What is ALS?

Amyotrophic Lateral Sclerosis (ALS) is a rapidly progressive neurodegenerative disease. Characterized by a gradual loss of upper and lower motor neurons (or motor neurons). These motor neurons, which control the movement of the voluntary muscles; they gradually decrease their functioning and die.

The consequence of the disappearance of motor neurons in the cerebral cortex; Brainstem and medulla is a muscular weakness that progresses seriously threatening motor autonomy; oral communication, swallowing and breathing. That is, patients begin to have problems with basic functions such as walking, swallowing, speaking, or getting out of bed.

ALS progresses gradually causing muscle atrophy and paralysis in almost every muscle in the body. Characteristically, this disease does not affect eye muscles, sphincter control, or skin sensitivity.

Nor does it usually cause damage to the cognitive capacity of the patient; although approximately 10-15% may present signs of frontotemporal dementia and in more than 35% of cases signs of cognitive impairment are detected.

Unlike what occurs in other neurodegenerative diseases, ALS is not characterized by producing a loss of consciousness or cognitive ability of the patient.

David Schick, an experienced neurologist and an associate clinical professor affiliated with multiple hospitals in the area, including NYU Langone Hospitals and Mount Sinai Hospital, addressed the complex needs of patients with amyotrophic lateral sclerosis (ALS) and other motor neuron diseases.

What are the symptoms of ALS?

Not all patients with ALS experience the same symptoms or the same sequences or patterns of progression, explains Dr. David Schick, a Neurology Specialist practicing in the New York area for over 11 years. While one person may continually trip on carpet edges, another may have difficulty lifting objects. The onset of symptoms will depend on the severity and location of the degenerative changes in the motor neurons of the brainstem and spinal cord.

Symptoms of ALS generally include tripping, dropping objects, abnormal fatigue in the arms and / or legs, impaired language, muscle cramps, abnormal muscle movements, and / or uncontrollable bouts of laughing or crying. In an attempt to describe the symptoms included in the course of the disease from a general perspective, NY neurologist David Schick includes:

§ Muscle weakness in one or more of the following: hands, arms, legs, or the muscles of speech, swallowing, or breathing. Muscle weakness is the most relevant symptom of ALS. It is due to the progressive death of motor neurons and manifests itself when 50% of the population of these cells has been lost.

§ Abnormal muscle movements and muscle cramps (especially in the hands and feet) and loss of reflexes.

§ "Swallowing the words" and difficulty projecting the voice. One third of patients start with bulbar paralysis. This is a variant of amyotrophic lateral sclerosis in which the first symptoms are difficulties in speaking and swallowing.

§ In more advanced stages, shortness of breath and difficulty in breathing and swallowing.

Because ALS attacks only motor neurons, the sense of sight, touch, hearing, taste, and smell are not affected.

Cure and Treatment

ALS has been an invisible disease to society for years. It has gone so unnoticed that no significant progress has been made to halt its progression or cure the disease, due not only to the great complexity of the disease, but also to the lack of financial and institutional support. Hence, as of today, there is no effective curative treatment for ALS. Many substances have been tested without demonstrating effectiveness in all cases.

Although no cure has been found for ALS, there is treatment. There are drugs that help to help fight the set of symptoms that accompany the disease (cramps, spasticity, sleep disturbances or salivation problems), prevent unnecessary complications and make life easier for patients and their families. There are also treatments to help with pain, depression, and constipation. Respiratory and nutritional care are key to improving the quality and life expectancy of patients.

Research is focusing on different aspects such as: discovering the molecular, biochemical and cellular mechanisms of the disease; identify the genes and mutations involved in ALS; develop new effective drugs against the progression of ALS; study possible biomarkers that are applicable to diagnosis, drug development or disease monitoring; study clinical aspects of the disease, its appearance, risk factors and impact on public health, especially studies on the affectation, attention and respiratory evaluation, cognitive deterioration and behavioral alterations; the impact on health, quality and costs of health services; development of image and communication technologies; the social impact.