Gilbert Gottfried, the beloved brash comic, died Tuesday after having battled a illness that his publicist recognized as a uncommon genetic muscle dysfunction.
Gottfried, 67, had kind II myotonic dystrophy, a form of muscular dystrophy, stated his longtime buddy and publicist, Glenn Schwartz.
It is not clear when Gottfried was identified with the illness, which has no remedy or therapy and usually seems when individuals are of their 20s and 30s.
His household stated in an announcement that he died after a “long illness.”
Elizabeth McNally, the director of the Northwestern University Center for Genetic Medicine, stated the “slowly progressive” situation is usually neglected and undiagnosed.
“People can have symptoms for quite a while even before they notice it,” she stated.
Symptoms of the illness, resembling respiratory or coronary heart muscle weak spot, are inclined to develop extra as folks age, she stated, so some sufferers could not get identified till no less than their 40s, 50s or 60s, or they could confuse their signs with different age-related well being issues.
“The things I always notice first in patients is that they have trouble getting up out of chairs and difficulty going upstairs,” McNally stated.
Other signs embody slurred speech, jaw locking and extended muscle contractions that make it troublesome to launch a doorknob, as an illustration.
In time, McNally stated, some sufferers could have issue taking good care of themselves, together with brushing their tooth or taking showers.
McNally stated coronary heart problems are widespread for the 2 sorts of myotonic dystrophy.
“The increased risk for irregular heart rhythms can really be quite significant,” she stated, including: “Sometimes people underestimate how much that can happen. There’s some stuff in textbooks that suggests it doesn’t happen very much in type II, but in my experience, I see it a lot in my type II patients.”
Gottfried died of recurrent ventricular tachycardia, an irregular rhythm within the decrease chambers of the center.
“Instead of beating normally and consistently, the heart just sort of starts going electrically haywire and goes very fast, and it can’t beat effectively,” McNally stated of the situation. “If that doesn’t correct itself quickly, within a matter of seconds, what can happen is the person can die.”
In some situations, she stated, medical doctors can deal with sufferers with pacemakers or defibrillators.
“Typically a good cardiologist should anticipate” ventricular tachycardia, she stated. “The patient should have a defibrillator to treat that, should it happen, so the person shouldn’t die.”
About 1 in 8,000 individuals are identified with both kind of the illness, though kind II is believed to be rarer.
Reynolds Lewis contributed.