Actress Selma Blair has announced that she has been diagnosed with multiple sclerosis. The 46-year-old Cruel Intentions actress made the announcement on : "I have #multiplesclerosis. I am in an exacerbation." She goes on to say: "I am disabled. I fall sometimes. I drop things. My memory is foggy. And my left side is asking for directions from a broken gps."
Blair also expressed her gratitude to Netflix producers for their understanding and support in her upcoming role as Harper Glass in the sci-fi drama Another Life. "By the grace of the lord, and will power and the understanding producers at Netflix, I have a job. A wonderful job."
Blair says she was diagnosed on August 15th of this year, but she believes that she has had symptoms for at least 15 years. She originally thought she had something simple, like a "pinched nerve." A friend finally talked her into seeing her brother, a Los Angeles neurologist. During the visit, she fell in front of him, leading to an MRI, which revealed the diagnosis.
Blair says she is coming forth now, despite being in the thick of it, because, "I hope to give some hope to others. And even to myself. You can't get help unless you ask ... I have probably had this incurable disease for 15 years at least. And I am relieved to at least know. And share."
What is Multiple Sclerosis?
Multiple sclerosis (MS) is a neuroinflammatory disease that affects myelin, nerve cell bodies, as well as the axons in the brain, spinal cord, and optic nerve. The term multiple sclerosis refers to the distinctive areas of scar tissue (sclerosis or plaques) that are visible in the white matter of people who have MS.
According to the , MS is thought to affect more than 2.3 million people worldwide. It most often appears between the ages 20 to 40, however, it can also affect children and older people. As with most autoimmune disorders, twice as many women are affected by MS as men. MS is more common in colder climates; the further from the equator, the higher the risk.
Susceptibility to MS may be inherited. Studies of families indicate that relatives of an individual with MS have an increased risk of developing the disease. Experts estimate that about 15% of individuals with MS have one or more family members or relatives who also have MS. But even identical twins have only a 1 in 3 chance of both having the disease. This suggests that MS is not entirely controlled by genes, and other factors must come into play.
What causes MS?
The ultimate cause of MS is damage to myelin, nerve fibers, and neurons in the brain and spinal cord. But how that happens, and why, are questions that challenge researchers. Evidence appears to show that MS is a disease caused by genetic vulnerabilities combined with environmental factors.
Immunologic Factors
Although there is little doubt that the immune system contributes to the brain and spinal cord tissue destruction of MS, the exact target of the immune system attacks and which immune system cells cause the destruction aren't fully understood. Supporting evidence for immune system involvement in the pathogenesis of MS includes:
- Inflammatory T cells, B cells and macrophages typically seen in MS lesions in biopsies or at autopsy
- IgG and IgM oligoclonal bands found in CSF but not serum of patients with MS
- Gadolinium enhancement on MRI seen in early stages of patients with relapsing-remitting MS- characteristic of inflammation found with disruption of the blood-brain barrier
- T helper 17-type immune activation associated with active MS lesions
- Immunomodulatory drugs that reduce certain T cell regulatory pathways can reduce MS disease activity.
Environmental Factors
Geographic: As mentioned above, MS is known to occur more frequently in areas that are further from the equator. Furthermore, people born in one area, but who move to another area before the age of 15, assume the MS risk of the area to which they have moved.
Vitamin D and Sunlight: Several studies have suggested that people who spend more time in the sun and those with relatively high levels of vitamin D are less likely to develop MS. Bright sunlight helps human skin produce vitamin D. Researchers believe that vitamin D may help regulate the immune system in ways that reduce the risk of MS. People from regions near the equator, where there is a great deal of bright sunlight, generally have a much lower risk of MS than people from temperate areas such as the United States and Canada. Other studies suggest that people with higher levels of vitamin D generally have less severe MS and fewer relapses (see ).
Infectious factors and viruses
Several viruses have been found in people with MS, but the virus most consistently linked to the development of MS is Epstein Barr virus (EBV).
, "only about 5% of the population has not been infected by EBV. These individuals are at a lower risk for developing MS than those who have been infected. People who were infected with EBV in adolescence or adulthood and who therefore develop an exaggerated immune response to EBV are at a significantly higher risk for developing MS than those who were infected in early childhood. This suggests that it may be the type of immune response to EBV that predisposes to MS, rather than EBV infection itself. However, there is still no proof that EBV causes MS."
What are the signs and symptoms of MS?
The symptoms of MS usually begin over one to several days, but in some forms, they may develop more slowly. They may be mild or severe and may go away quickly or last for months. Sometimes the initial symptoms of MS are overlooked because they disappear in a day or so and normal function returns. Because symptoms come and go in most people with MS, the presence of symptoms is called an attack or exacerbation. Recovery from symptoms is referred to as remission, while a return of symptoms is called a relapse. This form of MS is therefore called relapsing-remitting MS, in contrast to a more slowly developing form called primary progressive MS. Progressive MS can also be a second stage of the illness that follows years of relapsing-remitting symptoms (called secondary progressive MS).
Relapsing-remitting MS accounts for approximately 85%-90% of cases at onset. Secondary progressive MS usually occurs 10-20 years after the onset of the disease. Primary progressive MS is characterized by progressive disability from the onset of the disease with occasional plateaus or temporary minor improvements. The mean age of onset is 40.
A diagnosis of MS is often delayed because MS shares symptoms with other neurological conditions and diseases.
The first symptoms of MS often include:
- vision problems such as blurred or double vision or optic neuritis, which causes pain in the eye and a rapid loss of vision.
- weak, stiff muscles, often with painful muscle spasms
- tingling or numbness in the arms, legs, trunk of the body, or face
- clumsiness, particularly difficulty staying balanced when walking
- bladder control problems, with incontinence or urgency
- dizziness that doesn't go away
MS may also cause later symptoms such as:
- mental or physical fatigue that accompanies the above symptoms during an attack
- mood changes such as depression or euphoria
- changes in the ability to concentrate or to multitask effectively
- difficulty in making decisions, planning, or prioritizing at work or in private life
Other disorders found in patients with MS include:
- Transverse myelitis: a condition caused by inflammation in the spinal cord. Transverse myelitis causes loss of spinal cord function over a period lasting from several hours to several weeks. It usually begins as a sudden onset of lower back pain, muscle weakness, or abnormal sensations in the toes and feet, and can rapidly progress to more severe symptoms, including paralysis. In most cases of transverse myelitis, people recover at least some function within the first 12 weeks after an attack begins.
- Neuro-myelitis optica: a disorder associated with transverse myelitis as well as optic nerve inflammation. Patients with this disorder usually have antibodies against a protein in their spinal cord, called the . These patients respond differently to treatment than most people with MS.
- "Lhermitte's sign": an uncomfortable "electrical" sensation that runs through the back and into the limbs. The sensation can be ascending or descending. It is often elicited by bending the head forward.
How is MS diagnosed?
There is no single test used to diagnose MS. Several tests are often done to rule out or confirm the diagnosis. As there are many other disorders that can mimic MS, it is very important to perform a thorough investigation before making a diagnosis.
In addition to a complete medical history, physical examination, and a detailed neurological examination, an MRI scan of the head and spine should be done to look for the characteristic lesions of MS. In regions with active inflammation in MS, there is disruption of the blood-brain barrier and the dye will leak into the active MS lesion.
How is MS treated?
There is currently no cure for multiple sclerosis, however, there are currently several disease-modifying medications approved by the U.S. Food and Drug Administration (FDA) for use in relapsing forms of MS. They come in three forms:
Injectable (subcutaneous or intramuscular)
- : Interferons are signaling molecules that regulate immune cells.
- : Exactly how it works is not entirely clear, but research has shown that it changes the balance of immune cells in the body. It is approved only for relapsing forms of MS.
- Peginterferon beta-1
Oral
- : The drug prevents white blood cells called lymphocytes from leaving the lymph nodes and entering the blood and the brain and spinal cord.
Infused medications (Intravenous)
- : administered intravenously four times a year, has been approved for especially severe forms of relapsing-remitting and secondary progressive MS. This drug has been associated with development of certain types of blood cancers in up to 1% of patients, as well as with heart damage. Therefore, this drug should be used as a last resort to treat patients with a form of MS that leads to rapid loss of function and for whom other treatments did not stop the disease.
- : In March 2017, the FDA approved ocrelizumab (brand name Ocrevus) to treat adults with relapsing forms of MS and primary progressive multiple sclerosis.
- : works by preventing cells of the immune system from entering the brain and spinal cord. It is administered intravenously once a month. It is a very effective drug for many people, but it is associated with an increased risk of a potentially fatal viral infection of the brain called progressive multifocal encephalopathy (PML).
Disease-modifying medications have been shown to:
- Reduce the frequency and severity of clinical attacks (also called relapses or exacerbations)
- Reduce the accumulation of lesions (damaged or active disease areas) within the brain and spinal cord
- Appear to slow down the accumulation of disability
Severe relapses are usually treated with a 3-5-day course of high-dose, intravenous corticosteroids, often followed by a slow taper of oral prednisone.
Michele R. Berman, MD, and Mark S. Boguski, MD, PhD, are a wife and husband team of physicians who have trained and taught at some of the top medical schools in the country including Harvard, Johns Hopkins, and Washington University in St. Louis. Their mission is both a journalistic and educational one: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.