the disease's neurological symptoms.

Diagnosis:

To accurately diagnose this disease, doctors request patients to undergo certain tests such as magnetic resonance imaging (MRI). An MRI is considered to be the most effective diagnosing tool for this disease because it can produce a vivid image of the heart and the brain. Other tools such as a CT scan and a cerebral arteriography are also used. All of these tests are performed with the intention of revealing lesions as well as blockages in the patient's brain.

Skin biopsies may also be performed. This procedure involves the surgical removal of a tissue so that a microscopic study of it may be conducted. A biopsy of the skin could confirm an arteriopathy. Arteriopathy is a progressive type of an arterial disease. This is one of the primary characteristics of the Sneddon syndrome.

Treatment:

There is yet to be a defined treatment method for this disease. As of this moment, its treatment depends greatly on the symptoms as observed on the patient. And all the while, the necessary support systems are also being developed so that the patient will be able to live his life as normally as possible despite the disease.

The drugs administered to patients are usually anticoagulants, vasodilators, and immunosuppressive drugs. Anticoagulants are drugs that prevent blood clots and ensure that the normal flow of blood is facilitated. The common examples of this drug are warfarin and aspirin. Vasodilators, on the other hand, are drugs that dilate or widen the blood vessels. This effectively reduces the occurrence of high blood pressure in the patient. An example of this drug is nifedipine. Immunosuppressive drugs are used to regulate the elevated intensity of antiphospholipid antibodies in the patient's blood. This condition is very common in people with Sneddon syndrome.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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