An experimental gene therapy treatment appears to have helped eight children with a rare and incurable neurological disorder, although it may have been responsible for the death of one, researchers reported on Tuesday.
They said the treatment appeared safe and effective enough to try in more children with late infantile neuronal ceroidlipofuscinosis, or LINCL, a form of deadly Batten disease.
The treatment, in which a virus carrying the corrective gene was infused directly into the brain, appeared to slow the decline of eight out of 10 children treated, Dr. Ron Crystal of New York-Presbyterian Hospital/Weill Cornell Medical Center and colleagues reported.
“We are encouraged by this. It’s not a cure,” Crystal said in a telephone interview.
Like all forms of gene therapy, the hope is that the mutant cells will take up the new gene and start working normally.
Children with LINCL start showing symptoms at about age 4. They lose coordination, vision and speech and usually die unable to breathe on their own, between 10 and 12.
One child suffered an epileptic seizure weeks after treatment and died and another child died of unknown causes two years after treatment.
Eight of the children showed a measurable slowing of the inevitable decline usually seen in the condition.
Only about 200 children are alive with the disease globally at a given time.
“The disease is caused by mutations in the CLN2 (ceroid lipofuscinosis, neuronal 2) gene,” Crystal and colleagues wrote in their report, which was published in the journal Human Gene Therapy.
Why a grizzly gets you shivering—but not global warming
In my Science Confirms the Obvious post today, I discussed the first psychological proof (so say the authors) that humans can indeed experience emotions without immediately knowing why. We do this, they say, because we evolved that way. True, scientists love that explanation, but here it’s quite intriguing.
Say you’re walking through the woods and encounter a grizzly bear. You see it and freeze that instant—even before your stomach drops with fear. “After all, you are likely to live longer if you immediately stop moving at the sight of a growling grizzly bear,” write researchers Kirsten Ruys and Diederik Stapel of The Netherlands’ Tilburg University, “and do not need full awareness for such a response to be instigated.” Given the flood of unexpected stimuli we face moment to moment, quick reactions make sense for survival.
You, that bear, and other animals experience emotions such as fear, anger, or disgust. But only a few species are aware of their emotions. This ability helps humans judge and respond to the behavior of others in order to navigate social situations and, ultimately, grease the wheels of complex society.
The study got me thinking about a talk given by Harvard psychologist Dan Gilbert at last October’s Pop!Tech conference. He expounded on why humans are so savvy at grasping immediate threats like grizzly bears or baseballs hurtling at our heads but suck at grasping abstract, slowly approaching ones like global warming. According to Gilbert, a “very large part” of our brains is devoted to dealing with immediate threats, but a “very small part” is concerned about planning for the future.
Humans, apparently, are still in the early stages of evolving extended response mechanisms. But it seems likely that by the time we portion more of our brain to long-term dangers, there will be few grizzly bears around to worry about, and a whole lotta global warming.
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- Tags: Brain, EVOLUTION, Future Human, Psychology, Thought
Scientists find that our brain makes decisions in surprisingly predictable ways
Yet more news today about how our brains work and how they give off signals in advance of certain actions. We just told you about a study which detected brain activity slowdown in response to repetitive tasks and the way in which it can be an indicator for increased mistakes. We’re seeing another study today about a different brain activity that may reveal a decision before that decision is actually made.
The German researchers responsible for the study wanted to elucidate what happens in the brain’s planning centers just before someone comes to a firm decision. In order to keep ourselves from being overloaded with having to keep track of routine tasks, our brains put a lot of decisions into the background and process them in our subconscious. The tasks of which we’re aware should be totally controlled by our conscious mind. So the scientists were surprised to discover that they could anticipate which one of two decisions were going to be made by a subject up to seven seconds before that person noted making the decision.
The computer software they developed was able to detect particular activity patterns and determine whether one or the other choice was on its way. It isn’t a perfect predictor, however, which suggests that our conscious minds ultimately have say over our final answers.
Via MedGadget
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- Tags: Brain, Human Decision
