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Amyotrophic Lateral Sclerosis (ALS)

Name of disease:

Amyotrophic Lateral Sclerosis (ALS)

ICD-10 diagnosis code:

G12.21

Causes:

ALS is a disease caused by motor neuron damage. So far, scientific research has only found that the disease may be related to genetic and environmental toxins.

Pattern of inheritance:

About 10% of patients with ALS are genetic, and motor neuron atrophy is caused by mutations in the TARDBP gene.

Prevalence:

The median age of the patients was 62.2 years, and the ratio of males was higher than that of females, with a ratio of 1.75:1.

Diagnosis:

Diagnosis mainly relies on medical history, symptoms and various related tests, such as nerve conduction tests, electromyography, MRI, and lumbar puncture.

Age of onset:

The median age of the patients is 62.2 years old, and very few patients with familial inheritance may have onset as early as 20-30 years old.

Common signs and symptoms:

Weak ankles or legs that make oneself difficult to climb stairs Slurred speech Dysphagia Poor grip strength Muscle twitching Weight loss Thin extremities Inappropriate crying, laughing or yawning

Available treatments (medicinal and non-medicinal):

The only approved drugs for ALS are Riluzole in new oral suspension (Teglutik) or traditional pill form (Rilutek®), and Edaravone (Radicava™). Doctors will use creatine, vitamin E, vitamin C, COX-II inhibitors or calcium ion antagonists to relieve symptoms according to the patient's condition.

Disease management tips:

Onset phase: Neurologist will perform detailed EMG, nerve conduction velocity, MRI and other necessary examinations to confirm the diagnosis. Phase affecting working in the workplace: Diagnosed by a neurologist and assessed by a physical therapist, providing necessary medical and physical therapy to help control the disease. Phase affecting daily functioning: The patient should retain independence assistive devices such as supports or braces, orthotics, speech synthesizers, and wheelchairs Dysphagia phase: Requires insertion of a feeding tube and percutaneous gastronomy tube for feeding. Digestion or absorption problems and aspiration pneumonia caused by nasogastric tube feeding need to be prevented. Dyspnea phase: If a patient chooses tracheotomy due to dyspnea, he or she will need a ventilator to provide complete care. Appropriate respiratory care treatment is the most important for patients in this period.

References:

https://www.pharmacytimes.com/view/fda-approves-riluzole-oral-suspension-for-treatment-of-als https://www.hk01.com/ https://www.hksnmd.org/%E9%81%8B%E5%8B%95%E7%A5%9E%E7%B6%93%E5%85%83%E7%97%85/ https://www.rdhk.org/post/data?mid=15&id=519&lang=hk

Acknowledgment

This page is contributed by Lee's Pharmaceutical (HK) Ltd.