Amyotrophic Lateral Sclerosis
(ALS or Lou Gehrig's Disease)

Key Glossary Terms

Neurological Voice Disorders
Voice problems caused by abnormal control, coordination, or strength of voice box muscles due to an underlying neurological disease such as: stroke, Parkinson's disease, multiple sclerosis, myasthenia gravis, or ALS

Dysarthria
Difficulty forming words – presenting with imprecise consonants and hard-to-understand speech as seen with stroke patients

Speech Disorder
Malfunction of the tongue and/or lip muscles resulting in garbled words or parts or words.

Glossary

ALS is a progressive neurological disease that may present initially with speech/voice difficulties as the primary symptoms in up to 10 to 15 percent of patients, including:

• Spastic/strained voice
• Slurred speech
• Hypernasal voice

Eventually, other symptoms, such as weakness and spasms of the limbs, will appear and predominate. Most patients, however, notice weakness or non-coordination in their arms or legs before they begin to have vocal changes.

• The cause of ALS is unknown and no risk factors have been identified in most patients.
• A small percentage (5 to 10 percent) of cases run in families.
• The average age of onset is between 50 to 60 years of age.

A careful history and physical examination by a neurologist, along with blood tests and X-rays to rule out other (rare) causes, will suffice in making the diagnosis.

• Early stages of ALS can easily be missed. Vocal difficulties associated with ALS can mimic spasmodic dysphonia (if vocal spasms predominate) or vocal fold paresis (if vocal fold weakness and flaccid symptoms predominate). Patients who have only voice/speech problems may be sent to an otolaryngologist for care.
• Typically, within weeks to months ALS progresses to involve the arms/legs, at which point the diagnosis becomes more obvious. A consultation with a neurologist should result in a correct diagnosis.

• There is no cure for ALS – only supportive care.
• The disease is variable, but half of ALS patients die from respiratory failure within three years of onset of symptoms.
• With some slowly progressive forms of the disease, patients may live for 10 years. In these select cases, an otolaryngologist may perform the following:
  • Injections of Botulinum toxin, type A into the vocal folds, to relieve vocal spasms
  • Framework surgery that brings the vocal folds together to improve vocal fold vibration during sound production (medialization laryngoplasty), to relieve a breathy voice