A. Scott Nielsen MD, MMSc
Virginia Mason Multiple Sclerosis Center
This is a difficult question to answer because what is a "typical" case of MS?
The time from the first symptom of demyelination to presentation in a doctor's office is going to depend heavily on where in the nervous system the injury is occuring. For instance, if the injury is occuring in the pyramidal system (this is the circuit that involves strength and movement), then patients are much more likely to seek care early as opposed to a patient who experiences their first attack of MS (that produces symptoms) if it affects the sensory fibers (for instance, if there is a small area of numbness or tingling). This is particularly the case in young adults who may rationalize symptoms away as something minor because MS (or any chronic illness) is not supposed to affect us in our most productive years.
With that said, there have been some researchers who have looked into the time from first symptom onset to diagnosis. A group in Spain (Fernandez et al., Journal of Neurology 2010; 257(9); 1500-7) did just this and found that the mean time from first symptom to diagnosis is approximately 2.08 years, and the largest contributor to this delay was patients who waited an average of 19.2 months from first symptom to going to the doctor for the problem.
Since the diagnosis of MS is a clinical one (ie, we do not have a blood test--or any other test for that matter--that can definitely render an accurate diagnosis), there will be occasions where the diagnosis may be uncertain for quite some time. Since MS can be mimicked by other disease states (in particular, diseases that are treated with therapies different than the MS therapies), it is very important to "get it right" to the extent possible. While this can be frustrating for patients who struggle with the uncertainty of a diagnosis, it is too for the physician. Obviously, more research is needed to identify biomarkers of the disease that can help us diagnose sooner (and with improved accuracy). This is imperative as clinical trial evidence indicates that the sooner a person with MS is treated, the better the long-term outcomes.
A. Scott Nielsen MD, MMSc
Virginia Mason Multiple Sclerosis Center
By Revere (Rip) Kinkel MD
Director of the Multiple Sclerosis Program
Professor of Clinical Neurosciences
University of California San Diego
We spend so much time discussing alterations in sight and tactile sensations (e.g. pins and needles, burning sensations, electrical sensations, itching) in MS that we often forget that the other senses may be affected as well. I thought I would briefly discuss these for you today.
Acute hearing loss during the course of MS is relatively rare, although I suspect it occurs far more frequently than the reported 10 % incidence would suggest. Hearing loss at the onset of MS is even less frequent (1%) and often suggests another disorder masquerading as MS. Hearing loss may occur in isolation but is more readily associated with the multiple sclerosis when accompanied by other brainstem symptoms and signs such as vertigo, weakness or sensory disturbance on one side of the body, facial weakness or sensory disturbance sometimes opposite the affected side of the body, or incoordination of a limb. Unilateral hearing loss is more suggestive of an MS related cause, but MS may also rarely cause bilateral hearing loss. Many patients report symptoms such as tinnitus or fullness in the ear that may fall short of “hearing loss”, but still negatively affects auditory function and quality.
Hearing loss is almost always caused by inflammatory demyelination in the part of the brain called the brainstem where the auditory nerve (also called the eighth cranial nerve) enters the central nervous system, or in the auditory pathways within the brainstem. It is said that high frequency hearing loss is more common with involvement of auditory nerve entry zone and tract, whereas low frequency hearing loss is more common with involvement of the auditory brainstem pathways. When hearing loss occurs acutely in MS, it often recovers along with other symptoms of a relapse after treatment with high dose steroids. However, like other symptoms there are some people who recover very little and are left nearly deaf. Tests can be done to determine if the hearing loss is caused by a problem in the cochlea (the inner ear and therefore not caused by MS) or the brainstem (more likely related to MS) but these tests are rarely necessary in a person known to have MS who experiences hearing loss.
Altered smell and taste are reported more frequently in people MS (up to 2/3’s of people with MS may experience diminished smell perception and discrimination). Altered smell may even be a presenting symptom of MS, presenting either as diminished ability to smell (hypoguesia) or unpleasant abnormal smells (odors misinterpreted as smelling like gasoline, for instance). Rarely, patients will present with both altered smell and taste suggesting another problem such as a viral infection or a consequence of smoking. Altered taste can take many forms including inability to taste only sweet flavors, only sour or bitter flavors or both. The abnormal taste may affect only one or both sides and when it affects both sides the type of taste affected can differ on the two sides (i.e. sweet taste affected on one side and sour bitter tastes affected on the other side). In most situations people do not notice loss of taste when it affects only one side. Loss of or distorted smell is noticed with greater ease than loss of taste and often affects a person’s perception of smell as well.
The proportion of people with MS who experience a decrease in smell perception and discrimination increases with the duration of disease and is related to amount of demyelination (white spots on MRI) in the inferior frontal lobes and temporal lobes of the brain. Not infrequently the diminished sense of smell is associated with problems controlling emotions or behavior, staying on task, planning activities or thoughts and seeing them through to the end.
Both altered taste and smell have a large differential diagnosis. Common reversible causes of diminished smell include the common cold and other respiratory viruses, allergic rhinitis, chronic sinusitis, malnutrition and smoking. Other causes readily excluded by history, examination or imaging include head trauma, frontal lobe or suprasellar tumors, normal aging, Alzheimer’s disease and Parkinson’s disease. Diminished taste may also occur with common viral infections and with smoking but is particularly common with certain medications; for instance, most MS patients have noticed the bitter taste that occurs during the administration of high dose steroids to treat a relapse. Other correctable causes include vitamin B12 deficiency, zinc deficiency, and dry mouth either from medications or a disorder of salivary glands such as Sjogren’s syndrome.
So the next time someone tries to tell you that MS does not affect hearing, smell or taste pull out this blog and educate them. But never assume a problem affecting these senses is related to your MS without a thorough evaluation by your MS Specialist.
By Lori Ann Kostich M.S. CCC-SLP
Mandell Center for Multiple Sclerosis Treatment and Research
What is Dysphagia?
Persons living with Multiple Sclerosis (pwMS) may experience changes in their ability to swallow. The act of chewing food (mastication) may become more difficult, the actual act of swallowing may become more difficult, or it may become more difficult to comfortably chew and swallow certain foods. PwMS may experience symptoms of increased coughing during meals (“It went down the wrong pipe!”), having to “think” to begin the act of swallowing (after chewing), difficulty eating some foods because of dry mouth or difficulty eating some foods because the consistency of the food is too hard to chew. These symptoms of dysphagia may “come and go”, much like the symptoms of tingling or weakness in the arms and legs, leaving the pwMS to experience “bad swallowing days”. Or, the symptoms of dysphagia may be consistent, occurring daily at every meal.
The act of swallowing is a complicated process. Food must be mashed in the oral cavity (mouth) in preparation for swallowing. A bolus (the ball of mashed food) must be formed prior to the act of swallowing so the food goes down in one neat ball, allowing the food to pass throw the larynx and pharynx (the throat) in an organized manner. Sufficient strength in the tongue base is needed to propel the bolus down the esophagus (pipe to the stomach), gravity is not enough. Three valves must open and close in a timely, coordinated manner to allow for smooth passage of the bolus to the esophagus. Breakdown in any of the components listed, or a change in timing of the movement of the component can lead to symptoms of dysphagia. Cranial nerve involvement (decreased innervation of the tongue and structures in the throat secondary to lesion placement) as well as muscle weakness can contribute to the breakdown.
Coughing is not always a negative symptom of dysphagia. Coughing is a defensive mechanism that the human body has to clear substances in our system away from somewhere it doesn’t belong. Coughing will occur when a person’s body senses that something inappropriate (saliva, mucus, food, liquids) is headed down the trachea (windpipe) towards the lungs. When coughing occurs, the hard expulsion is the protective part of the cough, blowing any bit of food or other item away from the lungs. The inhale of the cough is the part of the cough that requires care, inhaling too hard can pull the “bit of food or other item” just blown away from the lungs back into the lungs, resulting in penetration (the substance goes to the level of the vocal cords or above) or aspiration (the substance passes the vocal cords and goes into the lungs). Substances that move past the vocal cords into the lungs can cause respiratory infection (aspiration pneumonia). In the less impaired population, aspiration pneumonia is relatively infrequent, as the pwMS is up and moving around, helping the body can manage the substance. The risk of aspiration pneumonia increases when the pwMS becomes increasingly physically impaired. It is as the pwMS becomes less active that swallow function needs to be more closely monitored.
However, pwMS report unexpected, significant, frightening coughing episodes, which occur infrequently, but occasionally result in necessity of the Heimlich maneuver. These coughing episodes occur without warning, and may leave the patient fatigued for the rest of the day, or for several days afterward. These coughing episodes are scary for the patient, and any family or friends who witness the event. If one of these events occurs, the pwMS should contact the physician managing the care of the pwMS, to allow the physician to decide if the pwMS should come to the office to be examined and/or what follow up is appropriate.
What to do-Exercises
What to do-Compensatory Strategies
Bolus Control (Management of the food inside the mouth)
Oral management of food prior to the swallow (chewing) is important to maximize swallow efficiency and safety on all food consistencies and at all levels or fatigue. PwMS have been shown to have weaker lingual (tongue) strength and reduced rate of lingual motion on repetitive tasks, especially in the tongue base. How well the tongue works can affect how efficiently the food is moved around in the mouth while it is being chewed to a soft, mushy consistency prior to swallow. How well the tongue works is also important as strength and timing of lingual function is critical to initiating the swallow in a coordinated manner, leading to the bolus moving past the opening to the lungs and into the stomach safely. Decreased lingual strength or coordination can lead to difficulty swallowing muscle meats (beef, chicken, pork), lettuces, foods with skins (apples, grapes), small foods (peas, corn, rice, nuts etc) and foods of mixed consistencies ( soups, pizza, sandwiches with many different ingredients etc).
What to do-Exercises
1. Tongue sweeps- sweep the tongue around the outside of the upper teeth, from molar to molar. Move the tongue across the teeth from right to left, then back from left to right. Repeat 15 times (or work up to 15 times, this can be tiring). Why? Being able to move the tongue accurately and completely throughout the oral cavity is necessary to manage food as its being chewed. This is a range of motion exercise to targeting this function.
2. Repeat the tongue sweeps around the lower teeth. Work up to 15 repetitions.
3. Tongue –in-cheek- Push the tongue hard against the inside of the cheek (like trying to push a hoe through it. First on the right, then on the left. Repeat 15 times. Why? Same reason as the tongue sweeps, good tongue motion can help manage the food as it’s being chewed.
What to do- Compensatory Strategies
1. Double Swallow- The oral cavity (mouth) should be clear after each bite of food is swallowed. If it is not, a second swallow may be needed to clear all the food from the mouth.
2. Liquid wash- The mouth should be clear after each bite of food is swallowed. If it is not, a small sip of liquid may be needed to clear all the food from the mouth after the swallow.
Dry mouth can also affect management of the bolus in the oral cavity. Many medications that are frequently and successfully prescribed to treat other symptoms associated with MS (bladder/bowel dysfunction and some medications for depression) have dry mouth as side effect. Having reduced saliva limits the pwMS’ ability to keep the bolus moist inside the mouth and allow the bolus to flow smoothly out of the mouth towards the stomach. Add to this the possibility of some tongue base weakness and the act of swallowing can break down and coughing or having to swallow multiple times for each bite of food. A symptom like dry mouth can make eating foods like pasta, bread, rice, muffins, cookies, or doughnuts difficult.
What to do- DO NOT STOP TAKING PRESCRIPTION MEDICATIONS INDEPENDENTLY. Medications are prescribed to provide a benefit, to treat some issue. Unfortunately, some medications come with side effects. It then becomes a decision to be made by the patient and the physician whether to stay on the medication, discontinue the medication, switch to another medication, or manage the side effect. Dry mouth is a side effect that can be managed.
What to do- Compensatory Strategies
1. Adjust diet to eat foods high in fluid content (melons or other fruit, soups)
2. Add moisture or fluid (dunk breads in coffee/tea/milk, add gravy to meats, dunk meats in apple sauce, add copious condiments to hot or cold sandwiches, add jelly or butter to breads/muffins), to make the bolus less dry.
3. Bite – sip- chew- mash- swallow- This means take a bite of whatever food, take a small sip of liquid AT THE SAME TIME, chew and mash it all together, then swallow. The goal is to make the bolus in the mouth as mushy as possible. A mushy bolus will slide down better than a dry bolus.
Referral to a Speech Language Pathologist
Speech Language Pathologists evaluate, develop treatment plans and train exercises to reduce the symptoms of dysphagia (swallowing problems).Initial referrals should be made for an evaluation by a Speech Language Pathologist to be evaluated in an outpatient setting because some swallowing difficulties such as providing training to address dry mouth can be treated in the office setting. At that visit, the Speech Language Pathologist can request additional objective tests be performed such as a Modified Barium Swallow Study (MBS or MBSS) or a Fiberoptic Endoscopic Evaluation of Swallowing (FEES). Referral for a dysphagia evaluation by a speech language pathologist should be made when the pwMS:
Every pwMS with an EDSS of 7.5 or higher should have a yearly swallow evaluation.
What happens during a dysphagia evaluation by a Speech Language Pathologist?
During a dysphagia evaluation the pwMS will be asked a series of questions about what the specific problem with the swallow is, medical history, and what medications being taken. An oral motor examination will be completed, which means the SLP will look at how the structures in the mouth move (such as lips and tongue), how prompt the pwMS can produce a single dry swallow (with no food) and how strong the pwMS can produce a cough. Sensation (numbness) will be checked on the lips, tongue, and the inside and outside of the cheeks. The pwMS will then be asked to eat foods of various consistencies, such as water, pudding, soft fruit, crackers and or bread. The Speech Language Pathologist will watch the pwMS eat, and look for the potential reason for the concerns/symptoms the pwMS has. A treatment plan will then be developed that may include exercises (the tongue is a muscle just like the bicep, it can be exercised) compensatory strategies or follow up instrumental/objective evaluation (MBSS or FEES). The pwMS will be expected to apply the recommendations made by the Speech Language Pathologist and report if there is improvement in swallow function at home or in restaurants.
What is a Modified Barium Swallow Study (MBSS)?
A MBSS is an objective swallow study that evaluates a pwMS’ swallow by watching the swallow happen on moving x-rays that takes pictures of the swallow in real time as the swallow happenes. The pwMS will be asked to drink and eat different consistencies of food and liquid that have traces of barium or other substance that will show the organization and movement of the bolus as it moves through the oral cavity and into the throat (pharynx) during the swallow. The MBSS is run by a radiologist and a Speech Language Pathologist. The Speech Language Pathologist will be looking at organization of the bolus, lingual motion and motion of the muscles in the throat to try to identify the reason for the patient’s concern/symptoms. Unfortunately, since the MBSS is a “snapshot” of a series of single swallows, if significant coughing is the reason for the referral, it may not happen on the day of the MBSS which may be frustrating for the pwMS, it is important not to try to “create” the symptom. A report will be written and sent to the treating Speech Language Pathologist, who can then adjust the treatment plan as appropriate.
While participating in the MBSS, the pwMS should ask to see the pictures. Ask to have the screen turned, and have the images explained. Most Speech Language Pathologists are happy to do this and answer any questions, but do not always think to do so. Seeing how the food is moving through the mouth and throat, and being able to see where “breakdown” in the swallow can happen will make the swallow less of a mystery and less concerning.
Search MBS/MBSS/Swallow Study on YouTube- a number of individuals have posted their own results for everyone to see.
What is a Fiberoptic Endoscopic Evaluation of Swallowing (FEES)?
A FEES is an objective swallow study to evaluate a pwMS’ swallow in real time by passing a camera through the nose, and “parking” the cameral at the level of the roof of the mouth (this is NOT the type of endoscopy where the camera is passed down to the stomach or into the lungs). The pwMS will be asked to eat and drink different foods of different consistencies of food and liquids. This is regular food, though the liquids will be tinted with food coloring to allow for better visibility to the camera. During this type of swallow study, the Speech Language Pathologist can watch the passage of the food and liquid through the video camera and see how the food moves over the back of the tongue and whether there is pooling or residue left anywhere in the swallow after the swallow. The Speech Language Pathologist is looking for any issues that would cause the patient’s complaints or concerns. This type of study is a “snapshot” of a pwMS’ swallow function, though it can continue longer than an MBSS due to the fact that there is no radiation involved. A FEES may be better suited to some patients who report that symptoms begin as a meal progresses, maybe as fatigue sets in. A pwMS should never try to “create” the symptom. The evaluating Speech Language Pathologist will generate a report that will be sent to the treating Speech Language Pathologist who can then adjust the treatment plan as appropriate.
When participating in a FEES evaluation, the pwMS should ask to see the pictures and the images of the swallow study. It helps in understanding what is happening during the swallow if the pwMS can see how the food is moving and where “breakdown” may be happening.
Search FEES on YouTube- there are studies posted for everyone to see.
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