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
Editor's Recommendations
Most Read Articles
17 Apr 2019
The ketogenic and Modified Atkin’s diets are effective treatments for children with refractory epilepsy of genetic aetiology, a new study suggests.
Pearl Toh, 17 Apr 2019
A contraceptive vaginal system (Annovera) releasing a combination of segesterone acetate and ethinyl estradiol (SA/EE) effectively inhibits ovulation up to a year even at low systemic levels of SA, reports a review study presented at ENDO 2019.
17 Apr 2019
Non-alcoholic fatty liver disease (NAFLD) – a clinicopathological spectrum comprising hepatic steatosis or non-alcoholic fatty liver (NAFL) and non-alcoholic steatohepatitis (NASH) – is the hepatic manifestation of metabolic syndrome recognized as a leading cause of liver morbidity and mortality. Dr Loo Wai Mun, Consultant at the Division of Gastroenterology and Hepatology, National University Hospital, Singapore, speaks with Audrey Abella to discuss how NAFLD can be best managed in primary care.
Jairia Dela Cruz, 2 days ago
Rupatadine at either 10 or 20 mg delivers superior improvements in nasal and ocular symptoms of seasonal allergic rhinitis compared with placebo, with both doses well tolerated by adult and adolescent patients, according to the results of a phase III trial.