What is Pediatric Multiple Sclerosis?
Multiple sclerosis (MS) is a chronic, inflammatory disorder in which immune cells inappropriately cause damage to the central nervous system (brain, spinal cord, and optic nerves). This primarily occurs in the form of demyelination or injury to the insulating coating of neurons. Early stages of the illness are characterized by acute onset of symptoms caused by inflammation, followed by partial recovery (relapsing-remitting multiple sclerosis). Over time, the disease can be associated with increasing disability in the absence of clear clinical attacks (secondary progressive multiple sclerosis). It is very rare to see progressive MS in pediatric patients.
What are the signs and symptoms of Pediatric Multiple Sclerosis?
MS most commonly involves demyelination in the brain, brainstem, optic nerves and spinal cord. Depending on the part of the nervous system affected, the signs and symptoms may vary, but common symptoms include weakness, numbness, vision changes, difficulty with balance and walking, and bowel/bladder dysfunction. Fatigue, pain and cognitive changes are also common symptoms.
How is Pediatric Multiple Sclerosis diagnosed?
The cornerstone of MS is central nervous system demyelination at multiple points in space and time. The 2017 Revised McDonald criteria represents the most recent iteration of formal diagnostic criteria for MS. While there are multiple ways to meet McDonald criteria for MS, general principles include having two or more lesions in sites of the central nervous system known to be affected by MS, and having at least two attacks of CNS demyelination or the presence of oligoclonal bands. Additionally, there should also not be a better explanation for a person's symptoms such as vascular, genetic and infectious disorders.
What are the causes of Pediatric Multiple Sclerosis?
Despite a significant amount of research investigating the underlying causes of MS, the exact cause is unknown. Most studies suggest that multiple sclerosis results when an individual who is genetically vulnerable to developing the disease encounters certain triggers in their environment in combination with certain nutritional and metabolic risk factors. Genetic factors include having specific types of genes involved in immune regulation. Environmental risk factors can include a low vitamin D level, a history of Epstein-Barr virus infection, obesity and a history of smoking.
How is Pediatric Multiple Sclerosis treated?
MS is treated with therapies aimed at decreasing the abnormal inflammatory response. This includes high doses of steroids, plasma exchange (PLEX), and intravenous immunoglobulin (IVIG). Steroids work by reducing the ability of inflammatory cells to enter the brain, interrupting their communication or interfering with their ability to make chemicals involved in inflammation. They can be administered orally or intravenously (IV). Most children do not have side effects, but some may experience mood changes, appetite changes, difficulty sleeping or irritability. Rarely, patients can experience infection or bone abnormalities. PLEX is used to clean the blood of inflammatory components. It requires special IV access and a portable machine to clean the blood. A patient’s blood is circulated through the machine and proteins such as antibodies and complement are removed. A therapy session takes about 90 minutes and is usually well tolerated. A patient usually receives five to seven treatments to complete a PLEX course. IVIG is made up of antibodies collected and pooled from blood donors that is thought to reduce the patient’s own abnormal immune response.
As MS is a chronic and relapsing disorder, prevention of new attacks relies on initiation of immunomodulatory/immunosuppressive medications. These are also sometimes referred to as disease modifying treatments (DMTs) and include therapies that can be given orally, by subcutaneous or intramuscular injection or infused through an IV. These therapies have differing mechanisms of action, safety risks and levels of effectiveness in preventing relapses. Selection of such medications is highly individualized and should involve consultation of a health care provider with experience in prescribing these medications.
In addition to evaluation by a neuropsychologist for cognitive, emotional and/or behavioral problems, patients may also require psychosocial support or therapy to manage the impacts of MS.