What is ALS and what are the causes?

Sandra Bullock’s longterm partner Bryan Randall has died at the age of 57 after a three-year battle with amyotrophic lateral sclerosis (ASL). His family shared a statement confirming that Randall “passed away peacefully” on Saturday (5 August), adding that he “chose early to keep his journey with ALS private and those of us who cared for him did our best to honour his request”. The statement, sent to People magzine, continued: “We are immensely grateful to the tireless doctors who navigated the landscape of this illness with us and to the astounding nurses who became our roommates, often sacrificing their own families to be with ours.” Bullock and Randall first met in 2015 and made their first public appearance together the following year. The model-turned-photographer’s family has asked for donations to be made to the ALS Association and the Massachusetts General Hospital. ALS affects nerve cells in the brain and spinal cord, its name coming from the Greek and referring to a wasting away of the muscles responsible for controlling voluntary movement caused by a lack of nourishment, the fault of a genetic mutation. As the ALS Association explains: “Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their demise. “When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. When voluntary muscle action is progressively affected, people may lose the ability to speak, eat, move and breathe. “The motor nerves affected when you have ALS are the motor neurons that provide voluntary movements and muscle control. Examples of voluntary movements are making the effort to reach for a smartphone or step off a curb. These actions are controlled by the muscles in the arms and legs.” Early symptoms of the disease, according to the US National Institute of Neurological Disorders and Strokes (NINDS), include: muscle twitches in the arm, leg, shoulder or tongue; cramps; tight or stiff muscles; muscle weakness; slurred or nasal speech; and difficulty chewing and swallowing. “The first sign of ALS usually appears in the hand or arm and can show as difficulty with simple tasks such as buttoning a shirt, writing, or turning a key in a lock,” the NINDS explains. “In other cases, symptoms initially affect one leg. People experience awkwardness when walking or running, or they may trip or stumble more often.” There is currently no cure for ALS but the US Food and Drug Administration has approved four drugs to treat the condition and ease the discomfort of sufferers: Riluzole, Nuedexta, Radicava and Tiglutik. It was first discovered by French neurologist Jean-Martin Charcot in 1869 and is also commonly known as Lou Gehrig’s disease in memory of the celebrated New York Yankees baseman (1903-41) who also suffered from it. While ALS affects all demographics, it is most common among people aged between 55 and 75 and, according to some studies, disproportionately affects former members of the armed forces, perhaps because of exposure to harmful toxins during their service careers although this has not been definitively substantiated. The ALS Association reports that, in 90 per cent of cases of ALS, there is no family history of the genetic mutation that causes the disease and, in the 5-10 per cent of cases in which there is, only a 50 per cent possibility exists of its being passed on. For more information, please visit the websites of the ALS Association or the National Institute of Neurological Disorders and Strokes. Read More Roberta Flack announces she has ALS and finds it ‘impossible to sing’ NIH to fund unproven ALS drugs under patient-backed law ALS drug wins FDA approval despite questionable data Brain’s appetite control centre different in overweight or obese people – study Areas with lower bird diversity ‘have more mental health hospital admissions’ Greg Rutherford rushed to hospital ‘screaming and clawing at his skin’