The Extraocular Muscles in ALS: A Research Riddle
Could the preservation of the eye muscles in ALS provide a new outlook on a deadly disease?
Anton Tjust |
Amyotrophic lateral sclerosis (ALS) is a devastating condition. An incurable neurodegenerative disease, it is characterized by a progressive loss of upper and lower motor neurons, leading to complete paralysis and eventually death through respiratory failure, usually within three to five years of symptom onset. Currently, the only treatment available is riluzole, but this drug only extends the lifespan of ALS patients by an average of two to three months.
Clinically, the disease can present itself with intriguing variability. It may manifest at any age, but often occurs in people who are middle-aged or older. Subsequent survival is also variable, with some patients surviving under a year, and a small proportion living for decades. When the patient experiences the first symptoms, they are mostly restricted to a single extremity – for example, the right hand. The disease most commonly presents itself distally with a mixture of upper and lower motor symptoms, such as weakness and loss of dexterity. This is later followed by a progression into more proximal muscles, appearance of the same symptoms in the ipsilateral leg, and eventually, involvement of all voluntary muscles in the body. In about 25 percent of the cases, the disease manifests in the bulbar region, first affecting muscles of articulation and mastication. Another variable trait in ALS is the degree of cognitive involvement. Some patients retain all of their premorbid personality and functions, and some patients develop an outright frontotemporal lobe dementia.
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