New urgency in battle against ‘bound legs’ disease

The harm done by konzo – a disease overshadowed by the war and drought it tends to accompany – goes beyond its devastating physical effects to impair children’s memory, problem solving and other cognitive functions.

Even children without physical symptoms of konzo appear to lose cognitive ability when exposed to the toxin that causes the disease, researchers report in the journal Pediatrics.

“That’s what’s especially alarming,” said lead author Michael Boivin, a Michigan State University associate professor of psychiatry and of neurology and ophthalmology. “We found subtle effects that haven’t been picked up before. These kids aren’t out of the woods, even if they don’t have the disease.”

Konzo means “bound legs” in the African Yaka language, a reference to how its victims walk with feet bent inward after the disease strips away motor control in their lower limbs. Its onset is rapid, and the damage is permanent.

People contract konzo by consuming poorly processed bitter cassava, a drought-resistant staple food in much of sub-Saharan Africa. Typically, the plant’s tuber is soaked for a few days, then dried in the sun and ground into flour – a process that degrades naturally occurring cyanide.

“As long as they do that, the food’s pretty safe,” said Boivin, who began studying konzo in 1990 as a Fulbright researcher in the Democratic Republic of Congo. “But in times of war, famine, displacement and hardship, people take shortcuts. If they’re subsisting on poorly processed cassava and they don’t have other sources of protein, it can cause permanent damage to the nervous system.

“Konzo doesn’t make many headlines because it usually follows other geopolitical aspects of human suffering,” he added. “Still, there are potentially tens of millions of kids at risk throughout central and western Africa. The public health scope is huge.”

To find out if the disease affects cognitive function, Boivin and colleagues from Oregon Health and Science University turned to the war-torn Congo. They randomly selected 123 children with konzo and 87 neighboring children who showed no signs of the disease but whose blood and urine samples indicated elevated levels of the toxin.

Using cognitive tests, the researchers found that children with konzo had a much harder time using working memory to solve problems and organize visual and spatial information.

They also found that konzo and non-konzo children from the outbreak area showed poor working memory and impaired fine-motor skills when compared to a reference group of children from a part of the region unaffected by the disease.

Konzo’s subtler impacts might seem minor compared to its striking physical symptoms, but Boivin noted that the cognitive damage is similar to that caused by chronic low-grade exposures to other toxic substances such as lead.

Scientists eventually may be able to prevent such damage by creating nontoxic cassava varieties and introducing other resilient crops to affected regions, Boivin said. Meanwhile, public health education programs are under way to help stop outbreaks.

“For now,” he said, “if we could just avoid the worst of it – the full-blown konzo disease that has such devastating effects for children and families – that’s a good start.”

'I don't want to pick!' Preschoolers know when they aren't sure

Children as young as 3 years old know when they are not sure about a decision, and can use that uncertainty to guide decision making, according to new research from the Center for Mind and Brain at the University of California, Davis.

"There is behavioral evidence that they can do this, but the literature has assumed that until late preschool, children cannot introspect and make a decision based on that introspection," said Simona Ghetti, professor of psychology at UC Davis and co-author of the study with graduate student Kristen Lyons, now an assistant professor at Metropolitan State University of Denver. [Preschoolers Use Introspection to Make Decisions]

The findings are published online by the journal Child Development and will appear in print in an upcoming issue.

Ghetti studies how reasoning, memory and cognition emerge during childhood. It is known that children get better at introspection through elementary school, she said. Lyons and Ghetti wanted to see whether this ability to ponder exists in younger children.

Previous studies have used open-ended questions to find out how children feel about a decision, but that approach is limited by younger children’s ability to report on the content of their mental activity. Instead, Lyons and Ghetti showed 3-, 4- and 5-year-olds ambiguous drawings of objects and asked them to point to a particular object, such as a cup, a car or the sun. Then they asked the children to point to one of two pictures of faces, one looking confident and one doubtful, to rate whether they were confident or not confident about a decision.

In one of the tests, children had to choose a drawing even if unsure. In a second set of tests they had a “don’t want to pick” option.

Across the age range, children were more likely to say they were not confident about their decision when they had in fact made a wrong choice. When they had a “don’t know” option, they were most likely to take it if they had been unsure of their choice in the “either/or” test.

By opting not to choose when uncertain, the children could improve their overall accuracy on the test.

"Children as young as 3 years of age are aware of when they are making a mistake, they experience uncertainty that they can introspect on, and then they can use that introspection to drive their decision making," Ghetti said.

The researchers hope to extend their studies to younger children to examine the emergence of introspection and reasoning.

(Image: Jupiter Images)

Bilingual children have a better “working memory” than monolingual children

A study conducted at the University of Granada and the University of York in Toronto, Canada, has revealed that bilingual children develop a better working memory –which holds, processes and updates information over short periods of time– than monolingual children. The working memory plays a major role in the execution of a wide range of activities, such as mental calculation (since we have to remember numbers and operate with them) or reading comprehension (given that it requires associating the successive concepts in a text).

The objective of this study –which was published in the last issue of the Journal of Experimental Child Psychology– was examining how multilingualism influences the development of the “working memory” and investigating the association between the working memory and the cognitive superiority of bilingual people found in previous studies.

Executive Functions

The working memory includes the structures and processes associated with the storage and processing of information over short periods of time. It is one of the components of the so-called “executive functions”: a set of mechanisms involved in the planning and self-regulation of human behavior. Although the working memory is developed in the first years of life, it can be trained and improved with experience.

According to the principal investigator of this study, Julia Morales Castillo, of the Department of Experimental Psychology of the University of Granada, this study contributes to better understand cognitive development in bilingual and monolingual children. “Other studies have demonstrated that bilingual children are better at planning and cognitive control (i.e. tasks involving ignoring irrelevant information or requiring a dominant response). But, to date, there was no evidence on the influence of bilingualism on the working memory.

The study sample included bilingual children between 5 and 7 years of age (a critical period in the development of the working memory). The researchers found that bilingual children performed better than monolingual children in working memory tasks. Indeed, the more complex the tasks the better their performance. “The results of this study suggest that bilingualism does not only improve the working memory in an isolated way, but they affect the global development of executive functions, especially when they have to interact with each other”, Morales Castillo states.

Music Education

According to the researcher, the results of this study “contribute to the growing number of studies on the role of experience in cognitive development”. Other studies have demonstrated that children performing activities such as music education have better cognitive capacities. “However, we cannot determine to what extent children perform these activities due to other factors such as talent or personal interest”.

“However, the children in our study were bilingual because of family reasons rather than because of an interest in languages.

Children With Brain Lesions Able To Use Gestures Important To Language Learning

Children with brain lesions suffered before or around the time of birth are able to use gestures – an important aspect of the language learning process– to convey simple sentences, a Georgia State University researcher has found.

Şeyda Özçalışkan, assistant professor of psychology, and fellow researchers at the University of Chicago, looked at children who suffered lesions to one side of the brain to see whether they used gestures similar to typically developing children. She examined gestures such as pointing to a cookie while saying “eat” to convey the meaning “eat cookie,” several months before expressing such sentences exclusively in speech.

“We do know that children with brain injuries show an amazing amount of plasticity (the ability to change) for language learning if they acquire lesions early in life,” Özçalışkan said. “However, we did not know whether this plasticity was characterized by the same developmental trajectory shown for typically developing children, with gesture leading the way into speech.  We looked at the onset of different sentence constructions in children with early brain injuries, and wanted to find out if we could see precursors of different sentence types in gesture.

“For children with brain injuries, we found that this pattern holds, similar to typically developing children,” she said. “Children with unilateral brain injuries produce different kinds of simple sentences several months later than typically developing children. More important, the delays we observe in producing different sentences in speech are preceded by a similar delay in producing the same sentences in gesture-speech combinations.”

Children with brain injuries also had a more difficult time in producing complex sentences across gesture and speech, such as conveying relationships between actions, for example saying “help me do it” while making a painting gesture.

“This in turn was later reflected in a much narrower range of complex sentence types expressed in their speech,” Özçalışkan said. “This suggested to us, in general, that producing sentences across gesture and speech may serve as an embodied sensorimotor experience, that might help children take the next developmental step in producing these sentences in speech.

“And if you bypass the gesture-speech combination stage, that might negatively affect developing a broader representation of complex sentence types in speech.”

The researchers also compared children with smaller brain lesions against children with large lesions, and found more of a delay in producing sentences, both in speech and in gesture-speech combinations, in children with large lesions.

The research has implications for developing interventions to help children with the language learning process, “as it shows that gestures are integral to the process of language learning even when that learning is taking place in an injured brain,” Özçalışkan said.

“When children do different kinds of sentence combinations across gesture and speech, that’s like a signal to the caregiver that ‘I’m ready for this,’” she said. “The caregiver can then provide relevant input to the child, and that could in turn help the child take the next developmental step in producing that sentence entirely in speech.”

Momentum builds in quest to find cure for childhood brain disease

Rasmussen Encephalitis strikes healthy kids; only known treatment removing half the brain.

How do you find a cure for a devastating pediatric brain disease so rare that it can take decades to build a meaningful research base?

In 2010, the parents of a patient created the Rasmussen Encephalitis (RE) Children’s Project to help solve this problem. In a short amount of time, the foundation has raised funds to establish a consortium of top researchers, build a collection of samples of the disease from around the world and support projects to study the disease tissue and search for genetic links. The goal is to find a cure.

Researchers at the David Geffen School of Medicine at UCLA have played a vital role in the ongoing research, and the foundation recently provided a second round of funding to continue their work. The gift of $125,000 builds on the organization’s donation of $111,000 made in 2011. 

"We are still in the early stages of research, but our momentum is building," said Seth H. Wohlberg, founder of the RE Children’s Project, and father of Grace, 15, who was stricken by the disease when she was 10 years old. "One of our key accomplishments has been to create an international system so that we can coordinate and transfer RE brain tissue and DNA material from the patients and parents. Collecting these samples is vital to advancing the research."

With the additional funding, UCLA researchers will apply cutting-edge DNA sequencing technology to determine whether a virus, or some other infectious agent, causes RE. They also plan to develop an animal model of the disease using cells obtained from the RE samples. 

The researchers include Dr. Gary Mathern, professor of pediatric neurosurgery and director of the UCLA Pediatric Epilepsy Program at Mattel Children’s Hospital; Carol Kruse, professor of neurosurgery; and Geoffrey Owens, visiting assistant researcher in neurosurgery.

"I am grateful to collaborate with a devoted father who has taken on the enormous task of advancing research for RE," said Mathern. "Thanks to his leadership, we now have the network to collect the tissue and DNA needed to study the brain, immunologic cells and genetics to unlock what causes this disease and develop new treatments or a cure. The RE Children’s Project has truly helped accelerate our research, bringing new information and resources that could have taken 10 more years to develop to the forefront today."

Rasmussen Encephalitis is a neurological disease that causes intractable seizures, cognitive deficits and paralysis of half of the body.  It is very rare and only a few hundred cases have been reported worldwide. RE typically affects previously normal children between the ages of two and ten years old. The disease process can run its course over a one to two year period during which time one half of the body is rendered useless and epileptic seizures continue unabated. 

An unusual feature of the disease is that it is usually confined to one hemisphere of the brain and is resistant to standard anti-seizure medicines. Currently the only known “cure” is radical- the surgical removal or disconnection of the affected side of the brain known as a hemispherectomy.

In the summer of 2008, the Wohlberg’s 10-year-old daughter Grace started to experience epileptic seizures. After months of testing, her parents learned that she had the extremely rare neurological disorder. Grace underwent an initial hemispherectomy surgery in February 2009. However, her seizures recurred so her parents then brought Grace to UCLA to complete the hemispherectomy which was performed by Mathern in March 2010. 

Today, Grace attends high school with the assistance of a full-time aide. While the surgery has stopped the seizures, Grace faces lifelong disabilities including partial blindness, cognitive issues and learning how to walk again. She is also active in helping her father promote the RE Children’s Project.

"It’s really supportive to let people know our story," said Grace. "Every year, my dad does a fundraiser and a lot of people come out to support it. It’s fun to be there and see all the people who care and want to help."

(Image: Wikimedia Commons)

First ever UK based language tool to decode baby talk

A tool which could radically improve the diagnosis of language delays in infants in the UK is being developed by psychologists.

A £358,000 grant to develop the first standardised UK speech and language development tool means that for the first time, researchers will be able to establish language development norms for UK children aged eight months to 18 months.

The tool will plug an important gap which has left UK researchers, education and health professionals at a disadvantage.

Until now, UK language experts have been forced to rely upon more complicated methods of testing child language development, or on methods designed for American English speakers which can lead to UK babies being misdiagnosed as being delayed in language development.

The two-and-a-half year project funded by the ESRC will also look into the impact of family income and education on UK children’s language development, as well as examining differences between children learning UK English, and other languages and English dialects.

The project is expected to make a major contribution to language development research as well as to the effectiveness of speech and language therapy and improved policy making.

Children’s complex thinking skills begin forming before they go to school

New research at the University of Chicago and the University of North Carolina at Chapel Hill shows that children begin to show signs of higher-level thinking skills as young as age 4 ½. Researchers have previously attributed higher-order thinking development to knowledge acquisition and better schooling, but the new longitudinal study shows that other skills, not always connected with knowledge, play a role in the ability of children to reason analytically. 

The findings, reported in January in the journal Psychological Science, show for the first time that children’s executive function has a role in the development of complicated analytical thinking. Executive function includes such complex skills as planning, monitoring, task switching, and controlling attention. High early executive function skills at school entry are related to higher than average reasoning skills in adolescence. 

Growing research suggests that executive function may be trainable through pathways, including preschool curriculum, exercise and impulse control training. Parents and teachers may be able to help encourage development of executive function by having youngsters help plan activities, learn to stop, think, and then take action, or engage in pretend play, said lead author of the study, Lindsey Richland, assistant professor of comparative human development at the University of Chicago.

Although important to a child’s education, “little is known about the cognitive mechanisms underlying children’s development of the capacity to engage in complex forms of reasoning,” Richland said.

The new research is reported in the paper “Early Executive Function Predicts Reasoning Development” and follows the development of complex reasoning in children from before the time they go to school until they are 15. Richland’s co-author is Margaret Burchinal, senior scientist at the Frank Porter Graham Child Development Institute at the University of North Carolina at Chapel Hill.

(Image: Shutterstock)

Device Helps Children with Disabilities Access Tablets

Imagine not being able to touch a touch-screen device. Tablets and smartphones—with all their educational, entertaining and social benefits—would be useless.

Researchers at Georgia Tech are trying to open the world of tablets to children whose limited mobility makes it difficult for them to perform the common pinch and swipe gestures required to control the devices.

Ayanna Howard, professor of electrical and computer engineering, and graduate student Hae Won Park have created Access4Kids, a wireless input device that uses a sensor system to translate physical movements into fine-motor gestures to control a tablet.

The device, coupled with supporting open-source apps and software developed at Georgia Tech, allows children with fine motor impairments to access off-the-shelf apps such as Facebook and YouTube, as well as custom-made apps for therapy and science education.

“Every child wants access to tablet technology. So to say, ‘No you can’t use it because you have a physical limitation’ is totally unfair,” Howard said. “We’re giving them the ability to use what’s in their mind so they have an outlet to impact the world.”

The current prototype of the Access4Kids device includes three force-sensitive resistors that measure pressure and convert it into a signal that instructs the tablet. A child can wear the device around the forearm or place it on the arm of a wheelchair and hit the sensors or swipe across the sensors with his or her fist. The combination of sensor hits or swipes gets converted to different “touch-based” commands on the tablet.

Children with neurological disorders such as cerebral palsy, traumatic brain injury, spina bifida and muscular dystrophy typically suffer from fine motor impairments, which is the difficulty of controlling small coordinated movements of the hands, wrists and fingers. They tend to lack the ability to touch a specific small region with appropriate intensity and timing needed for press and swipe gestures.

Can Going Hungry As a Child Slow Down Cognitive Decline in Later Years?

People who sometimes went hungry as children had slower cognitive decline once they were elderly than people who always had enough food to eat, according to a new study published in the December 11, 2012, print issue of Neurology®, the medical journal of the American Academy of Neurology.

“These results were unexpected because other studies have shown that people who experience adversity as children are more likely to have problems such as heart disease, mental illness and even lower cognitive functioning than people whose childhoods are free of adversity,” said study author Lisa L. Barnes, PhD, of Rush University Medical Center in Chicago.

For the African American participants, the 5.8 percent who reported that they went without enough food to eat sometimes, often or always were more likely to have a slower rate of cognitive decline, or decline that was reduced by about one-third, than those who rarely or never went without enough food to eat. The 8.4 percent of African American participants who reported that they were much thinner at age 12 than other kids their age also were more likely to have a slower rate of cognitive decline, also by one-third, than those who said they were about the same size or heavier than other kids their age. For Caucasians, there was no relationship between any of the childhood adversity factors and cognitive decline. Barnes said researchers aren’t sure why childhood hunger could have a possible protective effect on cognitive decline. One potential explanation for the finding could be found in research that has shown that calorie restriction can delay the onset of age-related changes in the body and increase the life span. Another explanation could be a selective survival effect. The older people in the study who experienced childhood adversity may be the hardiest and most resilient of their era; those with the most extreme adversity may have died before they reached old age.

Barnes noted that the results stayed the same after researchers adjusted for factors such as amount of education and health problems. The results also did not change after researchers repeated the analysis after excluding people with the lowest cognitive function at the beginning of the study to help rule out the possibility that people with mild, undiagnosed Alzheimer’s disease were included in the study.

Because relatively few Caucasians in the study reported childhood adversity, the study may not have been able to detect an effect of adversity on cognitive decline in Caucasians, Barnes said.