6- Amyotrophic Lateral Sclerosis Flashcards
- Definition & Diagnosis - Clinical Presentation - Prognosis - EDX Study - Dysphagia Rehabilitation: Risk Factor, Sign & Symptoms, Conservative & Interventional Management, Sialorrhea - Pulmonary Rehabilitation: Breathing, Cough & Secretion Management, Invasive (IV) & Non-Invasive Ventilation (NIV) - Motor of Rehabilitation - Psychology - Medication
Pathology of Amyotrophic Lateral Sclerosis (ALS) ππ
Amyotrophic Lateral Sclerosis (ALS)
Progressive neurodegenerative disorder characterized
Disease of UMN (Corticospinal & Corticobulbar tracts) and LMN (Anterior horn cells)
LMN βamyotrophicβ
Muscle denervation result in weakness, atrophy, and fasciculations
UMN βlateral sclerosisβ
Lateral corticospinal tracts sclerosis result in hyperreflexia and spasticity
Progressive bulbar palsy (Pure Bulbar Palsy)
- Dysarthria: Slurred speech, hoarseness, or decreased volume of speech
- Dysphagia: Aspiration or choking during a meal
Brachial amyotrophic diplegia (BAD)
- Upper limbs focus
Leg amyotrophic diplegia (LAD)
- Lower limbs focus
Progressive muscular atrophy (Full Body without Bulbar)
- Reduced finger dexterity and weakness or wasting of intrinsic hand muscles
- Wrist drop interfering with work performance
- Foot drop; patients may report a βslappingβ gait
- Tripping, stumbling when running
List 6 Amyotrophic Lateral Sclerosis-Plus Syndromes
ALS = Pyramidal .. think of other tracts
- Sensory loss
- Autonomic dysfunction
- Extrapyramidal signs
- Parkinsonism
- Ocular motility abnormalities
- Frontotemporal dementia (FTD)
What is the differential diagnosis of ALS? ππ MOCK
- Multifocal motor neuropathy (most common)
- Cervical cord/foramen magnum lesions (tumor, syringomyelia, syringobulbia, spondylosis)
- Vitamin B12 myelopathy
- Multiple sclerosis
- Spinal muscular atrophy
- Myasthenia gravis
- Thyrotoxicosis
Neurology Secrets 6th Edition Chapter 6 MND pg92
23 yr old male presents with bilateral hand weakness with upper and lower motor neuron signs, leg weakness and bilateral spasticity. What is diagnosis? ππ
- ALS
- Transverse myelitis
Canada Q Bank
Amyotrophic lateral sclerosis (ALS): Pathology, Affected vs Spared ππ
Pathology
- Degeneration of the anterior horn cell & corticospinal tracts
Affected
- Bulbar & Pseudobulbar: Dysphagia, Dysarthria, Emotional Liability, Sialorrhea, Dysphonia
- Thoracic: Respiratory failure
- Limbs: Asymmetric weakness, Atrophy & fasciculations
Spared (Face, BBS, Sensory)
- Sensation
- Extraocular muscles
- Bowel and bladder
- +/- Cerebellar
Cuccurollo 4th Edition Chapter 5 EDX pg447-448 Table 5-61
List 4 Causes of Bulbar vs Pseudobulbar palsy ππ
Common Sing & Symptoms π
Bulbar Dysfunction
- DYSPHONIA & Difficulty breathing (airway obstruction)
- DYSARTHRIA & Slurred speech
- DYSPHAGIA
- Oral: Difficulty in chewing, Drooling of saliva
- Pharyngeal: Nasal regurgitation.
- Oesophageal
Difference
- Flaccid (LMN) vs spastic (UMN) tongue and jaw
- Hypo (LMN) vs Hyperflexia (UMN) Jaw Jerk
- Emotional liabelity (pseudobulbar)
https://en.wikipedia.org/wiki/Bulbar_palsy
https://en.wikipedia.org/wiki/Pseudobulbar_palsy
Negative and positive factors affecting prognosis & Average survival. ππ MOCK
Positive Factors
- Percutaneous endoscopic gastrostomy (PEG) tube
- Non-invasive ventilation (NIV)
- Riluzole
- Multidisciplinary Clinics
Negative Factors
- Female
- Old age of diagnosis
- Definite diagnosis of ALS
- Bulbar Onset
- Rapid progression
Braddom 6th Edition Chapter 40 MND Box 40.5
Prognosis & Mobility of ALS π
Average survival
- 3-5 years from onset
- 15 months from diagnosis
Prognosis
- 50% die within 3 years
- 30% live for 5 years
- 10% live for 10 years
Wheelchair
- by 12β18 months
Cuccurollo 4th Eiditon Chapter 5 EDX pg446-447 Table 5-61
Braddom 6th Edition Chapter 40 Box 40.5
EMG Criteria for definitive ALS. ππ MOCK
Clinically definite ALS
- Active denervation with reinnervation must be found in three of four body segments (craniobulbar, cervical, thoracic, and lumbosacral).
- At least two muscles with different innervations should be abnormal
- LMN and UMN signs in three of four body segments
Clinically probable ALS
- Evidence of LMN and UMN signs in at least two regions
- PLUS UMN signs above the LMN signs.
Cuccurollo 4th Edition Chapter 5 EDX pg448 Table 5-61
Braddom 6th Edition Chapter 40 MND pg831 Box 40.5
ALS. Why they are considered at higher risk for malnutirion?
What are the Signs of Dysphagia? π
Risk Factor for Malnutirion
- Bulbar muscle dysfunction due to motor neuron involvement in the brainstem
- Fatigue and slow eating due to bulbar weakness β Increased time required to eat
- Difficulty transferring food to the mouth due to arm weakness.
- Depression
- Hypermetabolic state due to increased respiratory effort
Bulbar Involvement
- Dysarthria: Slurred speech
- Dysphonia: Hoarseness
- Dysphagia
- Oral Phase: Difficulty Chewing, Swallowing
- Pharyngeal Phase: Choking & Aspiration even with saliva
- Esophageal Phase: GERD, Vomiting
- Complications: Malnutrition, Aspiration pnuemonia & Weight loss
DeLisa 5th Edition Chapter 28 MND pg731
Braddom 6th Edition Chapter 40 MND pg836
Feeding Management in MND. ππ
FOOT
- Diet Type: Using thickener for liquids and soften moist solid food
- Diet Route: PEG tube with high caloric supplements
COMPENSATORY STRATIGIES
-
Low Risk Feeding Strategies
- Sit up at 90 degrees
- Small amounts
- Donβt mix solids and liquids (i.e., mixed consistencies)
- Supervision
- Remain upright 3o minute
- Focus on eating and eliminate any distraction (Tv)
-
Compensatory Techniques
- Chin tuck
- Head turn to weaker side
- Head tilt to stronger side
-
Swallowing Maneuvers
- Double, dry, effortful swallow
- Supraglottic & Supra-Supraglottic swallowing
-
Clearing Techniques
- Liquid mouth wash
- Manual removal by fingers
Dr. Maitham Note
Risks / Complications of PEG tube placement ππ
π‘ Overall risk of complications for PEG tube placement in patients with ALS is 6% to 10%
- Tube displacement
- Pneuomoperitoneum
- Hemoperitoneum
- Peritonitis
- Gastrointestinal bleeding
- Gastrocolic fistula
- Death
βMany patients decline a PEG tube because they are uncomfortable with the idea. This can be due to lacked information about the role and potential benefits of a PEG tube or mistaken by their swallowing feeling adequate. This often leads to a delay in the discussion, at which point the risk of placing the PEG increasesβ
Braddom 6th Edition Chapter 40 MND pg837
How to monitor feeding in ALS? ππ
When do you refer ALS patient for PEG insertion? ππ
MONITOR DYSPHAGIA
- Malnutrition
- Reduced caloric intake
- Weight loss more than 10% of bodyweight
- Dehydration
- Repeated aspiration pneumonia
- Choking
- Repeated aspiration pneumonia
EVERY 3 MONTHS
- Bodyweight measurement
- FVC >50%
PEG TUBE
- Once FVC <50%
- Moderate to high risk (operate vs palliative care)
Braddom 6th Edition Chapter 40 MND pg834 Box 40.4
DeLisa 5th Edition Chapter 28 MND pg732 Figure 28-7
Impact of Sialorrhea on Quality of Life, Managment & their S/E ππ
SIALORRHEA
- Anterior: Socially disabling & Oral infections
- Posterior: Choking and aspiration pneumonia
NON-PHARMA
- Suction machine
- Towel and verbal cueing
PHARMA
- Amitriptyline 25-50mg 3 times daily (Anticholinergic)
- Atropine drops 0.25 to 0.75 mg 3 times daily (Anticholinergic)
- Transdermal scopolamine (Anticholinergic)
- Botulinum toxin type A given every 3 months at a dose of 7 to 22.5 units into each parotid gland β jaw dislocation
INTERVENSION
- Radiation
- Surgical Excision
Braddom 6th Edition Chapter 6 MND pg837
Why ALS have comporomized respiratory function? ππ
BREATHING
- Bulbar Dysfunction βUpper Dysfunctionβ
- Sialorrhea
- Dysphaiga leading to aspiration Pneumonia
- Weak upper respiratory muscles leading to collpase of upper airways
- Respiratory Dysfunction βLower Dysfunctionβ
- Poor intercostal muscles leading to Inadequate ventilation β Atelectasis
COUGH
- Difficulty generating a strong cough
- Inability to take a deep breath
- Weak exhalation muscles
CLERANCE
- Inadequate mucus clearance
- Thick mucus due to anticholingeric agents, decreased intake of liquids and weakened cough
Dr. Maitham
List 5 Points of Rehabilitation for Restrictive Pulmonary Disease
-
PREVENTION
- Prevention of pneumonia, respiratory failure, intubation and mechanical ventilation
- Vaccinations for influenza, pneumococcal and COVID
- Avoid contact with sick people or crowded areas
- Avoid sedatives at night and the risk of possible aspiration
- Balanced diet to support respiratory function, immune system and healthy bodyweight
-
BREATHING
- Pursed lip breathing
- Diaphragmatic breathing
- Deep breathing and insufflations (Manual resuscitation bag, Ambu bag)
- Glossopharyngeal breathing: Each breath usually consists of six to nine air boluses (or up to 65), with each bolus consisting of 30 to 150 mL of air (usually 60β200 mL)
-
NON-INVASIVE VENTILATION
- Intermittent abdominal pressure ventilator (IAPV)
- Pneumobelt, Abdominal corset
- BiPAP
- Tracheostomy
- Intermittent abdominal pressure ventilator (IAPV)
-
COUGHIN
- Manually Assisted Cough
- Heimlich-type or abdominal thrust assist
- Costophrenic assist
- Anterior chest compression assist
- Abdominal binder
- Manually Assisted Cough
-
SECREATION
- Postural drainage
- Chest percussion
- Mechanical insufflation-exsufflation
- Suctioning
Cuccurullo 4th Edition Chapter 9 Pulmonary Rehabilitation pg697-699
Coarse of respiratory statues as ALS disease progress?
Caorse of respiratory dysfunction in ALS:
- FVC less than 50% of predicted should be offered the use of Noninvasive positive-pressure ventilation (NIPPV).
- Over time, bulbar symptoms that are severe enough that they will be unable to continue use of NIPPV because of failure of NIPPV to ventilate the patient effectively despite 24 hour/day use
- Consideration for invasive ventilation may be the only option for continued survival. Unfortunatly it no effect on the progression of the disease
- Patients will later develop complete paresis of all muscles including the extraocular muscles and develop a βlocked-inβ syndrome in which no communication is possible.
DeLisa 5th Edition Chapter 28 MND
Managment & Follow Up of Respiratory System in ALS/MND ππ
PREVENTION
- Pneumococcal vaccination
- Influenza vaccination
- Dysphagia assessment
FOLLOW UP Q3 MONTHS
- Peak Cough Flow (PCF) > 5 L/sec or >270 mL/min for effective cough
- PFT: Monitor FVC > 50% for functional breathing
- Respiratory Symptoms (Day and night time)
BREATHING
- Breath stacking: Segmental breathing
- Pursed-lip breathing
- Lung volume recruitment (LVR)
- NIV (BiPAP) if FVC <50%, nocturnal desaturation or respiratory symptoms
- Night Time Ventilation: nasal peice
- Day Time Ventilation: mouth peice
- Continuous 24 Hour: Day & Night symptoms
- IV (Tracheostomy)
- Severe bulbar weakness or inadequate ventilation even with NIV
- Pharmacology: Beta agonists & Anticholinergic bronchodilators
COUGHING
1. PCF < 270 mL/min
Manual assisted cough & huff coughing
2. PCF <160 mL/min or Bulbar Symptoms
Mechanical assisted cough βin-exsufflatorβ β Tracheostomy & Suction.
SECREATION
- Chest physiotherapy βPercussionβ
- Postural Drainage
- High-frequency chest wall oscillation
- Home suction device
- Room humidifier.
- Mucolytics such as N-acetylcysteine, 200 to 400 mg 3 times daily
- Nebulized saline
Dr. Maitham Summary
ALS. How does Respiratory insufficiency present? ππ
π‘ It can be applied for pulmonary rehab for any case
Day Time βPhysicalβ
- Morning headache
- Poor concentration
- Dyspnea
- Tachypnea
- Paradoxical breathing
- Fatigue
- Weak cough
- Drowsy
Night Time βSleepβ
- Orthopnea
- Nocturnal hypoventilation
- Insomnia
- Cyanosis
Physiological
- Polycythemia
- Pulmonary hypertension
Not all patients are sympatomatic
- The majority of patients with less than 50% predicted FVC will have mild or no respiratory symptoms.
- Experts recommend routine assessment of respiratory physiologic parameters in patients with ALS at every visit, regardless of the presence of symptoms.
Braddom 6th Edition Chapter 40 MND pg837
Explain to patient the Benefits of Pulmonary Rehabilitation ππ OSCE
PREVENTION
- Decreased hospitalization rates/reduced healthcare resource utilization
- Decreased anxiety and depression
BREATHING
- Increasing oxygen extraction O2 from arterial circulation.
- Conditioning peripheral musculature to reduce stress on the heart and lungs
- Reduction in dyspnea and respiratory rate (RR)
PERFORMANCE (ADLs - Ambulation - Exercise)
- Improvement in ADL performance and general quality of life
- Improvement in ambulation capacity
- Improvement in exercise tolerance, work output, mechanical efficiency
Cuccurullo 4th Edition Chapter 9 Pulmonary Rehabilitation pg649
How to monitor breathing in ALS patient? ππ
Breathing management in ALS ππ
When do you start NIV? ππ OSCE
MONITOR BREATHING
- Pulse Oximeter
- Sleep Study
- Nocturnal desaturations <90% of 1 cumulative minute
- Nocturnal desaturations 88% for 5 cumulative mins
- ABG
- Hypoxia SAO2 <92%
- Hypercarbia: start NIV if PaCO2 > 45 mmHg
- PFT
- Forced vital capacity (FVC) < 50%: Volume of air that can be forcefully and maximally exhaled after deepest inhalation.
BREATHING MANAGEMENT
Non-Pharma
- Breath stacking: multiple sequential inhalations, increase inspiratory volume and peak cough flow (PCF)
- Lung recruitment with self-inflating resuscitator bag
- NIV (BiPAP) if FVC <50%, nocturnal desaturation or respiratory symptoms
- IV (Tracheostomy) if severe bulbar weakness or inadequate ventilation
Pharma
- Beta agonists
- Anticholinergic bronchodilators
STARTING NIV
- Forced vital capacity (FVC) < 50%
- PCF < 270mL/min
DeLisa 5th Edition Chapter 28 MND
List 3 causes why individual with ALS may experience cough impairment. ππ
How to monitor cough in ALS patient? ππ
Cough management in ALS ππ
COUGH IMPAIRMENTS
Cough is recognised as a defence reflex mechanism, with three phases:
- Inspiratory Phase: diaphargm and external intercostal
- Compressive Phase: Forced expiratory effort against a closed glottis
- Expiratory Phase: Opening of the glottis, with subsequent rapid expiration
Cough impairment due to
- Reduced inspired volume due to diaphragm and external intercostal muscles weakness
- Inability to close the glottis completely during the compressive phase due to bulbar muscle dysfunction
- Inability to compress and expel intrathoracic gas because of expiratory muscle weakness
COUGH MONITORTING
1- Peak cough flow (PCF)
Start NIV if PCF < 270 L/min
Associated with poor cough and a high risk of respiratory infection.
2- Maximum inspiratory/expiratory pressure (MIP & MEP)
Start NIV if MIP < 60 mmH2O
Inacurate due to bulbar weakness (inadequate oral seal or upper airway collapse)
3- Sniff nasal inspiratory pressure (SNIP)
Correlates with hypercarbic respiratory failure
COUGH MANAGEMENT
PCF >270 L/min
- Manual insufflation with self-inflating resuscitator bag with breath stacking maneuvers.
- Manual cough-assist βHeimlich maneuverβ
PCF <270 L/min
1- Mechanical insufflation-exsufflation
Providing positive airway pressure for a few seconds, then suddenly switching to negative pressure to simulate the pressures created in a healthy cough.
How: 4 to 6 insufflation-exsufflation cycles, 30 seconds rest
Frequency: 3 to 4 treatments per day, increased for 1-2 times per hour in case of respiratory tract infection.
Beginning at +15 cm H2O insufflation pressure/.15 cm H2O exsufflation pressure, increased in 5 to 10 cm H2O increments, to a target +40/.40 cm H2O and oxygen saturation greater than 94%
DeLisa 5th Edition Chapter 28 MND pg728
Bronchial secretions management in ALS ππ
NON-PHARMA
- Chest physiotherapy βPercussionβ
- Postural Drainage
- High-frequency chest wall oscillation
- Room humidifier.
PHARMA
- Mucolytics such as N-acetylcysteine, 200 to 400 mg 3 times daily
- Nebulized saline
INTERVENSION
- Home suction device
DeLisa 5th Edition Chapter 28 MND
Cuccurollo 4th Edition Chapter 9 Pulmonary Rehabilitation
List 4 Benefits of Lung volume recruitment (LVR) π
- Reduce atelectasis (open airways)
- Mobilize secretions (clear mucus)
- Improve pulmonary hygiene
- Improves peak cough flows (air volume)
DeLisa 5th Edition Chapter 30 Myopathy pg773
Indication of non-invasive ventilation (NIV) in ALS ππ
VITALS - BLOOD - SPECIAL
- FVC <50% of predicted
- Nocturnal oximetry of 88% or less for five consecutive minutes.
- MIP β€60 cm H2O
- PaCO β₯45 mm Hg
Braddom 6th Edition Chapter 40 MND pg834 Box 40.4
List 4 Advantages of NIV π
PHYSIOLOGY
- Reduce energy requirement for breathing
- Slows the rate of respiratory decline
- Delays the need for tracheostomy
DAY-TIME
- Reduce physical fatigue
- Reduce dyspnea
- Reduce Somnolence
- Improve Concentration
- Improve Cognitive function
- Reduce Depression
NIGHT-TIME
- Improve Sleep quality
Considering oxygen therapy for neuromuscular disease? ππ
Oxygen therapy should almost never be used in patients with ALS. It worsens respiratory symptoms and hypercapnea and can lead to hypercapnic coma or respiratory arrest.
Braddom 6th Edition Chapter 40 MND pg834 Box 40.4
Motor Symptoms in ALS π
Upper Limb
- Reduced finger dexterity, cramping, stiffness
- Weakness or wasting of intrinsic hand muscles
- Difficulty with buttoning clothes, picking up small objects, or turning a key.
- Patients may develop wrist drop.
Lower Limb
- Tripping, stumbling when running
- Foot drop, βslappingβ gait
- Muscle atrophy, waddling gait, genu recurvatum
- Spasticity may compromise gait.
- Unsteadiness
- Painful joint contractures
List 4 causes of joint pain in ALS. ππ
- Pressure Ulcer β pressure management
- Neuropathic Pain β Amitriptyline or Neurontin (gabapentin)
- Spasticity β Anti-spasticity, Physical modalities
- Contracture β ROM
- Cramps β Acetaminophen (1,000 mg every 6 hours) or NSAID
Spasticity. Any precautions in ALS? ππ
Non-Pharmacological
- Physical therapy β Slow (30 second sustained), static muscle stretching
- Positional splinting (watch for pressure areas)
Pharmacological
- Oral Baclofen β 5 to 10 mg two to three times a day, titrating up to doses of 20 mg four times a day β S/E weakness, fatigue, and sedation.
- Benzodiazepines β major side effect is respiratory depression
- Sirdalud (Tizanidine) β 2 to 8 mg three to four times a day β S/E muscular weakness
- Botulinum toxin β make sure not to cause dysfunctional weakness
Surgical
- Intrathecal baclofen β successful in ALS
Braddom 6th Edition Chapter 40 MND
DeLisa 5th Edition Chapter 25 MND pg731-732
ALS patient referred for ALC, what would like to prescribe?
Thoracic
- Cervical orthoses for head drop
Upper Limb
- Wrist-hand orthosis for wrist drop
- Universal cuff for eating
- Grip bars, benches, reachers
Lower Limb
- Ambulatory: AFOβs, rollator walkers, canes
- Non-Ambulatory: Powered WC
- Good cushioning (gelfoam)
- Custom back support for the scoliosis.
Home Modifications
- Hospital bed (air or dense foam)
List 4 Bathing assisted devices for ALS.
Toilet
- Grab bars
- Commode chair
- Raised toilet seat
Bathing
- Hand-held showers
- Bath tub benches
Write PT, OT & SLP Plan for ALS in OPD visit.
PT
- ROM and Strength Ex (Sub-maximal, avoid fatigue)
- Aerobic Exercises
OT
- Energy conservation
- Hand dexterity (writing, cutting, dressing & hygiene)
- Transfer and ambulation β Wheelchair or sling
SLP
- Breathing exercises
- Dysphagia management (food modifications & exercises)
Benefits of exercise for ALS
- Beneficial effect on mood, psychological well-being appetite, and sleep.
- Fighting depression and improving pain tolerance.
- Improvement in functional ability, decrease in disability
- Reduction in the rate of decline
Exercise Prescription & List 5 Exercise Precautions for ALS ππ
Exercises Prescription
- Range of motion and stretching exercises are considered safe
- Aerobic exercises (Low impact like cycling)
- Warm pool therapy, walking mid-chest height.
- Mild-to-modest resistance exercises for 15 minutes twice daily.
- Resistance training has been shown to improve function and spasticity.
Precautions
- Fatigue
- Dyspnea
- Eccentric
- Cramps
- Prolonged DOMS
- Weakness post exercise
Braddom 6th Edition Chapter 40 MND pg834 Box 40.4
Mood and Cognitive Disorders in ALS & Tx
Cognitive Disorders in ALS
- Depression
- Anxiety
Treatment
- TCA: Amitriptyline 25mg daily (Tx Insomnia & Drooling)
- SSRI: Cipralex 10mg daily
Pharmacological treatment for ALS.
1- Riluzole
MOA: inhibits presynaptic release of glutamate
Dose: 50 mg twice daily
Adverse reactions: dizziness, asthenia, and gastrointestinal disorders, elevated LFTs
Routine monitoring of liver function tests is recommended.
2- Radicava