FAS-Foreign Accent Syndrome

In the late 1800’s, neurologists Broca and Wernicke discovered lesions (or damage) to the left side of the brain that could possibly destruct speech patterns. Today this is known as Foreign Accent Syndrome or (FAS). FAS is a neurological disorder that causes a person’s speech to be gradually or suddenly accented. It affects both Broca’s area and Wernicke’s area in the brain. The underlying effects of FAS are often led by traumatic brain injuries, hemiplegic migraines, strokes, and/or multiple lesions to the brain (Garst & Katz, 2006).

brocas area
Broca’s area & Wernicke’s area located on left hemisphere of the brain.

The left hemisphere of our brain is generally associated with areas that process speech, language, logic, and facts. The right hemisphere is usually affiliated with our surroundings, such as spatial recognition, art, music, and understanding tone. Broca’s area, located in the left frontal lobe, is responsible for forming our words while Wernicke’s area, located near the left temporal lobe, puts those words into sentences (NIH, 2008). Neurologists find that these parts of the brain have something to do with speech production and thus resulting in the patient sounding foreign. This result can cause placement of tongue, pronunciation issues, and slurring of words when speaking (UTD, 2015). Now that you have an understanding of FAS and of the brain, let’s move on to how a patient can take this info and their symptoms to the doctor.

Proper diagnosis comes first for a FAS patient. A patient who may experience slurring of words, frequent headaches, stroke-like symptoms, or memory loss, must seek medical attention immediately. Although with the rarity of FAS, these symptoms can present themselves at any time to any healthy person. A treatment plan for FAS doesn’t end with the first doctor’s visit. A patient with this syndrome will visit a radiologist, neurologist, speech therapist, and a neuropsychologist for months, if not years to improve their situation (Garst & Katz, 2006). A patient will undergo multiple CT scans and MRI’s, but what is most important is the fMRI (functional MRI). The fMRI measures activity in the brain by measuring its blood flow whereas the MRI only shows contrasting tissue images of the brain (UCSD, 2015). In order for doctors, scientists, and researchers to understand this syndrome further, fMRI’s should be the basis of all brain scans for these patients. Since this syndrome is so rare, many doctors and researchers want to and should learn more about how FAS affects the brain and speech.  A very popular case study of FAS occurred in 1941 during a German air-raid when a Norwegian woman was injured on the left side of the brain by scrap metal. She experienced speech loss and improved language abilities within the year after the incident. During this time, doctors diagnosed her with FAS (Garst & Katz, 2006). On the other hand, patients who suffer from Aphasia (meaning without speech) can experience speech loss due to the lesions or neural damage on or near Wernicke’s area. FAS patients tend to also mispronounce or delete certain vowels or consonants while speaking (Garst & Katz, 2006). Even though these areas are in different locations of the brain, they are both involved in key aspects of processing speech.

Ellen Spencer, from Indiana, is one of the rare documented cases that currently lives with FAS. She did not suffer from a stroke, but a severe headache. Even though her speech is heavily accented, her singing sounds much like her original voice. In her video, listed below, she sings “Amazing Grace” and her foreign accent is nowhere to be found. Why is this you ask? The right side of the brain is responsible for music and singing information, therefore she is able to use the singing area of her brain to alleviate the foreign accent. I had the opportunity to talk with Ellen via email and her side of the syndrome is very thought-provoking. She says, “The psychosocial aspects can be devastating. Since it’s so rare, getting validation or proper diagnosis is often first a failure and a challenge.” Ellen has also become an advocate for FAS worldwide and even has contributed to a book on the topic titled Foreign Accent Syndromes: The stories people have to tell that is published by Psychology Press to be used in many universities and hospitals. The book, linked below, is written by Jack Ryalls and Nick Miller.

After a few email tags with Ellen and finding out more information on FAS, I do hope that more research will be conducted on this syndrome because there is not much known about it right now and many patients are being misdiagnosed. Even though it is rare, with approximately 100 documented cases, the underlying events that lead to FAS happen all too often (Youtube, 2015). As headaches, head injuries, and strokes occur worldwide every day, conducting research on this syndrome can possibly lead to a proper diagnosis in future patients. This is essential to prevent further harm, and finding out what exactly is causing each specific case and if there is possibly a cure for those living with FAS.

Here’s a link to the Ryalls & Miller FAS book that Ellen Spencer contributed to:
http://www.amazon.com/Foreign-Accent-Syndromes-stories-people/dp/1848721536

Video of Ellen Spencer:

Video of Ellen Spencer on Outrageous Acts of Science (start at 23:00 minutes):

References

Garst, D., Katz, W. (2006). Foreign Accent Syndrome. The ASHA Leader, 11.10, 10-31. Retrieved on June 20, 2015 from http://leader.pubs.asha.org/article.aspx?articleid=2278208

Miller Ph.D., Dave, Thompson Ph.D., Bert, Harrub Ph.D., Brad. The Origin of Language and Communication. Apologetics Press. Retrieved on June 21, 2015 from http://www.apologeticspress.org/image/rr/2002/r&r0208a.jpg

(2008, October). Aphasia. National Institute on Deafness and Other Communication Disorders (NIDCD). Retrieved on June 21, 2015 from http://www.nidcd.nih.gov/health/voice/pages/aphasia.aspx#causes

About FAS/Speech Samples. (2015). Foreign Accent Syndrome (FAS) Support. Retrieved on June 21, 2015 from http://www.utdallas.edu/research/FAS/about/

What is fMRI? (2015). Center for Functional MRI in the Department of Radiology. Retrieved on June 22, 2015 from UC San Diego School of Medicine http://fmri.ucsd.edu/Research/whatisfmri.html

Spencer, E. (2013, May 11). “Foreign Accent Syndrome-Ellen5e learning and teaching Ellens-FAS_Birthday_4.” [Youtube]. Retrieved on June 20, 2015 from https://youtu.be/tfKj82vYm_Y

Outrageous Acts of Science Season 3. (2015, February 25). “Outrageous Acts of Science Season 3 Episode 6 ‘Tested on Humans.’” [Youtube]. Retrieved on June 23, 2015 from https://youtu.be/SMVlUey6Mc0?t=23m

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What ALS means to me

ALS, also known as Amyotrophic Lateral Sclerosis, is a terminal disease that causes the neurons (nerve cells) in the brain to break down all muscles in the body as well as breathing and talking. The famous baseball player for the Yankees, Lou Gehrig, died of ALS in 1936 and this disease is often named after him (Bear et al, 2001, p. 432). Here’s a breakdown of the scientific name:
A: meaning “no” or “negative”
Myo: refers to the muscles
Trophic: refers to nourishment

Therefore, Amyotrophic stands for “no nourishment to the muscles.” The word “lateral” is the area in the spine where the brain tells the muscles what to do. Lastly, the word “sclerosis” which is often heard with Multiple Sclerosis, stands for hardening. As neural signals from the brain and spinal cord send messages to the muscles that have sclerosis, the messages are stopped due to the “non-absorbable” muscle tissue. In the picture below, you can see that the axon is attached to the muscle. Nerve fibers feed off the axon and contract when triggered by a signal. With ALS, sclerosis hardens on the nerve fibers and blocks the signal from permeating through the membrane to the muscle causing degeneration (Medicaluniya, 2012). The meaning of ALS is very important for patients and families to understand, as well as how it begins to affect the body.

Normal nerve cell and muscle vs. ALS-altered nerve cell and dying muscle.
Normal nerve cell and muscle vs. ALS-altered nerve cell and dying muscle.
In 2005, my family received the news that my Papa was diagnosed with advanced stages of Lou Gehrig’s disease. For us, this was not only a shock, but something that would ultimately turn into a life changing learning experience. Like many others in the world, this was the first time we had encountered ALS and Lou Gehrig’s disease, so we were walking into the situation blind. We were told what to expect many times from many different doctors but nothing quite prepares you for the mental and physical deterioration until you experience it firsthand.

Our family made the decision after we found out about the diagnosis to move my Papa into our house, the doctors had warned us numerous times that he would need more and more special care as the disease progressed. In the beginning, he was still my Papa, he still would pester my cats, he could still sit on the back porch and smoke a cigarette, and he would still try with all of his might to get my brother and myself to watch Alfie with him. Slowly, my Papa began to experience changes, the changes that we were warned of at the doctors, one day he was fine (or so it seemed in my eyes) and the next day he could not walk to the bathroom on his own. He was physically falling apart in front of my eyes; our next step was to get him a motorized wheelchair. You see with a disease that is so physically deteriorating, you have to do everything in your power to try and let the person suffering have what little independence they have left. For us, it was the motorized wheelchair and giving Papa my brother’s room, so he would have a space of his own and feel more comfortable in our home.

Papa and I on prom night.
Papa and I on prom night.
Maybe I was slightly naïve at the time, even after hearing the prognosis from the doctors, but I was still convinced that one day my Papa would get out of his wheelchair. I was not sure how or why something so debilitating had to happen to someone I loved so much, but I was hopeful for a cure. Sadly ALS is not something you recover from, it is something that ultimately takes your life. For my Papa he only continued to worsen after we had to get him the motorized wheelchair. At first it was little changes like his mood over silly things such as getting him the wrong sauce for his fish, then it was bigger; his speech was all but absent. My mother had become a master at reading his lips and was able to help him communicate with us, thankfully. It was not really until the last few months of his battle with his disease that we all came to realize that the only positive outcome from all of this was going to be that he would not have to suffer anymore. In his final months, his breathing was labored, the only thing we could do was place him in hospice until it was his time to go.

While there is still no exact testing for ALS, there are ways to help doctors give a more accurate diagnosis. For this a doctor must rule out other similar diseases and would then be able to tell you if you have the qualifications of ALS. With my Papa, he had suffered from a couple of strokes before receiving the diagnosis of ALS. Many military men who are veterans, like my Papa, also have a higher risk of getting this disease making it a “service-connected disease” (ALSA, 2015). I’ve always wondered if this disease could ever affect someone who has never served in the military or been a spouse of someone who has. This disease mainly affects men (due to their service), however it is not impossible for females to get ALS.

The disease is also considered to be sporadic, as in not hereditary, according to the ALS foundation site (ALSA, 2015). Since there is no exact testing for this disease, there is a part of me that wonders if I or any of my family members could possibly get this terrible disease. The ALS researchers have been making strides in finding biomarkers that would help with earlier diagnosis and possible treatment for anyone might suspect having ALS (ALSA 2015). To care for someone with ALS, is not only hard on the soul but also tough on the pocket. Many households who take care of loved ones with this disease tend to spend a quarter of a million dollars on medical needs, doctor’s visits, and housing care (ALSA, 2015). Coming from experience, money has no factor on taking care of someone that you love in their last days. It is also important to remember that not only the person with ALS is suffering but the loved ones caring for the person affected also are grieving in their own way, and need special attention to help them cope with changes they are watching their loved ones go through on a daily basis.

Although this may be a rare disease among many others out in the world, this is a fatal and quick one. Doctors usually put patients in “stages” according to their illness, but with ALS, patients are given less than five years to live. My Papa lived with ALS less than two years. He also had stage 4 emphysema. To have a disease take you by storm so quickly, it is like dumping a bucket of ice over your head and having your breath taken away. This is how the huge YOUTUBE ice bucket challenge came to be. It was meant to bring awareness to the disease and to raise money for not only testing but hoping to one day find a cure so that maybe one day families will not have to deal with losing someone so quickly and painfully. I’m very proud of anyone who supported the Ice Bucket Challenge and also donated to the ALS foundation so that sooner than later, people that are living with this horrible disease can have a cure. My Papa did have a cure though. He had his family by his side at all times. He accepted Jesus, an answered prayer of my mom’s, and he went with dignity. Although it is still very hard for me to talk about, like I said before, this was one of the greatest learning experiences of my life. I now know about a disease that not everyone has the chance to learn about, and having experienced it first hand, it only makes me want to help support and educate others even more. I will forever support the ALS foundation and their journey of finding a cure!

Here’s an Ice Bucket Challenge video compiled from many users from the ALS Association website

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References
What is ALS? (2015). About ALS. Retrieved on June 8, 2015 from ALS Association http://www.alsa.org/about-als/what-is-als.html

Bear, Mark F., Conners, Barry W., Paradiso, Michael A. (2001). Neuroscience: Exploring the Brain. Baltimore, MD: Lippincott Williams & Wilkins

1st Picture: Leung, Jeremy. (2010, September 4). ALS (Amyotrophic lateral sclerosis). Retrieved on June 8, 2015 from http://len.epfl.ch/webdav/site/len/shared/import/migration/ALS1.jpg

2nd Picture: owned by self

The ALS Association. (2014, September 17). Ice Bucket Challenge: Thank You from The ALS Association. [Youtube]. Retrieved on June 9, 2015 from https://youtu.be/ObC0lcC-hLA?list=UUQD7Myz4Ssut-TNOM_N5HAw

Medicaluniya. (2012, May 21). Lou Gehrig’s Disease – Effects of Lou Gehrig’s Disease. [Youtube]. Retrieved on June 8, 2015 from https://youtu.be/pOvvW8gbWSA

Microglia: The Robin to our Batman (the brain)

Recently in my online class, I learned about the different cells just within the CNS (Central Nervous System). For those of you thinking that we only had a one-part nervous system or just need a refresher course in biology, our nervous system consists of many parts. The main systems that we learn about in grade school are the CNS and the PNS. First, the CNS or the Central Nervous System, controls the brain and spinal cord.  Second, the Peripheral Nervous System, controls everything outside of the brain/spinal cord. The microglial cell is a part of the CNS, which I will be discussing further in this topic.

The swollen microglial cells (shown on the right) can result from a traumatic brain injury.
The swollen microglial cells (shown on the right) can result from a traumatic brain injury.
Microglia cells are non-neuronal cells that are within our brain and spinal cord. The cells act as a special immune system by protecting the brain and removing debris that has been left over by dead neurons, also known as nerve cells. Since the cells act as an immune system, they respond much like how our body responds to the flu or the common cold.

Brain Injury and Microglial Cells

For example, swelling of the brain immediately comes to my mind when a brain injury occurs. Possibilities are endless when it comes to a diagnosis, such as a concussion, stroke, encephalitis, or even something as small as a headache. Much like when you scrape your arm on something sharp, you bleed and minutes later, the platelets in your body come rushing to the rescue and create a healing, protective barrier from the outside world.

The microglia cells do the same thing! They swell up when injured to protect the brain against any foreign invaders.  Crazy? I think it’s pretty amazing!

When a person undergoes brain trauma or even the slightest of injuries to the head or spinal cord, the microglia cells are activated to respond quickly to the trauma site (Streit 2000). According to Streit and his studies, microglial cells aid the injured neurons by restoring them back to health, whereas a severely injured neuron cell will or can release a toxin to alert the MG cell to destroy it. Our bodies have a unique way in taking care of dying cells so that they can do no more harm or cause mutations.

Ending thoughts

In response to what I’ve learned in class about microglial cells and also through reading a few articles, it seems that even something as small as the MG cell, can have a huge impact on our brain and spinal cord in traumatic events. On occasion, we can lose brain cells from injuries, head-banging to our favorite rock song too hard, or dozing off to the laughing gas the dentist gives us, but we can always count on Robin (the microglia cells) to help restore or give a merciful death to our neurons, while Batman (the brain) stays focused on keeping us alive and well!

Please feel free to sound off down below in the comment section! I would love to hear your opinions/thoughts/ideas, etc.  I also found a really neat video showing how microglial cells engulf neural debris (approx. 1 min. long).

Citations:
Video: https://youtu.be/KC-iW1d1p34

Streit, Wolfgang J. (2000). Microglial Response to Brain Injury: A Brief Synopsis.        Toxicologic Pathology, 28(1), 28-30. doi: 10.1177/019262330002800104

Picture: https://www.bcw.edu/cs/groups/images/documents/images/mdaw/mday/~edisp/ucm_001511.jpg

My Hx form 

Hello! My name is Victoria Allen and I’m a SAGE student at CCC. I have lived in PA for less than two years. I was born and raised in a small town in Tennessee, which I then left to head off to California in 2010. I lived in Southern California for three years, and then moved to PA. Those three years were the best years of my 20’s (so far!).

 I’m taking this class to fulfill my science degree electives but I have always been interested in how the brain works. There is so much to discover that we can not even fathom.

I enjoy traveling, going to the movies, eating, hanging out with my friends, annoying my cats, taking naps, and reading. I love my family, friends, and above all, my God. 

 I’m looking forward to this class and everyone’s discussions.  

Nice to meet you!

Welcome to my new blogpost! I’m new to the blogging world and am very excited about learning how to express my ideas through this platform. This summer, I am taking an online class, BioPsychology, that requires me to “blog” over the course of 7 weeks. I will be writing about my thoughts on certain topics throughout the weeks to come and I welcome you to share your thoughts as well!

Thanks for stopping by!

Victoria Allen