10 Questions
What is the primary reason for sensory deficits in ALS patients?
Immobility due to muscle weakness
What is the percentage of ALS patients who show pyramidal tract involvement?
80%
Which of the following is a characteristic of muscle weakness in ALS patients?
Asymmetrical pattern
What is the percentage of ALS patients who complain of loss of exercise tolerance in the initial phase?
90%
Which of the following is NOT an exclusion criterion for ALS?
Fasciculation
What is the term for spontaneous laughing and/or crying in ALS patients?
Pseudobulbar palsy
Which of the following is a common bulbar symptom in ALS patients?
All of the above
What is the pattern of muscle weakness in ALS patients?
Distal to proximal
Which of the following is a early clinical marker of ALS?
All of the above
What is the primary site of onset in ALS patients?
Any of the above
Study Notes
Clinical Presentation of ALS
- Widespread muscle weakness leads to total paralysis
- Respiratory failure leads to death
- Faster degeneration in brainstem, cervical, and lumbar regions
- Limb involvement in men is more vigorous than in women
Five-Point Scale of Severity
- Stage 1: Mild disease
- Stage 2: Moderate disease
- Stage 3: Severe disease
- Stage 4: Non-functional movements
- Stage 5: Death
Prognosis of ALS
- Poor
- Death due to respiratory failure
- Death within 2-5 years after diagnosis
- Some patients may survive 5-20 years after diagnosis
Treatment (Rx)
- Medical Rx: No curative treatments, only minimal effective therapies
- Riluzole: Mildly better than placebo treatment for ALS
- Regular Vitamin-E intake: Has only minimal evidence
- Cannabis for spasticity reduction
- Multidisciplinary treatment can increase lifespan
Treatment for Muscle Spasm & Pain
- 73% of patients complain of pain in later stages
- Baclofen, NSAID, Dantrolene Sodium, Diazepam, Opioids
- Botulinum toxin injection
- Moderate exercise regimen reduces spasticity at 3 months
- Hydrotherapy with medications
- Spasm relief by stretching and increased movements
- Regular medication log needed to modify drug therapy
Treatment for Dysphagia & Dysarthria
- Dysphagia: Hydration to increase saliva viscosity to reduce aspiration
- Decongestants, antidepressants, and atropine type drugs
- Surgical removal of salivary glands or severing the PSN supply
- Percutaneous Endoscopic Gastrostomy (PEG) in severe cases
- Nutrition
- Dysarthria: Palatal lift prosthesis to reduce hypernasality
- Breathing exercises
Treatment for Respiratory Insufficiency
- Breathing exercises at early stage
- Thoracic mobility exercises
- Ventilator support for severe cases
- Oxygen therapy
- Tracheotomy
- Physiotherapy
Treatment Exercise Prescription
- Improve compliance
- Formal exercise program and enjoyable physical activity
- Include activities with opportunities for social and personal development
- Strengthening exercises: concentric > eccentric
- Endurance training not more than 15% minutes
- Adequate oxygen, hydration, and carbohydrate loads
- Avoid overwork exercise training
Physiotherapy Stage 1 Characteristics
- Mild weakness
- Clumsiness
- Ambulatory - CAN WALK
- Independent ADLs
- Rx: Continue normal activities, ROM, stretching, and yoga
- Add gentle strengthening exercises
- Provide psychological support
Physiotherapy Stage 2 Characteristics
- Moderate selective weakness
- Slightly decreased independence in ADLs
- Ambulatory
- Rx: Continue ROM, stretching to prevent contractures
- Continue cautious strengthening exercise
- Consider orthotic support
- Use adaptive equipment to facilitate ADLs
Physiotherapy Stage 3 Characteristics
- Severe selective weakness
- Moderately decreased independence in ADLs
- Easily fatigability with long-distance ambulation
- Ambulatory
- Increased respiratory effort
- Rx: Continue stage 2 program
- Continue ROM, stretching to prevent contractures
- Continue cautious strengthening exercise
- Consider orthotic support
- Use adaptive equipment to facilitate ADLs
- Encourage deep breathing exercises, prescribe wheelchair
Physiotherapy Stage 4 Characteristics
- Hanging arm syndrome with shoulder pain, edema in hand
- Wheelchair dependent
- Severe lower extremity weakness
- Increased respiratory effort
- Able to perform ADLs but fatigue easily
- Rx: Heat, massage, edema management
- Active assisted ROM exercises
- Encourage isometric exercise programs
- Arm slings, motorized wheelchair with arm rest
- Encourage deep breathing exercises
Physiotherapy Stage 5 Characteristics
- Severe lower extremity weakness
- Moderate to severe upper extremity weakness
- Wheelchair dependent
- Increasing dependence on ADLs
- Pressure sores
- Rx: Positioning, encourage family members to care for the patient
- Home modifications
- Electric hospital bed with antipressure mattress
- Active assisted ROM exercises
- Mechanical ventilation support
Physiotherapy Stage 6 Characteristics
- Bedridden
- Completely dependent on ADLs
- Rx: Dysphagia management: soft diet, tube feeding, PEG
- Positioning, encourage family members to care for the patient
- Suctioning, surgery
- Oxygen therapy
- Tracheotomy
- Electric hospital bed with antipressure mattress
- Medications to decrease impact of dyspnea
This quiz covers the clinical presentation and prognosis of Amyotrophic Lateral Sclerosis (ALS), including symptoms, stages of severity, and disease progression. Learn about the signs and diagnosis of ALS.
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