AMYOTROPHIC LATERAL SCLEROSIS

Amyotrophic Lateral Sclerosis (ALS) is a condition that affects the basic functional unit of the central nervous system, which are the neurons. This type of disease is believed to result from an interplay of both heredity and environmental factors.

The disease is common among individuals 50 years old and above but can be found in the younger population with no sexual predominance. A typical presentation of this disease condition includes mild weakness described as a heavy feeling of the affected side to the more severe respiratory distress syndrome.

TYPES

Amyotrophic Lateral Sclerosis is classified depending on the area of the neuron involved: either upper-lower motor neurons. It can either be one of the following:

 

  • Typical ALS consists of the brain ( upper motor neuron group) and the spinal cord (Iower motor neuron) and is the most common type and accounts for approximately 90% of the total ALS cases.
  • Primary Lateral Sclerosis involves the lower motor neuron segment or the spinal cord. Accounts for only 5% of the total number of ALS cases and patients with this type of ALS have a better prognosis because it has a slower disease progression compared to the typical ALS.
  • Progressive muscular atrophy (PMA) affects approximately 5% of ALS cases and involves the legs and the arms. Patients with this type have more prolonged survival, and symptoms usually last for several years before becoming full-blown.

 

SYMPTOMS

 

The initial presenting symptoms of ALS is a weakness but can either be any of the following:

  • Difficulty in walking or ambulation
  • Frequent stumbling and taking miscalculated steps
  • The inability to sustain the handgrip sometimes complained as the frequent inability to continue holding a water glass.
  • Disparity on the display of emotions such as breaking into a laugh in serious conversations
  • Several patients report inability to think clearly and frequent forgetfulness (not mistaken as an early stage of dementia)
  • Numbness and frequent tingling of the extremities.
  • Severe cases present with breathing difficulty, inability to chew and swallow bolus of food, and further progress to difficulty in swallowing water and other liquid.

DIAGNOSIS

Arriving at ALS diagnosis is very tricky since you have to narrow down your differentials to arrive at the specific working impression. ALS mimics other disease conditions such as cerebrovascular accidents (stroke), Parkinson’s disease (shuffling gait), and behavioral changes (an early symptom of dementia).

Nerve conduction studies may be requested by your physician to evaluate a specific muscle group’s response to the inflicted stimulus.

A complete blood count may be requested to rule out infectious causes of muscle weakness.

 

TREATMENT

The management of ALS involves a multi-system approach from the use of medications to physical rehabilitation employment. Treatment options are as follows:

  • Use of the drugs Riluzole and Edaravone, orally, and intravenous administration respectively, can minimize ALS progression.  No single therapy can reverse the effect of ALs on the muscles. Corroborative treatment can slow down the disease course.
  • Occupational and Physical therapy- enables the patient to carry out activities necessary for daily living.
  • Exercises or activities that increase lung capacity prevent the accumulation of phlegm in the lung parenchyma, which will become the source of recurrent infection.

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