Multiple Sclerosis and Amyotrophic Lateral Sclerosis Flashcards
What is Multiple Sclerosis (MS)?
MS is one of the most common non-traumatic neurological conditions affecting young and middle-aged adults, characterized by discrete areas of damage in the brain, spinal cord, and optic nerves.
How common is MS in Canada?
Canada has one of the highest rates of MS per capita in the world. Most people are diagnosed between ages 20 and 49, with the average age being 43.
Who is more likely to be diagnosed with MS?
75% of people in Canada living with MS are female, and females are three times more likely to be diagnosed than males.
What body functions are most commonly affected by MS?
Vision, coordination, speech, strength, sensation, bladder control, and sexual function.
Is MS a terminal disease?
No, MS is not terminal, and most people live normal lifespans. However, it can greatly affect quality of life, with an unemployment rate of 60-80% among those with MS.
What is the primary contribution of massage therapy for people with MS?
Easing secondary effects of the disease in the body’s soft tissue, which can impair mobility and function and reduce quality of life.
What is myelin?
Myelin is a proteolipid sheath covering most axons in the CNS, made by oligodendrocytes. It insulates neurons, prevents impulse mixing, and speeds up conduction of nerve signals.
What is demyelination?
Demyelination is the process by which myelin sheaths are lost or destroyed. MS is the most common demyelinating condition in the CNS.
What are plaques in MS?
Plaques are characteristic lesions of MS, appearing as greyish spots in white matter where myelin destruction occurs. They average 1-15 mm in size.
What happens at an active plaque site?
Active plaques are edematous inflammatory lesions with infiltrating lymphocytes and macrophages. These immune cells consume myelin, attack oligodendrocytes, and rapidly kill them.
What is the role of astrocytes in plaque formation?
Astrocytes synthesize fiber processes to form a living scar (gliosis) at the plaque site, making the tissue harder than the surrounding area.
What characterizes old, inactive plaques in MS?
Old plaques are fields of permanently demyelinated axons filled with glial fibers in spaces where myelin bundles used to be.
What is an exacerbation in MS?
Exacerbation, also called a relapse or flare-up, is a period of active demyelination with sudden onset of neurological symptoms, lasting 6-8 weeks and resolving over 2-3 months.
What is remission in MS?
Remission is the period between exacerbations, which may return a person to normal or near-normal function, or leave considerable new disability depending on the disease stage.
What is the average frequency of MS exacerbations?
The average rate of exacerbations is every eighteen months.
What causes MS exacerbations?
MS exacerbations are caused by an autoimmune reaction where immune cells accumulate in CNS venules, breach the blood-brain barrier, and attack myelin, leaving behind plaques.
Where are MS plaques typically found?
MS plaques are denser around blood vessels and the ventricles. They may be new or reactivations of old sites.
How do immune cells attack in MS?
Immune cells release chemicals that cause inflammation and damage to myelin, neurons, and oligodendrocytes, perceiving myelin as an antigen and producing antibodies against it.
What happens when myelin is stripped away?
Uncovered axon sections transmit poorly or erratically due to lost insulation, causing dysfunction in pathways dependent on those signals.
What role do oligodendrocytes play during MS remission?
Oligodendrocytes remyelinate axons, often restoring normal or near-normal function in the early stages of MS.
Why does remyelination become less effective over time in MS?
Repeated attacks and cumulative damage reduce oligodendrocyte effectiveness, eventually leading to their dysfunction or disappearance.
What replaces oligodendrocytes in chronic MS plaques?
Glial scars (astrocytic fibrillary gliosis) fill the plaques, as astrocytes produce glial fibers after inflammation decreases and microglia disappear.
What happens when nearby plaques join in MS?
Larger fields of tissue form where impulse conduction is disrupted, leading to more severe symptoms and deficits.
What determines the severity of neurological deficits in MS?
Axonal damage/loss and the development of permanent inactive plaques are major determinants of profound neurological deficits.
When are exacerbations in MS more likely to occur?
Exacerbations are more likely if the person’s overall health is impaired, or during periods of illness.
Do all individuals with MS experience a well-defined course of exacerbations and remissions?
No, some have an extreme form of the disease with little or no remission, while others may only have one or two exacerbations in their lifetimes or experience a low-grade chronic type.
How does the progression of MS affect the distinction between flare-ups and remissions?
As the disease progresses, the distinction between states of flare-up and remission becomes less clear.
What are the possible disability outcomes in MS?
Disability outcomes range from clinically insignificant to total physical disability, with a variety of acute, subacute, and inactive lesions scattered throughout the CNS.
What is the suspected cause of MS?
MS is believed to result from a combination of immunological, environmental, infectious, and genetic factors.
How is MS succinctly described in terms of its cause?
MS is an immune-mediated neurological disorder precipitated in a genetically susceptible individual by exposure to an environmental factor, likely a virus.
Which genetic factor is strongly linked to MS development?
Changes in HLA genes, especially the HLA-DRB1*1501 variation, are strongly linked to increased MS risk.
Which immune cells play a significant role in MS?
T cells and B cells are the key immune cells involved in the abnormal immune response in MS.
What happens when T and B cells enter the CNS in MS?
Once in the CNS, T and B cells release chemicals that cause inflammation and damage.
How many genes have been identified that contribute to MS risk?
About 200 genes contribute a small amount to the overall risk, including HLA-DRB1*1501, the most strongly associated variation.
What is the risk of developing MS in the general population, and how does it compare for identical twins?
The risk in the general population is about 1 in 750–1,000. For identical twins, if one has MS, the other has about a 1 in 4 risk.
Which ethnic group has the highest prevalence of MS?
White people of Northern European descent have the highest prevalence of MS.
How does geography influence the risk of developing MS?
MS occurs more frequently in areas farther from the equator. People moving before age 15 assume the risk of their new area; those moving later retain the risk of their origin area.
Why might vitamin D levels be linked to MS risk?
People living closer to the equator have higher vitamin D levels due to greater sun exposure, which may support immune function and protect against MS.
How does obesity influence the risk of developing MS?
Obesity in childhood, adolescence, or early adulthood increases the risk of MS and may contribute to inflammation and MS activity.
How does smoking affect MS risk and progression?
Smoking increases the risk of developing MS, speeds up disability progression, and worsens relapsing MS. Stopping smoking slows disability progression.
Which virus significantly increases the risk of MS if contracted during adolescence?
Epstein-Barr Virus (EBV), the most common cause of infectious mononucleosis, more than doubles the risk of MS.
What role does the gut microbiome play in MS risk?
Changes in gut bacteria have been linked to MS, with certain bacteria strains elevated in individuals with MS, contributing to immune system dysfunction.
How do viral infections relate to MS exacerbations?
30–60% of MS exacerbations occur shortly after viral illnesses, such as colds or flu, suggesting that viruses can reactivate the disease.
Does stress play a role in MS onset or relapses?
A relationship is considered possible but not strongly demonstrated. Stress can drain energy, making symptoms more noticeable during demanding times.
Why is MS notoriously difficult to diagnose?
MS has non-specific symptoms that come and go, making diagnosis time variable from days to years. Often, it’s given as a probable diagnosis until clinical behavior over time confirms it.
What role can massage therapists play in diagnosing MS?
They can observe patterns of symptom development and recurrence, suggesting neurological consultation when MS is suspected.
What are the diagnostic criteria for MS?
- Evidence of damage in at least two separate CNS areas.
- Evidence that damage occurred at different times.
- Rule out other possible diagnoses.
What tools help speed the MS diagnostic process?
The Revised McDonald Criteria (2017), MRI, cerebrospinal fluid analysis, and evoked action potential (EP) testing.
What is the clinical presentation of MS symptoms?
Symptoms vary widely between individuals and fluctuate over time. Onset and progression are unpredictable, often starting in lower limbs and progressing asymmetrically.
Which visual symptom is most common in MS, and how does it present?
Optic neuritis, presenting as unilateral vision loss, light sensitivity, and pain behind the eye, often with a central blind spot (scotoma).
What are other common visual symptoms in MS?
- Blurred vision
- Double vision (diplopia)
- Impaired color perception
- Nystagmus (rapid, horizontal eye movement)
- Oscillopsia (jumping images)
- Internuclear ophthalmoplegia (failure of one eye to adduct while the other abducts)
Is total blindness common in MS?
No, but many individuals with MS are legally blind.
What are the first symptoms to appear in about half of MS cases?
Somatosensory symptoms, including sensory reduction (hypoesthesia, anesthesia), heightened sensitivity (hyperesthesia), and abnormal sensations (paresthesias and dysesthesias).
What is the “MS Hug”?
A squeezing sensation around the torso, often described as feeling like a blood pressure cuff tightening. It is a common first symptom or sign of relapse in MS.
What is Lhermitte’s Sign in MS?
An electric shock or buzzing sensation down the back, sometimes into the extremities, when the head is flexed forward. It indicates dorsal column involvement.
What are common motor symptoms of MS?
- Problems with walking (e.g., ataxia/dystaxia or drunken sailor’s gait)
- Tremors, especially intention tremor
- Tone disorders, ranging from hypotonia to spasticity
- Painful cramps and spasms
- Quadriplegic spastic paralysis in advanced stages
- Hyperactive stretch reflexes, clonus, and Babinski’s Sign
What are frequent motor-related subjective feelings in MS?
Clumsiness, stiffness, and “heaviness” in limbs, often accompanied by vertigo.
What facial motor symptoms are common in MS?
- Facial myokymia (rapid flickering facial muscle movements)
- Bell’s Palsy (facial paralysis due to cranial nerve VII dysfunction)
- Dysphagia (difficulty swallowing)
What are the primary speech problems in MS?
Dysarthria (difficulty controlling speech muscles) and scanning speech (monotonal, disconnected speech).
What are the characteristics of MS-related fatigue?
- Occurs daily, often in the morning after restful sleep
- Worsens throughout the day
- Aggravated by heat and humidity
- Comes on suddenly and is severe
- Often unrelated to depression or physical impairment
What are primary emotional symptoms in MS?
- Emotional lability (pathological laughing or weeping)
- Depression during exacerbations
- Euphoria in advanced stages, leading to indifference to health and loved ones
What secondary emotional symptoms are common in MS?
Feelings of depression or anger related to the unpredictability of the disease and life adjustments.
Which cognitive functions are often affected by MS?
- Information processing
- Memory
- Attention and concentration
- Executive functions
- Visuospatial abilities
- Verbal fluency
What urinary, digestive, and sexual symptoms are associated with MS?
- Constipation
- Urinary frequency, urgency, and incontinence
- Sexual dysfunction
How can kidney damage occur in individuals with MS?
Kidney damage may result from abnormal reflexes impairing bladder emptying, leading to recurrent urinary tract infections.
Why does sensitivity to heat affect people with MS?
Heat interferes with transmission along already impaired nerve fibers, worsening neurologic symptoms. This effect is temporary but can be intense.
What types of pain are common in MS?
- Ischemic muscle pain due to rigidity and spasticity
- Pain from abnormal postures and altered gait
- Trigeminal neuralgia
- Dysesthesia, an abnormal burning pain from non-painful stimuli like touch
What are seizures in MS caused by, and how common are they?
Seizures result from abnormal electrical discharges in injured or scarred brain areas. They occur in 2-5% of people with MS, slightly higher than the 3% incidence in the general population.
What secondary symptoms of MS are particularly significant for massage therapists to address?
- Fibrous contractures from low or poor usage of limbs
- Poor circulation in bedridden patients, hypotonic/spastic muscles, or dependent edema
- Back pain from altered gait or prolonged sitting
- Degenerative joint changes from altered biomechanics
- Compression and overuse syndromes (e.g., tendinitis from wheelchair or walker use)
How can massage therapy benefit individuals with MS?
- Minimize effects of fibrous contractures
- Improve circulation in affected muscles
- Alleviate back pain and address biomechanical stresses
- Treat compression and overuse syndromes
- Rehabilitate soft tissue injuries from frequent falls
What is Clinically Isolated Syndrome (CIS)?
CIS is the first episode of neurologic symptoms caused by inflammation and demyelination in the CNS, lasting at least 24 hours. It is characteristic of MS but does not meet the criteria for diagnosis.
What increases the likelihood of a second episode and MS diagnosis after CIS?
The presence of MS-like lesions on an MRI indicates a high likelihood of a second episode and diagnosis of relapsing-remitting MS (RRMS).
What is relapsing-remitting MS (RRMS)?
RRMS is the most common MS type, with defined exacerbations followed by partial or complete remissions. Approximately 85% of people with MS are initially diagnosed with this type.
What is secondary progressive MS (SPMS)?
SPMS follows RRMS and involves progressive worsening of neurologic function and accumulation of disability over time.
What is primary progressive MS (PPMS)?
PPMS involves worsening neurologic function and disability from symptom onset without early relapses or remissions, accounting for approximately 15% of MS diagnoses.
What is Amyotrophic Lateral Sclerosis (ALS)?
ALS, also known as Lou Gehrig’s disease, is a degenerative motor neuron disease leading to muscle weakness, paralysis, and the inability to swallow, speak, and breathe.
What is the prognosis for ALS?
There is no cure, and 80% of people with ALS die within two to five years of diagnosis.
What factors contribute to the degeneration of motor neurons in ALS?
- Faulty protein transport in nerve fibers
- Faulty apoptosis
- Mitochondrial dysfunction
- Excitotoxicity from excess glutamate
- Neuroinflammation
What are the suspected causes of ALS?
ALS likely results from interactions between genetic and environmental factors, including smoking, exposure to neurotoxins, heavy metals, military jobs, and concussions.
What is the difference between familial and sporadic ALS?
- Familial ALS: Accounts for less than 10% of cases, with a 50% chance of inheritance per child.
- Sporadic ALS: Over 90% of cases, mostly with underlying genetic susceptibility, affecting twice as many males as females.
How is ALS diagnosed?
ALS is diagnosed by eliminating other diseases using tests like blood/urine studies, EMG, NCV, MRI, muscle/nerve function tests, pulmonary function test (FVC), and genetic testing.
What are some early symptoms of ALS?
Tripping, dropping things, difficulty with fine motor tasks, slurred or “thick” speech, muscle cramping, weakness, and twitching.
What is spinal/limb onset ALS, and which areas are usually affected first?
It is the most common ALS type, where symptoms begin in either the arms or legs but not both.
What are the lower limb symptoms of spinal/limb onset ALS?
Stumbling, tripping, difficulty running, and foot drop.
What are the upper limb symptoms of spinal/limb onset ALS?
Decreased finger dexterity, cramping in the arm or hand, weaker hands, and arm/hand stiffness.
What symptoms indicate lower motor neuron (LMN) degeneration in ALS?
Muscle weakness and atrophy, fasciculations, cramps, hyporeflexia, flaccidity, dysphagia, dysarthria, and dyspnea at rest.
What symptoms indicate upper motor neuron (UMN) degeneration in ALS?
Muscle stiffness, hyperreflexia, and emotional lability.
What is bulbar onset ALS, and what percentage of patients does it affect?
Bulbar onset ALS affects 20-25% of patients and begins with symptoms like dysarthria, dysphagia, hypernasal speech, laryngospasm, and sialorrhea.
How does bulbar onset ALS progression differ from spinal/limb onset?
Bulbar ALS progresses faster and often involves limb weakness later in the disease.
What cognitive changes occur in ALS?
30-50% of patients develop cognitive impairment, and 15-20% develop ALS-FTD, which affects rational thought, impulse control, and personality.
What are common autonomic symptoms in ALS?
Rarely orthostatic hypotension or dizziness but advanced cases may have blood pressure fluctuations and tachycardia.
What are the general symptoms of ALS progression?
Dyspnea/apnea, dysphagia/aphagia, dysarthria/anarthria, muscle fatigue, weight loss, insomnia, excessive saliva, or dry mouth.
What assistive devices are typically needed as ALS progresses?
Mobility devices, feeding tubes, mechanical ventilation, and speech assistive devices.
What are the most common causes of death in ALS patients?
Aspiration pneumonia and respiratory insufficiency.
What emotional challenges are common in ALS patients?
Depression and anticipatory grief about losing independence, changing social roles, missing activities, and facing death.
What are the primary goals of ALS treatment?
To slow disease progression, provide comfort, and promote independence as long as possible.
What strategies are used to combat weakness and fatigue in ALS patients?
Fall prevention strategies, energy conservation techniques, orthoses, and adaptive equipment.
What is the average survival time for ALS patients, and how can it be extended?
Average survival is 3-5 years post-diagnosis, but longer survival (up to 10 years) is possible with care from multidisciplinary ALS clinics.
How can massage therapy help ALS patients?
By addressing the effects of the disease in the body’s soft tissues and providing comfort, especially in palliative and hospice phases.
What medications are available for ALS treatment in Canada?
Two medications slow progression and can extend survival by 3-6 months; other medications manage disease effects.
What are the key differences between MS and ALS?
MS:
- Myelin destruction in CNS affects sensory and motor neurons.
- Majority is exacerbation-remission type.
- Affects females more.
- Not terminal (shortens life by ~7 years).
ALS:
- Motor neuron disease affecting voluntary NS.
- Diagnosed later in life (ages 40-70).
- Inheritable.
- Less common (~4,000 Canadians affected).
