MS & ALS Flashcards
Multiple Sclerosis basic info
Lifelong chronic neurological condition Age of onset 20-50 (15 youngest) Gender – women childbearing age Autoimmune disease -Demyelination -Loss of central nervous system axons Multitude of symptoms -“Multiple scars” accumulate on the brain and spinal cord -Mobility impairment one of the first signs -85% reporting gait disturbance
MS disease process
- “Immune mediated disease” b/c don’t know what is actually getting attacked
- Will feel sharp pain, burning from disruption of signals along myeline/nerve
- Patches that body makes – similar to putting electrical tape around electrical wires
- “multiple” and “sclerosed” plaques
MS symptoms: sensory
numbness, disturbed pain sensation, hypersensitivity, heat sensitivity, impaired position sense, paresthesia
MS Symptoms: Motor
- Motor: weakness, spasticity, hyperreflexia, ataxia, intention tremors, fatigue, clumsy, foot drop, gait
- Bowel/Bladder dysfunction
- Speech problems, dizziness, tremors
MS Symptoms: Visual
Double vision, Pain behind eyes, Blurred vision, Blind spots, Nystagmus
MS Symptoms: Cognitive
Approximately 50% Memory loss/ disturbance, Decreased attention, Executive function disorders. Usually mild
MS Symptoms: Psychological
Depression/euphoria, Impulsivity, Lability (emotional, crying)
MS Diagnosis
- Diagnosis: decreases in CNS (abnormal reflexes), changes in vision (evoked potentials) may suspect
- Head MRI
- Spine MRI
- Lumbar Puncture
- Neurological Exams
- History of at least two “attacks” separated by a period of reduced or no symptoms for RRM
Types of MS
Four main types:
RR, SP, PP, PR
Relapsing-Remitting MS
-85% relapse, remit
– a lot of symptoms, decreased or completely resolved, then another flare up
-(steady increase in peak/valley line)
Secondary-Progressive MS
-Peak/valley some, then gradual decline in function – increase in symptoms (peak/valley at first, then straight line)
Primary-Progressive MS
- 10% of MS patients
- constantly increasing in symptoms (straight line)
Progressive-Relapsing MS
- slight improvements but continually progress
- (steep peak/valley line)
MS Mobility
- Ability to move freely is affected
- Upper and lower extremity
- Upper extremity important to ADL’s and gait
MS Upper Extremity Impairments
- Not routinely assess in individuals with MS
- Need for valid and reliable outcomes
- May be smaller less noticeable changes
- Integration and targeted interventions
MS Prognosis
- 60% remain fully functional after 10 years
- 30% remain fully functional after 30 years
- More than 60% are ambulatory after 25 years
- Usually does not signify decrease in life expectancy
MS: Common Problems an OT May Address
- Fatigue (80%) !!!!!!!
- Muscle weakness
- Gait, Balance, & Coordination Problems
- Numbness (face, body, extremities)
- Vision Problems (double vision, poor contrast, eye pain, or heavy blurring)
- Dizziness and Vertigo
- Tremor or Ataxia
- Cognitive Components
- ADLs, personal hygiene (Debilitating, can’t do the simple things)
- Generally people do better in the morning & not as much in afternoon
Upper Extremity Tests Used in the MS Population
-Impairment - Fugl-Meyer (motor, sensory, coordination, reflexes)*
-Fine motor performance - 9 hole peg test**
-Gross motor performance - Box-&-block**
Combination fine and gross - Wolf motor function test* - & ARAT*
-Functional and task-oriented - Modified Jebsen test of hand function - TEMPA
General OT Treatment for MS
- Occupational performance modification (task, tools, ENERGY CONSERVATION)
- Adaptive devices, techniques as needed
- Conditioning/strengthening as tolerated
- Cognitive compensation (day planners, pill boxes, electronic devices)
- Visual compensatory techniques (contrast, large print, minimize eye fatigue, special glasses)
- Pain/spasticity management (stretching, modalities)
- Tremor/Ataxia intervention (proximal stabilization or support; task modification; adapted equipment; orthoses)
- Home, workplace modifications
- Patient/caregiver education, resources
OT Utilization by Clients with MS
- Literature suggests only 30-40% of client with MS have ever had OT services
- Those most likely referred to OT have: greater severity of disease; poorer mobility levels; more severe musculoskeletal problems; greater difficulties in multiple functional areas
- Areas of occupation requiring the most assistance (patient report): going up/down stairs; getting in/out of tub or shower; heavy housework; yard work
MS Exercise Programs
- For many years, patients with multiple sclerosis have been advised not to participate in physical exercise due to:
- some patients were reported to experience symptom instability during exercise as a consequence of increased body temperature
- Need to preserve energy: leaving more energy for ADL’s
- Best solution: gradual, unhurried, progressive daily activity and exercise.
- OT’s can facilitate the development of a balanced schedule.
ALS =
=Amyotrophic Lateral Sclerosis =Lou Gehrig’s disease -Fatal, late onset neurodegenerative disease UMN and LMN -Age of onset – 40-60 -Gender – males more than women (3:2) -Prevalence – one of most common
Motor Neuron Disease
- Group of progressive neurological disorders that destroy motor neurons, the cells that control voluntary muscle activity including speaking, walking, breathing, swallowing and general movement of the body.
- Have cognitive ability
Motor Neuron Diseases (NMD)
- Amyotrophic Lateral Sclerosis (ALS) – UMN & LMN
- Primary lateral sclerosis (PLS) – UMN
- Progressive muscular atrophy – LMN
- Bulbar palsy (pseudobulbar and progressive bulbar palsy) – cranial nerves 9-12
- Spinal muscular atrophy (SMA) – wasting of muscles – genetic
Why is it called ALS?
- “Amyotrophic” comes from Greek roots that mean “without nourishment to muscles”
- “Lateral” means “to the side” and refers to the location of the damage in the spinal cord.
- “Sclerosis” means “hardened” and refers to the hardened nature of the spinal cord in advanced ALS.
UMN vs. LMN
Upper motor neuron:
- Babinski reflex
- Overactive reflexes
- Spasticity
- General weakness
Lower motor neuron:
- Atrophy/ weakness of extremities
- Twitching, cramps/Fasciculations seen under the skin
Corticobulbar Tract
- Dysphagia - difficulty swallowing (choke)
- Dysarthria - impaired quality of speech production (lose ability to talk eventually)
Epidemiology of ALS
- Incidence: 4-8/100,000; 5,600 new cases/year
- Sociodemographic profile:
- Midlife: Onset 40-70 y/o; avg 58 y/o
- Whites
- Males (20% more common)
-ALS is responsible for 2 deaths/100,000
annually
Etiology of ALS
- Approximately 90% occurs spontaneously of unknown etiology (anyone, anytime)
- 5 – 10% have familial ALS –(mutation of SOD 1 gene)
Testing Procedures for Diagnosis of ALS
Diagnosis of exclusion
- electrodiagnostic tests including electomyography (EMG)
- blood and urine studies
- spinal tap
- x-rays, including magnetic resonance imaging (MRI)
- myelogram of cervical spine (x-ray of spine)
- muscle and/or nerve biopsy
- thorough neurological examination
Prognosis of ALS
- About 50% of people with ALS live 3 or more yrs;
- 20% live 5 or more;
- 10% live 10 or more.
- Most die of respiratory failure in 3-5 years
- Cognition, occulomotor function, sensory skills, bowel and bladder function usually not impaired
Six Stages of ALS
1: Ambulates, I w/ADL, mild weakness
2: Ambulates, moderate weakness in some muscles
3: Ambulates, severe weakness in some muscles
4: WC, almost I w/ADL, severe weakness LE’s
5: WC, dependent ADL, severe weakness UE/LE
6: Bedridden, maximum assistance required
ALS Clinical Manifestations: Initial
- Small muscles of hands and feet often 1st affected
- Hand weakness/atrophy, asymmetrical foot drop
- Problems using the hands for activities such as dressing, washing and buttoning clothes
- Problems walking (pulling feet up, trips/falls)
- Night cramps
- Speech/swallowing problems
ALS Clinical Manifestations: Progressing to..
- Increasing weakness in muscles of extremities, head/neck, trunk
- Loss of Voluntary movements of arms and legs
- Increasing difficulty with speech, swallowing and chewing
- Difficulty breathing - ventilation support
ALS Medical Management
- Riluzole
- Only FDA approved drug for tx of ALS
- Modest benefit in increasing survival time
- Slows the disease process
- Stem cell/gene therapy is promising…
- Symptom management:
- Spasticity/tone (Baclofen)
- Night time breathing machines
- Alternate feeding methods
- Depression
- Pain
Newer ALS Treatment Methods
-BCI = Brain Computer Interface – electrodes placed in brain – cap on head or implanted – can stare at letters & communicate that way
General ALS Management Goals
- Provide information about the disease process, treatment options, and stay up-to-date with best practices
- Promote functionality and independence
- Communicate effectively with all other healthcare providers involved in the care plan to ensure seamless, well coordinated care
- Give people with ALS emotional support, hope, encouragement, and attention (time) in an accepting environment
- Respect the treatment preferences and priorities of people with ALS
- Involve family caregivers – provide and refer support as necessary
- Make ongoing assessments to guide timely referrals
- Suggest ways to manage the activities of daily living in a safe, efficient, and comfortable manner
- Assist in selecting, acquiring, and fitting assistive devices
- Liaise with community agencies that offer support and assistance accessing necessary equipment, funding and services
- Be open to discussions about death and dying, including reviewing advance directives and end-of- life care plans
The ALS Healthcare Team
- PCP
- Neurologist
- OT
- PT
- Speech Language Pathologist
- Dietician
- Gastroenterologist
- Respiratory Therapist
General OT Intervention for ALS
- Occupational performance adaptation
- Psychosocial adjustment
- Community participation skills
- Caregiver training
- Functional mobility adaptation
- Assistive technology (home, work, community)
- UE management (activity/exercise, splints, coordination)
- Joint protection, pain management, energy conservation
- Positioning, safety, skin integrity
