Rare Disease Day

Today, February 28, 2014, is Rare Disease Day. What is considered a rare disease? Well, by comparison, for drugs, a disease or condition is considered rare if it affects less than 200,000 persons in the United States. For medical devices, a disease or condition is considered rare when it occurs so infrequently in the US that there is no reasonable expectation that a medical device for such disease or condition will be developed without assistance. For rare diseases, according to the National Institutes of Health there are about 6,800 rare diseases and conditions. In total, nearly 30 million Americans (maybe one in ten) suffer from at least one rare disease. Stiff-Person Syndrome or SPS is one of those rare diseases. Last year, after meeting Shane James who was diagnosed with SPS in 2007,  I devoted several blogs to increasing awareness of this syndrome.  You can find out additional information through  NORD, the National Organization for Rare Disorders (www.rarediseases.org) in the USA, and the Steve Waugh Foundation (www.stevewaughfoundation.com.au) in Australia. 

Anosognosia and the Brain, 2

Although no one is exactly certain about what causes anosognosia, brain studies suggest that the lack of awareness may be linked to deterioration of the frontal lobes, especially the right frontal lobe (frontal lobes have an important role in planning, problem-solving, social interactions, and putting together the context and implications of experiences); and perhaps some atrophy in the temporal lobes as well. It can be extremely frustrating for friends and family members. If and when they bring up concerns, the involved family member may insist they are being unfairly criticized because there is nothing wrong. Reportedly, sometimes anosognosia is selective. An individual may realize he or she has a problem with one activity but lack awareness about other issues. This just reminds me how important it is to do everything within my power to maintain optimum brain function!

Watch for more next week (anosognosia and mental illness).

Anosognosia and the Brain

Anosognosia. Is that a new word for you? It’s sort of the opposite of hypochondria, when individuals think they have every illness they hear about. With anosognosia, individuals seem completely unaware that they have an illness, even after being told about their diagnosis. Although not well know, it is a common consequence of fronto-temporal dementia. (Estimates are that 42% of patients with early Alzheimer’s exhibit symptoms of anosognosia.) Sandra Weintraub, professor of psychiatry and neurology at Northwestern University, explained it this way: “This is not denial. It’s a lack of insight and awareness. Everyone else around them is aware they’re not the same, and they are not.”

More tomorrow.

Gender and the Stressed Brain, Cont'd

Males need to develop effective stress management strategies. In addition to using those to help them handle stress, they can help female brains in their circle of life to brainstorm ways of dealing with stressors that seem to impact their brains differently.  When under pressure or stress, the male brain’s spatial functions are activated. As a result, he may stop talking altogether. Three major symptoms of stress for the male brain: withdrawing, grumbling, and shutting down. Under stress, males tend. to activate the Fight-Flight stress response and may wonder the reason female brains (if they do activate-Flight-Fight) quickly move into Tend-Befriend. Though socialization likely plays a part, in the big picture it’s all about differences in brain structure and function . . .

Gender and a Stressed Brain

It’s fairly common knowledge now that male and female brains respond to stressors differently. Because of physiological factors, the female brain seems innately more reactive to stressors. Effective stress management strategies are critical for everyone but especially for the female brain. Studies have shown that females are at twice the risk for stress-related problems including depression and PTSD (Posttraumatic Stress Disorder). When under stress or pressure the female brain’s function of speech is activated and she may talk nonstop. Three major symptoms of stress for the female brain are: overwhelm, overreaction, and exhaustion. Under stress, females may go into Fight-Flight initially, but quickly fall back into using the Tend-Befriend stress response. Males respond differently and exhibit different symptoms (more on that tomorrow). 

To Meditate or Not to Meditate

Some people are frightened of “meditation,” others are too busy to investigate, and still others tried something that did not work for their brains and "threw the baby out with the bath water,” as the old metaphor puts it. Studies have shown any number of benefits that can accrue to your brain, heart, and body from meditating: improved memory, stress relief, slower heartbeat, and so on. In fact, almost any activity can lead to a meditative experience if you are aware in the moment and engage your senses. The good news? You can choose to meditate by developing a style that works for you. The key is to slow down and become aware of what is happening in the present moment. It’s so easy to start thinking about all the things you need to do in the future, or all the things you crammed into yesterday, or whether you turned off the iron or the dryer or turned on the dishwasher or the coffee maker. Your mind will want to do that (out of habit) and will start to drift. Bring your thoughts back to the present moment and calm your nervous system. If it’s raining, you might close your eyes and concentrate on the sound of rain against the window pane or watch them strike the glass if you're visual. If you like specific odors, you might light a fragrant candle and concentrate on the scent. If you are tuned into sound, you might listen to yourself breathing in and out and in and out. You might prefer creating a calming picture or scene in your mind's eye and focus on that. Start with just five calming minutes. Practice again tomorrow, and so on. Each brain is different and each brain can meditate.

Brain, Heart, and Appreciation

Do you begin the day by choosing to experience a sense of genuine appreciation? There will always be a place for proper eating and appropriate physical exercise. However, the old proverb says a merry heart is good medicine. Studies at the Institute of HeartMath have shown that an appreciative heart can help improve your health. If you have difficulty thinking of something to genuinely appreciate in the present moment, recall a time when you felt sincere appreciation and then recreate and experience that same feeling. By doing this you can increase the smoothness in your heart rhythms (something HeartMath refers to as coherence), reduce your levels of emotional stress, and positively impact your health. In today’s world I can always find something to appreciate and feel good about doing so, knowing that I am also benefiting my heart and its close connection with my brain.

Gray Matter - White Matter

Back to Connectomes. Researchers at USC have created the first map of white-matter (think of it as vital scaffolding) connections in the human brain. Their work just published in the open-access journal Frontiers in Human Neuroscience, has major implications for understanding brain injury and disease. Van Horn, researcher at the USC Institute for Neuroimaging and Informatics and the Laboratory of Neuro Imaging, put it: “Just as when you remove the internet connection to your computer you won’t get your email anymore, there are white matter pathways which result in large scale communication failures in the brain when damaged.” When those white matter pathways are damaged, brain areas served by those connections may wither …  Protect your Brain!

Andrei Irimia, John D. Van Horn, Systematic network lesioning reveals the core white matter scaffold of the human brain, Frontiers in Human Neuroscience, 2014, DOI: 10.3389/fnhum.2014.00051 (open access)

Gray Matter - White Matter

I’ve been following the emerging research about Connectomes, the mapping of connections within the brain. I find it fascinating, especially in relation to differences between male and female brains. My coauthor and I included some of that information in the recently released third edition of our book Your Brain Has a Bent (not a Dent). You no doubt are already familiar with the concept that your brain has both gray matter and white matter. Think of gray matter as the outermost portion of your brain It is filled with neurons where information is processed and stored. Yes, injury to gray matter can create any number of different types of injury. Think of white matter as the pathways that connect everything in the brain. When white matter is damaged, small brain injuries may have devastating effects. More tomorrow on new connectome research that is part of a worldwide scientific effort to map the 100 billion neurons and 1,000 trillion connections in the living human brain: led by the Human Connectome Project and the Laboratory of Neuro Imaging at USC.

Ear-plug Protection

As an auditory, I am very sensitive to sounds, especially loud sounds. I also love musical concerts, but often the decibel level is more than my ears want to hear. I carry earplugs with me everywhere and pop them in any time I need to take the decibel level down a bit but still want to be able to hear conversation and so on. I also find them very helpful on airline flights. Several people have asked me what type of earplug protection I use. At the risk of sounding like I'm making a commercial "plug," I will include the information. Actually, I'm indebted to my friend David Hegarty for this, as he gave me my first pair of earplugs one year for my birthday and I've not been without them since. The company is Etymotic Research, and I prefer the High-Fidelity Earplugs. My carrying case has a phone number on it, as well:  847-228-0006. Studies have shown that loud sounds can damage one's inner ear and contributed to decreased levels of hearing over one's lifetime--nothing I want to contribute to. Smile.

http://www.etymotic.com/hp/er20.html

Happy Valentine's Day

Do you remember receiving your first valentine? I do, although the person who sent it never signed a name ... so I don't have a clue about its origin. My mother typically purchased a sheet or two of valentine cards for my brother and me to give to our friends. We'd carefully cut them out and decide who each would go to, but I don't think we signed many of them, either. I also recall that quite a few of those little cards had printed verses that began with "Roses are red, violets are blue..."  Recently, while looking for something else, I ran across the supposed original of that verse. It was in a collection of English nursery rhymes: Gammer Gurton's Garland (1784). That version went like this:

The rose is red, the violet's blue, the honey's sweet, and so are you.
Thou art my love and I am thine; I drew thee to my Valentine:
The lot was cast and then I drew, and Fortune said it should be you.

That's not the version I learned. It was only the first line and read:  "Roses are red, violets are blue, honey is sweet, and so are you." I guess things change over time . . .  Anyway, have a valentine day!

Auditory Sensory System

The first embryonic cells are sound sensitive. By about one and a half months in the womb, a baby’s auditory system is virtually complete. Every cell in the developing fetal body is influenced by the energy reflected in sound waves.  Once the baby is born, the melodies of speech are also vital to communication between mother and infant. Diane Deutsch, Professor of Psychology at the University of California, says that parents often use exaggerated speech patterns when communicating with their babies. Sometimes known as "motherese," these exaggerated speech patterns differ considerably between languages. The auditory pathways continue to develop until age 7-10 years. According to Jane M. Healy, PhD, author of Your Child’s Growing Mind, Music, human speech, and environmental sounds are all important in a child's balanced auditory diet.

Learning Before Birth

When babies are born, they are already familiar with the "melody" of their mother's speech. Researchers have recorded (from inside the womb at the beginning of labor) the sounds produced by the mother that the fetus is able to hear. Ken Robinson in "Out of Our Minds" puts it this way: "The phrases reaching the baby have been filtered through the mother's tissues, however, so that the crisp, high frequencies, which carry much of the information important for identifying the meanings of words, are muted, whereas the musical characteristics of speech--its pitch contours, loudness variations, tempo and rhythmic patterning--are well preserved." It is thought that this early exposure to musical speech sounds may begin the process of learning to speak. And this in-utero exposure begins quite early in the gestation process.

Perfect Pitch and Languages

According to Diana Deutsch, Professor of Psychology at the University of California, not only do brain areas related to language and music overlap considerably, a person's native tongue influences the way he or she perceives music. Depending on the language the listener learned growing up, the same succession of musical notes may sound quite different. For example, speakers of tonal languages (including Mandarin) are more likely than Westerners to have perfect pith. In one study quoted by Ken Robinson in his book "Out of Our Minds," 92% of Mandarin speakers who began music lessons at or before the age of five had perfect pitch. Only 8% of English speakers had perfect pitch with comparable musical training. And "listening" begins in utero.

Speech-Music Link

In his book "Out of Our Minds," Ken Robinson reported some fascinating information about learning languages and a connection with music. The brain is very plastic during childhood so that children who are born into multilingual households learn all the languages to which they are regularly exposed. They can learn three or four different languages, or more, quite easily. Apparently,  there is a strong relationship between speech and song and music--in the process of learning to speak. The areas of the brain that are primarily concerned with music and language overlap considerably. More tomorrow.

Do You Know What Broca's Area Does? #2

UCSD News reported on the first experiment to use Intra-Cranial Electrophysiology or ICE to document how the human brain computes grammar and produces words. Scientists evaluated both speaking and reading. Recordings when patients spoke words revealed that one small part of the brain, Broca’s Area, engages in processing the identity, structure, and sounds of words, separately and in a quick sequence. It can do three different functions at three different times (within a quarter of a second). Patterns of neuronal activity showed lexical, grammatical, and articulatory computations at roughly 200, 320 and 450 milliseconds after the target word was presented. These patterns were identical across nouns and verbs and consistent across patients. Ned T. Sahin, PhD, stated: “It has been clear for some time that the expressive/receptive model is out of date, and now it is clearer that Broca’s area has several roles, in both expressive and receptive language.”

Do You Know What Broca's Area Does? #1

Were you taught that Broca’s area in the  frontal cortex of the left hemisphere handles expressive language (speaking) while Wernicke’s area in the posterior area of the left hemisphere handles receptive language (reading and hearing)? That information appears to be out of date, even though some text books reportedly still include it. Using a procedure called Intra-Cranial Electrophysiology (ICE), researchers discovered that it’s neither that simple nor that clear cut. Principal investigator Eric Halgren, PhD, professor in the UCSD Department of Radiology, found that aspects of word identity, grammar, and pronunciation are all computed within Broca’s area. “Crucially, information about the identity of a printed word arrives in Broca’s area very quickly after it is seen, in parallel with its arrival in Wernicke’s. These results suggest that Broca’s area actually consists of several overlapping parts, performing distinct computational steps in a tightly timed choreography, a dance that may simply have been undetectable due to the level of resolution of previous methods.”

Want to Learn and Remember Better?

Research at Montreal Neurological Institute and Hospital (The Neuro) of McGill University has shown that different patterns of training and learning lead to different types of memory formation. In every organism they studied, researchers reported that formation of memories is highly sensitive not only to the total amount of training but also to the pattern of trials used during training. Specifically, spaced training (distributed over time) is superior at generating long-term memories as compared to  massed training (at very short intervals). "It is a well known psychological principle that learning is better when training trials are spaced out than when given all together," says Dr. Wayne Sossin, neuroscientist and lead investigator. This study identified differences between the two types of training at the molecular level. Interestingly, the process of strengthening communication between nerve cells (neurons), called synaptic facilitation, is controlled by the release of the neurotransmitter serotonin.

http://www.sciencedaily.com/releases/2009/10/091001163730.htm

Brain Training to Stay Sharp #2

ACTIVE reportedly is the largest study ever done on cognitive training. According to George Rebok, an expert on aging and a professor at Johns Hopkins University in Baltimore, who led the study, "What we found was pretty astounding.” Results showed that FIVE years later, participants of the training performed better than their untrained counterparts in all three measures. Not only that. Participants evidenced improvements in reasoning skills and processing speed that could be detected as long as TEN years after the course ended. This is very welcome news in the search for ways to keep the mind sharp as 76 million baby boomers in the United States advance into old age. A new study may soon be underway that attempts to identify whether this training actually strengthened the brain in the way that exercising builds muscle. Last week, NIA put out a request for proposals that would study whether cognitive training causes physical changes in the brain. That study will be fun to follow!

http://www.newsmaxhealth.com/Health-News/brain-training-cognitive-skills/2014/01/13/id/546707

Brain Training to Stay Sharp #1

It always surprises me when I hear people poo-poo the concept of “brain training to stay sharp.” A colleague of mine, Tom Butler, isn't one of them. Last month he surprised me (a great early birthday gift) with information about  a study known as the Advanced Cognitive Training for Independent and Vital Elderly (ACTIVE).  For those of you who are on board with the research about slowing the onset of symptoms of aging, findings of the ACTIVE study were recently published in the Journal of the American Geriatrics Society. And, for those of you who are actually doing 30 minutes of challenging brain exercises every day, this might just help motivate you to keep doing it! The study involved a federally sponsored trial of 2,832 adults with an average age of 74 when they began the program. The goal was to evaluate how three brain training programs focusing on processing speed, memory, and reasoning ability impacted cognitively normal adults as they aged. Note: the training consisted of 10-12 sessions lasting 60-75 minutes each. We’re not talking about 30 minutes of challenging brain exercise a day for the rest of your life; just 10-12 sessions. The results of the study tomorrow.