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.

Patient Education

Delivery of Diagnostic News

  • Ensure that diagnosis is given in person in a quiet, comfortable and private location
  • Patient’s family or caregiver should be present
  • Determine patient’s knowledge of amyotrophic lateral sclerosis (ALS)
    • Allow for questions and give reassurance
  • Respect cultural and social background of patient
  •  Provide written information about disease and support groups, if available
  •  Offer a second opinion, if the patient wants it

Reassurance

  • Explain complications are manageable
  • Involve patient in care decisions and reassure patient that their treatment decisions will be respected

Sharing of Information

  • Information needs to be provided to patient and family in a timely manner which is appropriate for decision making
    • Needs to be delivered well in advance of major management decisions (eg mechanical ventilation)

Genetic Counseling and Testing

  • Should be offered only in cases with known familial history of amyotrophic lateral sclerosis and sporadic amyotrophic lateral sclerosis cases with the characteristic phenotype of the recessive D90A-SOD1 mutation
  • Pre-symptomatic genetic testing should be offered in 1st-degree adult blood relatives of patients with known gene mutation
  • Testing should be availed voluntarily and with informed consent
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