ALS (Amyotrophic lateral sclerosis / Lou Gehrig’s Disease)

Often referred to by the name of a famous baseball player, Lou Gehrig, who died from the disease, ALS often develops in people between the ages of 40 and 70 but can develop at an earlier age. According to the ALS Association, the disease effects as many as 30,000 American with an average of 5,000 new cases diagnosed each year.

There are three types of ALS:

  • Sporadic ALS – The most common type of the disease, accounting for roughly 90 to 95 percent of all cases in the U.S., it occurs randomly without cause to people who have no family history of the disease.
  • Familial ALS – Only five to 10 percent of cases in the U.S. are this type which suggests that the disease may be inherited.
  • Guamanian – Throughout the 1950s there was a high incidence of ALS observed in Guam and the Trust Territories of the Pacific Islands

At Brigham and Women’s Hospital, the Division of Neuromuscular Disease and Electromyography (EMG) within the Department of Neurology, provides comprehensive diagnostic evaluation, consultation and management of all neuromuscular disorders, including ALS, peripheral neuropathies, myopathies, muscular dystrophy, and myasthenia gravis. Our team of physicians, nurses, and researchers is dedicated to advancing the understanding and treatment of ALS and related disorders.

ALS is one of the many diseases and conditions that form the focus of our Neurosciences Center. Our multidisciplinary team of neurologists, neurosurgeons, psychiatrists, and radiologists offers the most innovative and advanced treatments and therapies for Parkinson’s and all diseases of the nervous system. Our dedication to discoveries and research in these areas has made us the choice for patients from the region and around the world.

Amyotrophic Lateral Sclerosis Topics

Symptoms of ALS

At the onset of ALS the symptoms may be so slight that they are frequently overlooked. Often, the first symptom is weakness in a limb that develops over a few weeks. As the disease progresses, symptoms include:

  • muscle weakness in the hands, arms, legs or the muscles of speech, swallowing or breathing
  • twitching and cramping of muscles, especially those in the hands and feet
  • impairment of the use of the arms and legs
  • “thick speech” and difficulty in projecting the voice
  • in more advanced stages, shortness of breath, difficulty in breathing and swallowing

As motor neurons get sick and die, a person progressively loses the ability to move, walk, speak, swallow, and breathe. Additionally, as with other serious illnesses, depression becomes a common symptomatic condition. ALS is usually fatal within two to five years of diagnosis.

The symptoms of ALS can resemble symptoms for other conditions or medical issues. It is important for symptoms, such as these, to be fully evaluated by a specialist who can provide a proper diagnosis.

Diagnosis of ALS

Making an accurate diagnosis in the early stages of ALS can be more difficult, as the beginning signs and symptoms can be considered indications for other medical conditions. There is no one test or procedure to ultimately establish the diagnosis of ALS. It is through a clinical examination and series of diagnostic tests, often ruling out other diseases that mimic ALS, that a diagnosis can be established. At Brigham and Women’s Hospital, our ALS specialists – neurologists and neuro-radiologists – provide expert evaluation and diagnosis utilizing the latest in advanced imaging technologies.

In addition to a thorough medical history and detailed neurological examination, diagnostic testing for ALS can include:

  • Electromyography (EMG test)
  • Nerve Conduction Velocity (NCV)
  • Spinal tap - to measure pressure in the spinal canal and brain
  • Magnetic resonance imaging (MRI) – a diagnostic imaging procedure that uses a combination of large magnets, radiofrequencies, and computer technology to produce detailed images of organs – such as the brain – and structures within the body.
  • X-ray
  • Myelogram
  • Biopsy
Treatment of ALS

The Department of Neurology at Brigham and Women's Hospital offers the most innovative and advanced treatments and therapies for all diseases of the nervous system. Our clinical team - one of the largest and most specialized teams available anywhere - includes neurologists, neurosurgeons, psychiatrists, and radiologists.

Although no current treatment slows or halts the progression of ALS, researchers at Brigham and Women’s Hospital made an important discovery in August of 2012 that could lead to the development of a treatment for the disease. The BWH researchers were the first to discover that changes in monocytes (a type of white blood cell) are a biomarker for ALS. This new discovery could help to determine the path leading to the development of specific medications that control the increase of monocytes, which have now been shown to be a factor in the development of ALS.

Because there currently is no cure for ALS, current treatments focus on easing symptoms and making day-to-day life more manageable for patients. Riluzole is an FDA-approved medication for the treatment of ALS. How it works is not exactly known, but it seems to prevent some damaging effects of the disease. Studies have shown it also may improve functioning and survival, but because of its potential side effects on the liver, close monitoring is required. In addition, various medications and supplements may be prescribed as the disease progresses to help ease symptoms. These include:

  • Medications that relieve stiffness in the limbs and throat
  • Medications that ease cramps
  • Medications that control excess saliva that is common with the disease
  • Nutritional supplements to slow muscle decline and weight loss

Psychiatry Services

Living with ALS does have an impact on mental health and often can lead to depression. At Brigham and Women’s Hospital, ALS patients have access to the services they need to cope with the challenges they face on a day-to-day basis.

Specialists within the Department of Psychiatry at Brigham and Women’s Hospital provide a broad range of programs, including specialized services that address the unique needs of patients living with chronic conditions, such as ALS.

Therapy Services and Nutrition Plans

An integral part of ALS treatment is ensuring that each patient receives all the support services they need to help manage the impact the disease has on their overall health and well-being.

Part of the multidisciplinary care provided at Brigham and Women’s Hospital, many patients with Parkinson’s disease also benefit from:

  • Speech therapy
  • Physical therapy
  • Occupational therapy
  • Nutrition counseling

Managing the symptoms of ALS is a process that may be challenging for people with the condition, their caregivers, and the medical team. However, it is important to know that there are many resources available at Brigham and Women’s Hospital for support and assistance.

Multidisciplinary Care

Brigham and Women’s Hospital provides a multidisciplinary approach to patient care, collaborating with colleagues in other medical specialties. If your neurologist or neurosurgeon discovers an underlying illness or concern, you will be referred to an appropriate Brigham and Women’s Hospital physician or allied health professional for an expert evaluation.

Additional Resources

Go to our online health library to learn more about ALS.

Visit the Kessler Health Education Library in the Bretholtz Center on the main Brigham and Women’s Hospital campus, where patients and families can access computers, printed information and guides, and a knowledgeable staff.

Access a complete directory of patient and family services at Brigham and Women’s Hospital.

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