amyotrophic%20lateral%20sclerosis
AMYOTROPHIC LATERAL SCLEROSIS
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with undetermined etiology that primarily involves the motor neurons in the cerebral cortex, brainstem and spinal cord.
There is no cure and the mean duration of survival is 2-5 years without tracheostomy and ventilator support.
Clinical hallmarks of ALS are: presence of upper & lower motor neuron features involving the brainstem and spinal cord and progressive limb weakness, respiratory insufficiency, spasticity, hyperreflexia, and bulbar symptoms such as dysarthria and dysphagia.

Supportive Therapy

Sialorrhea

  • Sialorrhea or drooling may cause social stress and is associated with aspiration pneumonia
    • Tends to be due to poor handling of saliva
    • Suctioning of secretions using a portable home suction device is recommended
  • Distinguish between thick mucus production and sialorrhea
  • Low-dose radiation therapy for medically refractory sialorrhea reduces salivary production, but may cause side effects (eg erythema, sore throat, nausea)

Bronchial Secretions

  • Provide a portable suction device and humidifier
  • Patient and caregiver should be taught the technique of assisting expiratory movements using a manual-assisted cough
  • A mechanical cough-assisting device (insufflator-exsufflator) may also be used for acute respiratory infection
  • Cricopharyngeal myotomy may be done for frequent cricopharyngeal spasms and severe bronchial secretions

Cramps and Spasticity

  • Physical therapy, exercise and hydrotherapy may help reduce cramps and spasticity

Pain (Musculoskeletal Pain, Skin Pressure Pain caused by Immobility)

  • Physiotherapy
  • Repositioning and pressure area care
  • Pressure-relieving cushions and mattress should be used

Venous Thrombosis

  • May be prevented by physiotherapy, limb elevation and compression stockings

Weakness and Disability

  • Physiotherapy and physical therapy may be used all throughout the course of disease
    • Exercise may increase muscle strength, endurance and range of motion
  • Orthotics (eg ankle foot orthosis, neck collars)
  • Adaptive aids (eg walking frame, wheelchair)

Cognitive Decline

  • In all patients with frontal dysexecutive syndromes, care needs to be taken to ensure informed consent during decision-making; capacity issues may need to be considered
  • Carers/healthcare professionals should be informed of the symptoms of dysexecutive syndrome and trained in their management

Palliative Care

  • Shared decision-making between physician and patient is needed
    • Religious and cultural values needs to be considered
  • Continued discussion throughout illness to understand and follow patient’s choices even if they change throughout their care
  • Goal is to provide adequate relief from symptoms at the terminal stage
    • Treat dyspnea, anxiety and pain as required
  • Consider hospice care, if available
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