Amyotrophic Lateral Sclerosis (근위축성 측색 경화증 -- 루게릭병)

New York Yankee Lou Gehrig (1903 - 1941 ) is remembered for his major league baseball records and his consecutive game streak in the 1920s and '30s ― and for his tearful retirement at age 36, when he was diagnosed with the fatal disease that now bears his name. Amyotrophic lateral sclerosis (ALS), often referred to as Lou Gehrig's disease, causes motor neurons in the brain and spinal cord to degenerate, leading to muscle weakness, coordination problems, and, eventually, total paralysis and death. Throughout the progress of the disease, mental function is not affected.

 

ALS 는 자율 운동 신경을 공격하는데, 걷기, 말하기, 몸짓과 호흡 등에 사용되는 것들이 여기에 포함된다. (호흡은 불수의근과 자율신경 모두 관여하는데, 왜냐하면 너는 숨을 멈출 수 있다 ― 적어도 일시적으로 ― 때에 맞추어.) 신경세포가 사멸함에 따라 이 근육들은 없어진다: 사지가 얇게 보여지기 시작하며, ALS 를 가진 사람들은 허약함, 연축, 경련 및 쥐남, 불분명한 발음과 그들의 팔과 다리를 사용함에 있어서의 어려움 등을 경험할 수 있다. 그들은 발을 헛디디거나, 물건을 떨어뜨리거나 혹은 통제되지 않은 웃음이나 울음을 터트리게 되는 수도 있다. 좀더 증상이 심화된 단계에서는 그들은 호흡을 하기 위해서 영구적인 산소호흡기가 필요할 수도 있다.

 

About 15 new cases of ALS are diagnosed in the United States every day; 60 percent of these individuals are male, and 93 percent are Caucasian. Most people develop symptoms between the ages of 40 and 70 and live for 3 to 5 years after diagnosis ― although for some, the disease advances less quickly or, in rare cases, may halt completely. The medication riluzole (Rilutek) can minimize the release of the chemical glutamate, an effect that appears to help slow the illness's progression in some cases. Scientists aren't sure why, but people with ALS have more glutamate in their spinal fluid than healthy people do.

 

대부분의 ALS 의 경우는 발현이 산발적이었는데, 이는 가족력으로 인해 사람들에게 영향을 끼치지 않는다는 뜻이다. 이 질환은 진단하기 어려운데; 의사들은 혈액검사, X-ray, 전기신경자극검사를 수행하여야 하며 모든 다른 질환들을 배제시켜야 한다. 약 5에서 10 퍼센트의 경우에서 사람의 가족력이 있다는게 나타나서 1991년에 과학자들은 이러한 타입의 ALS 를 21번 염색체의 유전자로 연결지었다. 오늘날 유전자검사는 가족력의 ALS 를 결정지을 수 있다. 비록 여전히 증상에 대해 많이 의존하고 있지만 말이다.

 

 

An extremely high incidence of ALS was observed in Guam and the Trust Territories of the Pacific in the 1950s. This subset of the disease bears the name Guamanian ALS.

 

Gehrig played 2,130 consecutive games, earning him the nickname the Iron Horse, before his illness forced him into retirement. His farewell speech at Yankee Stadium is among the most famous moments in the history of baseball: before a packed crowd, speaking haltingly into a microphone, the first baseman declared that despite his "bad break," he considered himself " the luckiest man on the face of the earth" because of the support of his fans and the opportunity to play baseball.

 

The British theoretical physicist Stephen Hawking (1942- 2018 ) has remarkably survived several decades with ALS. He was diagnosed at age 21, married and fathered three children after his diagnoses, and has used a computerized voice synthesizer to "speak" ever since he had a breathing device implanted in his throat.