Physiotherapy for ALS

ALS (also known as Lou Gehrig’s disease) is a disorder that causes death of neurons that control voluntary muscles. It is progressive neurodegenerative disorder that causes muscle weakness, disability and death. There is no cure currently for ALS.

Signs and Symptoms:

• Progressive muscle weakness is the hallmark feature.
• Initially, muscle weakness starts in the legs or arms but then spreads to other muscle groups. There is no associated pain.
• Other symptoms include muscle cramps, muscle twitching, problems with speaking and swallowing, weight loss, breathing difficulties.
• Symptoms do not include loss of bowel/bladder control, sensation, cognitive function, extra-ocular muscles, and sexual function.
• Initially patients have problems breathing with activities and movement, and then eventually patients have problems breathing when resting.

Causes:

ALS has no known cause in 90-95% of cases (sporadic mutation). 5-10% of the cases are inherited. Within the spinal cord, the main region affected is the cortical spinal tracts; this area controls the movements of the limbs and trunk.

Risk Factors:

• Established: Age, family history
• Accumulating evidence: Cigarette smoking.

Who gets it?

• Caucasians more likely when compared to African, Asian and Hispanic ethic groups.
• Incidence of ALS increases every 10 years after age 40, and peak age is 74. Mean age of onset is 62 years.
• Male: Female ratio is 1.3:1.5 but is 1:1 after age 70.

Diagnosis:

There are no specific diagnostic tests for ALS. EMG and nerve conduction studies can help show loss of function. Diagnosis is not certain unless the changes are found in at least 3 spinal regions (cervical, thoracic, lumbosacral or face, throat, tongue, jaw). Motor conduction velocity is usually normal (or slightly low), and sensory conduction studies are normal. Biopsy can show neurological changes in the wasted muscles.

Treatment:

• Symptom based management:
  • Respiratory Management: PFT, noninvasive (PPV – patients facemask should be ideal) or invasive ventilation (for long term survival). After initial diagnosis is made, patients should know the options for respiratory care such as tracheostomy and chronic ventilator support. If patients choose to forgo ventilation, anxiolytics or opiates should be prescribed to help breathing and anxiety.
    • Patients should undergo PFTS (Pulmonary function testing) every three months starting at the time of diagnosis.
  • Immunizations:
    • Annual seasonal influenza vaccination and pneumococcal vaccination is recommended.
  • Trouble swallowing:
    • Initial treatment is to change the consistency of the food that the patient is eating.
    • Use of percutaneous gastrostomy tube placement may be helpful, but there is no evidence about PEG affecting quality of life.
  • Trouble speaking:
    • Speech therapy is not helpful, use other methods of communication.
  • Muscle Spasm:
    • Levetiracetam, carbamazepine and phenytoin.
  • Muscle Weakness:
    • Assistive devices (canes, crutches, walking frames) can be used in early course of disease.
    • Some data indicates that stretching and strengthening exercises are helpful. Home physiotherapists can provide the appropriate rehabilitation in this instance.
  • Riluzole – Only drug that has impact on survival in ALS. Recommended to have riluzole 50 mg twice daily for patients with ALS.

Prognosis:

Most ALS patients only survive within three to five years of diagnosis. 30% of patients live five years after diagnosis and 10-20% can survive greater than 10 years. Longer survival is noted in patients who experience symptoms at younger age, male gender, and only limb involvement.

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