However, new research is showing us what happens in the brain at the genetic level when the brain is mildly injured many times or severely injured one time to cause neurological deterioration over time and the eventual development of dementia.Continue reading →
Today, February 7, 2016, the 50th Super Bowl game in NFL (National Football League) history will be played by the Carolina Panthers and the Denver Broncos. Therefore, it is appropriate to discuss the other dark and dangerous side of this football game – and all the ones played before and all the ones that will be played after – before it is played.
Super Bowl games have become extravagant and lavish productions in the last ten years or so, intended to bring into the audience people who normally either don’t have interest in the game or don’t normally watch football. Super Bowl games also represent an obscenely huge financial windfall for the NFL and for advertisers with enough money to pay for the coveted and outrageously expensive advertising spots during the game.Continue reading →
Our brains are very soft organs that are surrounded by spinal fluid and are protected by the hard outer covering of our skulls.
Under normal circumstances, spinal fluid cushions the brain and keeps it from crashing into the skull. However, if our heads or our bodies are hit hard, our brains can slam into our skulls and result in traumatic brain injuries (TBIs). TBIs are also caused when the skull is fractured and the brain is directly damaged by outside force.
Although concussions, which we’ll discuss later, are sometimes referred to as mild TBIs, the reality is that no injury to the brain is mild and repeated injuries will lead to neurological degeneration that includes dementia.
TBIs are complex neurological injuries that result in a wide variety and severity of symptoms and disabilities.
The least severe symptoms of TBIs – and these may not happen immediately and, in fact, may occur some time after the injury, can include:
Temporary loss of consciousness
Temporary memory loss
Grogginess and sleepiness
Double vision or blurred vision
Nausea or vomiting
Sensitivity to light
Slow reaction to stimuli
The most severe symptoms of TBIs can include:
Extended loss of consciousness or coma
Permanent and severe brain damage
Partial or complete motor paralysis
The most common causes of TBIs, according to the Centers for Disease Control, are:
Car accidents (14.3%)
Head/body collisions with people or things (15.5%)
In the category of TBIs from falling, most of the falls occur disproportionately in the very young (55% of falls among children occur in children between the ages of 0 and 14) and the very old (81% of falls among adults occur in adults who are 65 or older).
Most of the TBIs in the Other category (19%) are from personal firearms and military weapons.
A type of TBI that is more frequently in the headlines today is Chronic Traumatic Encephalopathy (CTE). CTE is brain damage that occurs as a result of repeated concussions (a concussion is defined as injury to the brain from a direct blow to the head or from the head or upper body being violently shaken).
The first identified variant of CTE was described in 1928 by forensic pathologist Dr. Harrison Stanford Martland as pugilistic (from the Latin word pugil, which is translated as “boxer” or “fighter”) dementia. The symptoms included tremors (Parkinsonism), slowed movement, mental confusion, and speech difficulties.
In 1973, the neuropathology of pugilistic dementia was discovered and described by a team of pathologists led by J. A. Corsellis who documented their findings after performing thorough autopsies on the brains of 15 deceased boxers.
Former boxing heavyweight champion Muhammad Ali began boxing in Kentucky when he was 12 years old.
By the age of 18, he had boxed his way to the heavyweight gold medal at the Olympics (1960).
A few months later Ali began his professional boxing career. He quickly gained national prominence because of his skill in the ring and his trademark quote: “Float like a butterfly, sting like a bee. The hands can’t hit what the eyes can’t see.” He boxed professionally until his retirement in 1981.
In 1984, Ali was diagnosed with Parkinsonism (the tremors of pugilistic dementia) and his neurological health has deteriorated steadily to include all the advanced symptoms of this variant of CTE.
His wife, Lonnie, is his caregiver and contributed to a moving article that AARP published last year about what she and Ali deal with on a daily basis as a result of the neurological degeneration that CTE has caused.
CTE has increasingly become a major health concern in the high-contact sports of professional wrestling, ice hockey, soccer, and football as more and more current and retired athletes are showing symptoms consistent with CTE.
In recent years, football – and especially professional football – has become the focal point for a closer examination of CTE. Not only has this sport become more violent in terms of how the game is played, but how concussions are treated – or not treated – has also come under greater scrutiny.
A class action lawsuit has been filed – and a tentative agreement reached with the NFL – by retired NFL football players and/or their families (some of the players have already died from neurodegenerative causes) which claims that players were not (a) adequately protected from suffering concussions, (b) medically treated properly following concussions, and (c) provided adequate medical compensation to treat the burgeoning costs of CTE as it progresses.
This gist of this lawsuit is that the NFL used – and abused – these players to fabulously guild the seemingly-endless coffers of the NFL, often forcing the players by intimidation or fear to get back on the field as soon as they could after suffering a concussion (often in the same game), and then abandoned their responsibility to their former employees (as part of their contractual agreement) as soon as the employees began costing them money instead of making them money.
If there is a silver lining in all of this, it is that the younger NFL players have a much greater awareness of the relationship – and their increased risk – between professional football and CTE.
They are aware of the very real probability that they will be one of the 1 out of every 3 players who develops CTE.
And they’re choosing their long-term health, including their brain health, over temporary fame and fortune.
An unprecedented number of younger – and in-their-prime in the professional football world – NFL players have already retired before the 2015-2016 season begins.
Cortland Finnegan – Age 31
Jake Locker – Age 26
Jason Worilds – Age 27
Chris Borland – Age 24
While Finnegan, Locker, and Worilds did not publicly cite CTE as a factor in their premature retirements from the NFL, there can be no logical reason to doubt that the mounting evidence was a factor in their decisions.
Borland just finished his rookie season (2014-2015) with the San Francisco 49ers, but he revealed after the season that he suffered a concussion in training camp last fall. Instead of reporting the concussion, Borland covered it up so that he could continue to practice and win a starting position on the team.
This is the kind of competitive pressure that gets put on these young players by the NFL (yes, Borland made the decision and he bears the responsibility for it, but had he reported the concussion, he would have been replaced and lost the starting spot and may not have played all season).
Fortunately, though, Borland came to his senses and realized how much he had jeopardized – and would continue to – his neurological health.
As he said on the March 16, 2015 edition of ESPN’s Outside the Lines, “”I just thought to myself, ‘What am I doing? Is this how I’m going to live my adult life, banging my head, especially with what I’ve learned and knew about the dangers?'”
We can only hope that more athletes in high-contact sports will know the higher risks of TBIs they face, not just in the professional leagues, but at the amateur levels, and they will choose to walk away from certain neurological damage.
In the meantime, we have a better understanding just in our daily lives of how TBIs can happen and what the results can be, so I hope that we’re a little more observant and attentive after falls with our little loved ones and our older loved ones, especially those already going through the journey through dementias and Alzheimer’s Disease, as they are even more prone to falling than the general elderly population.
As Sergeant Phil Esterhaus says at the end of every roll call on Hill Street Blues (a favorite TV show of mine during my high school and college years), “Hey, let’s be careful out there.”
There are several types of common early-onset dementias. Early-onset dementias are categorized as dementias where the onset of symptoms is prior to age 65. These dementias can occur as early as the 30’s, but more typically become symptomatic in the 40’s and 50’s.
Early-onset dementias, unfortunately, are still off the main grid for medical staff – a classic instance of fixed expectations that dementias won’t be an issue for a person until after age 65 – and our loved who are diagnosed with early-on dementias face challenges that our older loved ones who are suffering with these diseases don’t face. These include:
Difficulty getting a correct diagnosis
Loss of employment and income
Difficulty getting Social Security Disability Insurance, Medicaid, and other employment-related disability insurance
Loss of health insurance and high-out-of-pocket costs for medical care
High out-of-pocket costs for long-term care
Lack of appropriate medical care, residential care, and community services (all of these are geared toward an older population)
Early-onset dementias typically are harder to diagnose because other than the dementia systems, sufferers are usually healthy, active, and aware there is a problem.
Additionally, the symptoms of early-onset dementias usually don’t have memory impairment as the predominant feature. Most often, behavioral and personality changes occur first, so usually the first type of treatment is psychiatric instead of neurological.
The causes of early onset-dementias fall into three categories: random, genetic, and lifestyle.
Random early-onset dementias are just that. There’s no concrete link to a cause. My opinion is that few of these in this category are actually random, but the causative issue(s) have not been identified yet.
Genetics plays an important role in certain early-onset dementias (and, although the scientific community has overlooked or disregarded the familial aspect of elder-onset dementias, it appears very likely, from observation, that if there’s a family history of elder-onset dementias, there may be a genetic predisposition for development of elder-onset dementias in subsequent generations).
Three genes are known to have mutations in the case of some sufferers of early-onset dementia, Alzheimer’s type (symptoms related to these genetic mutations usually begin in the 30’s and 40’s):
Amyloid precursor protein gene (APP) on chromosome 21
At least 1/3 of all sufferers diagnosis with early-onset dementia have Alzheimer’s Disease (remember that Alzheimer’s Disease is a type of dementia, but is not inclusive of all types of dementia, just as all photocopiers are not Xerox photocopiers and all facial tissues are not Kleenex facial tissues).
This type of early-onset dementia is more common in women than men. Once symptoms appear, the duration of the disease averages eight years.
A recent example is the 2011 diagnosis of former University of Tennessee women’s head basketball coach, Pat Summit, who was diagnosed with early-onset dementia, Alzheimer’s type, at age 59. Coach Summitt stayed with the team one more season, but was not actively coaching that season.
Coach Summitt retired in 2012 and has begun the Pat Summit Foundation to raise Alzheimer’s Disease awareness.
The novel, Still Alice, written by neuroscientist Lisa Genova, gives a scientific, compassionate and compelling look from the inside out of a 50-year-old Harvard psychology professor as early-onset dementia, Alzheimer’s type enters and progresses through her life.
Since the publication of Still Alice in 2007, Genova has continued her work with bringing the neuroscience of all types of dementias in the same compassionate and compelling style of her first novel through subsequent books and through documentaries produced with her husband, who is a filmmaker.
The second most common type of early-onset dementia is vascular dementia. Vascular dementia can occur because of:
Multiple cortical infarcts (small areas of tissue that have died from the lack a blood supply) that are most often caused by transient ischemic attacks (TIA’s) or silent strokes and characterized by stepwise deterioration of cognitive function
Small-vessel disease, resulting in a more subtle decline of cognitive function
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
Rare cause of early-onset subcortical strokes and dementia
Caused by a mutation of Notch 3 gene on chromosome 19
MRI shows diffuse white matter lesions on the cerebral hemispheres, especially in the anterior temporal lobes and external capsules
With early-onset vascular dementia, there are usually lifestyle factors involved such as uncontrolled or undetected high blood pressure and an unhealthy diet. Recent scientific research has also linked high cholesterol levels with the development of vascular dementia.
The third most common type of early-onset dementia is frontotemperal dementia (FTD), also known as Pick’s Disease, which affects the frontal and temporal lobes of the brain. FTD usually has an onset between the ages of 45 and 65. Its average duration is eight years.
There are three types of FTD: behavioral variant FTD, semantic dementia, and primary progressive (also known as progressive nonfluent) aphasia.
In about half the cases of FTD, there is a positive family history for the disease, indicating a probable genetic link (although researchers have not yet identified the genetic mutation).
FTD can co-occur with motor neuron diseases (ALS, also known as Lou Gerhig’s Disease, is an example of a motor neuron disease), but only about 10% of sufferers of only motor neuron diseases develop dementia, resulting in a very aggressive course of the illness.
FTD presents differently from early-onset Alzheimer’s Disease and early-onset vascular dementia because the first symptoms involve changes in personality and social conduct while memory, perception, and visuospatial skills remain unchanged.
The most common indicators are:
Speech and language difficulties
As FTD progresses, other symptoms become apparent:
Difficulty behaving appropriately in new and unfamiliar situations
Loss in inhibitions (disrobing is not uncommon)
Loss of social skills
Executive function deficits
Decreased verbal fluency
Compulsive or repetitive behavior
Lack of insight
Inappropriate sexual behavior
The semantic dementia form of FTD includes symptoms of:
Difficulty with correctly naming objects (people, places, and things)
Impaired understanding of the meaning of words
Inability to understand substitute words
However, in this form of FTD, speech remains fluent and cognition remains intact. MRI scans show more atrophy of the anterior temporal lobe than the posterior temporal lobe.
The primary progressive (progressive nonfluent) aphasia form of FTD is characterized by:
Progressive decline in all language skills, with no other cognitive deficits
Increased difficulty with speech and speaking (by the end of the disease, most sufferers don’t speak at all)
Speech and speaking is not fluent and requires a great deal of effort
MRI scans show predominant atrophy of the left perisylvian region of the temporal lobe.
The fourth most common type of early-onset dementia is Lewy Body dementia. I’ve included the link to my post about Lewy Body dementia for a full description, but will include a brief summary of the dementia’s primary symptoms:
The fifth most common type of early-onset dementia is Wernicke-Korsakoff Syndrome (alcohol-related dementia). This is a lifestyle dementia, brought on by long-term, heavy alcohol consumption.
Characteristics of Wernicke-Korsakoff Syndrome include:
Damage to the limbic structures and frontal lobes
Executive functioning impairment
Autobiographical memory is frequently affected resulting in confabulation (making up stories)
Memory loss stops where it is when drinking stops (damage already done remains)
As shown by the MRI scan above, there is general cortical atrophy along with damage to the frontal, parietal and cingulated regions of the brain, with the majority of the damage occurring in the frontal lobe.
There are two other less common types of early-onset dementia that we’ll discuss.
Everyone is born with this gene. However, in Huntington’s Disease, an inherited mutated copy of this gene (on chromosome 4), produces a defective form of the huntingtin protein that causes degeneration and death of the neurons, especially in the center of the brain.
Because this gene is a dominant gene (as opposed to a recessive gene), everyone who inherits the mutated copy of the gene will, at some point, develop Huntington’s Disease.
Symptoms typically appear between ages 30 and 50, but it can begin at a very young age or appear in the very elderly. Primary symptoms include:
Lack of muscle coordination in the arms, legs, head, face and upper body
Progressive decline in thinking and reasoning skills, including memory, concentration, judgment and the ability to plan and organize
Mood disturbances, including depression, anxiety, anger, and irritability
The last type of early-onset dementia, which is extremely rare, is Creutzfeldt-Jakob Disease (CJD). CJD is characterized by rapid neurological degeneration. It is always fatal, and death usually occurs within six months to a year of onset.
CJD belongs to a class of human and animal diseases called transmissible spongiform encephalopathies (TSEs), because the infected brain looks like a sponge. The average age of onset for CJD is 60.
“Mad Cow Disease” is the bovine equivalent of CJD (although it tends to affect younger people, with the average age of onset being 26).
There are three types of CJD:
Sporadic (no known cause) – accounts for about 90% of cases
Inherited (family history of the disease) – accounts for 5-10% of cases
Acquired (transmitted by exposure to brain or nervous system tissue, usually through certain medical procedure) – accounts for less than 1% of cases
The symptoms of CJD include:
Rapidly progressive dementia
Problems with muscular coordination
Personality changes, including impaired memory, judgment, and thinking
As CJD progresses, mental impairment becomes severe. Sufferers often develop involuntary muscle jerks (myoclonus), and they may go blind.
Eventually, they lose the ability to move and speak and become comatose. Pneumonia and other infections often occur as well, and they generally end in death.