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Aphasia as a Symptom of Autoimmune Encephalitis

Aphasia as a Symptom of Autoimmune Encephalitis

May-26-2020 | Sarah Reitz, PennNeuroKnow.com

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 Aphasia is a language disorder affecting at least 2 million Americans that impairs the ability to produce or comprehend speech, along with the ability to read or write1. It can result from any injury or disease that damages the language centers of the brain2, including autoimmune encephalitis (AE). To better understand aphasia in the context of AE, it is important to first know how the brain processes language as well as the different types of aphasia that can occur as a result of damage to any of these language centers.

How is language processed in the brain?

The ability to comprehend and use language is a complicated process that involves many regions of the brain. A few of the areas typically associated with language include Broca’s area, Wernicke’s area, the insular cortex, and the angular gyrus (Figure 1). Broca’s area, found in the left frontal cortex, together with the insular cortex, are important for producing language (called expressive language). Wernicke’s area and the angular gyrus, both located in the temporal lobe, play a major role in understanding the context and meaning of language (called receptive language)3-5.

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What is aphasia?

There are many different types of aphasia, ranging from mild forms that involve occasionally struggling to find the right words, to much more severe forms that limit nearly all forms of communication. The level of aphasia varies dramatically from patient to patient, with each person having his or her own unique language difficulties. Still, aphasia has been classified into a few main subtypes based on the main language deficits experienced by the patient (Figure 2).

 

One of the more mild forms of aphasia is anomic aphasia. People with anomic aphasia have difficulty finding the right words or naming things, sometimes describing the phenomenon as having the word on the tip of their tongue. However, they can often describe the word in detail, such as “the blue pants made of denim” when they are looking for the word “jeans”. Sometimes, patients will only have difficulty naming specific categories of words (such as nouns, or only words related to a specific activity), though the reasons for this are unknown. They are able to speak and write fluently, though they will often use vague terms to describe the word they are having difficulty with. Additionally, the ability to read remains intact6.

 

Intermediate forms of aphasia include Broca’s aphasia and Wernicke’s aphasia2,6. Like their names suggest, these types of aphasia result mainly from damage to either Broca’s or Wernicke’s areas. Broca’s aphasia is sometimes known as “non-fluent aphasia” due to the extreme difficulties in speech production experienced by the patient. Expressive language is severely limited, with speech oftentimes consisting of fewer than 4 words at a time. In contrast, patients with Wernicke’s aphasia can still produce fluent speech without much effort, but their ability to understand the meaning of words is impaired. So, while they can connect words into sentences and speak easily, the sentences themselves will usually not make sense and often consist of jumbled, disjointed words. Because comprehending the meaning of words is damaged, patients with Wernicke’s aphasia also have a very difficult time with reading and writing. Other people experience various combinations of these two types of aphasia, which are classified in Figure 2 based on the symptoms exhibited6.

The most severe form of aphasia is global aphasia. This disorder severely limits both receptive and expressive language. The ability to read and write is nearly nonexistent, as patients can neither comprehend nor produce language. While their language abilities are almost absent, patients with global aphasia can have normal non-language related intellectual and cognitive capabilities2,6.

Interestingly, aphasia is not limited to written and spoken languages. Because visual languages like sign language are also processed and produced by the language centers of the brain7, they can also be impaired, causing sign language speakers to lose the ability to use or understand sign language2,8.

Aphasia in autoimmune encephalitis

Aphasia is caused by damage to the language centers of the brain. While strokes are the most common cause9, any disease or injury that damages the regions of the brain involved in language can cause aphasia. These include things like brain tumors, traumatic brain injuries, progressive neurodegenerative disorders, and autoimmune encephalitis. The specific regions damaged by the injury or disease will determine which type of aphasia results2.

 

There are multiple case reports that document instances of aphasia in patients with autoimmune encephalitis. Many of these patients suffer specifically from anti-NMDAR encephalitis10-13, although aphasia has also been recorded in other types of AE14. A 5-year study of 501 patients diagnosed with anti-NMDAR encephalitis tracked symptom development and found that speech problems are common early symptoms. Around 70% of patients over the age of 12 showed some form of a speech disorder, while nearly 85% of those younger than 12 exhibited symptoms of a speech disorder10! In at least one case, however, aphasia developed 19 months after the first AE symptoms, suggesting that there is no definitive timeframe for development of this disorder12.

 

Various types of aphasia have been reported with AE. Some patients show symptoms similar to Broca’s aphasia, with effortful speech and trouble producing language11. Others have reported more severe, global aphasia consisting of trouble both comprehending and expressing language12. In one case, a woman with anti-NMDAR encephalitis had persistent aphasia for 6 months with no other symptoms typically experienced in AE13. What is clear is that aphasia in AE, just like aphasia caused by other injuries or disease, exists on a spectrum and can include any number of language impairments.

 

 

Recovery from aphasia

Because so many injuries and diseases can cause aphasia, there is no standard treatment for the disorder. Each patient must be treated individually, taking many factors into consideration. However, speech-language therapy is critical for all types of aphasia2.

 

One major factor considered in treatment plans is the cause of the aphasia. Aphasias resulting from neurodegenerative disorders show much lower rates of recovery due to the progressive nature of the neurodegeneration15. However, aphasia resulting from other causes will usually show at least some degree of improvement with proper treatment2. In the case of autoimmune encephalitis, aphasia symptoms tend to disappear once appropriate treatment for the underlying encephalitis is administered11-14.

 

Other factors that may determine a patient’s outcome are the type of aphasia the patient is experiencing as well as the level of communication skills prior to aphasia onset. A rich vocabulary before the onset of aphasia tends to predict a better recovery2.

 

Advancements in aphasia research, and the underlying diseases that cause it will continue to inform better treatments, improving outcomes for millions of people across the world and helping them regain their communication abilities.

Download Aphasia .pdf

Image References:

Figure 1 image via The Conversation, CC BY-ND. https://theconversation.com/what-brain-regions-control-our-language-and-how-do-we-know-this-63318

Figure 2 image via the National Aphasia Association, https://www.aphasia.org/graphic-assets/

References:

  1. Aphasia Statistics. National Aphasia Association Available at: https://www.aphasia.org/aphasia-resources/aphasia-statistics/. (Accessed: 2nd May 2020)
  2. Damasio AR (1992) Aphasia. NEJM 326(8):531-539
  3. Friederici AD (2011) The brain basis of language processing: from structure to function. Physio Review. 91(4)1357-1392
  4. Van Ettinger-Veenstra H, McAllister A, Lundberg P, Karlsson T, Engstrom M (2016) Higher language ability is related to angular gyrus activation increase during semantic processing, independent of sentence incongruency. Hum. Neurosci. 10:110
  5. Oh A, Duerden EG, Pang EW (2014) The role of the insula in speech and language processing. Brain Lang. 135:96-103
  6. Aphasia Definitions. National Aphasia Association Available at: https://www.aphasia.org/aphasia-definitions/. (Accessed: 2nd May 2020)
  7. Hickok G, Love-Geffen T, Klima ES (2002) Role of the left hemisphere in sign language comprehension. Brain and Lang 82:167-178
  8. Damasio A, Bellugi U, Damasio H, Poizner H, Van Gilder J (1986) Sign language aphasia during left-hemisphere amytal injection. Nature 322:363-365
  9. Aphasia FAQs. National Aphasia Association. Available at: hyyps://www.aphasia.org/aphasia-faqs/. (Accessed: 2nd May 2020)
  10. Titulaer M et al. (2013) Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 12:157-165
  11. Bhat P, Ahmed A, Jolepalem P, Sittambalam C (2018) A case report: anti-NMDA receptor encephalitis. Journal of Comm Hospital Internal Med Perspect 8(3):158-160
  12. Mario LSJ, Ramiro R, Ruth DVA, Jose F, Carmen OL, Mariana EN (2017) Cortical aphasia and apraxia as main clinical presentation of anti-NMDAR encephalitis relapse with a positive CSF PCR for cytomegalovirus. Neuropsych 7(5):620-628
  13. Constantinides VC et al (2018) Anti-NMDA receptor encephalitis presenting as isolated aphasia in an adult. Neurocase 24(4)188-194
  14. Hayata Y, Hamada K, Sakurai Y, Sugimoto I, Mannen T, Takahashi Y (2014) Anti-glutamate ε2 receptor antibody-positive and anti-N-methyl-D-aspartate receptor antibody-negative lobar encephalitis presenting as global aphasia and swallowing apraxia. Case Rep Neurol 6:291-296
  15. Primary Progressive Aphasia. National Aphasia Association Available at: https://www.aphasia.org/aphasia-resources/primary-progressive-aphasia/. (Accessed 12th May 2020)

 

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Become an Advocate by sharing your story. It may result in someone receiving an accurate diagnosis who is suffering right now and is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

 

 

International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.

 

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A Picture Paints 1,000 Words

A Picture Paints 1,000 Words

June 27, 2018 | Camilla Svensson

Our 15 year old daughter, Amanda, was diagnosed with autoimmune encephalitis and found antibodies of GAD65 in blood samples. Everything began two months ago, when she became very tired and suffered from headache and nausea and began to get cramps in one leg. A month ago, her personality changed, and she became like a completely different person. Then we realized something was wrong and drove her to the hospital.

 

 Amanda has always been a positive girl and great at school and in sports. She is also a good artist and loves to draw and paint. But this disease is so horrible it has taken all of it away from Amanda.

We were lucky and got one of the best doctors in Sweden, Johan Lundgen M.D. and he made sure she got treatment quickly. She got steroids, and then IVIG, and just had her first round of rituximab .

This picture was drawn at the beginning of her illness. It was the first picture she managed to draw after the first major attack of her autoimmune encephalitis. This artwork contains so much emotion.  She didn’t need to try to verbally explain anything to us, which was a struggle for her.  So without her having to talk, we understood what she meant through this art.  Now long after this was drawn, Amanda’s health declined, and she became much worse again.  She couldn’t draw properly after that.

Yet now we hope it’s about to turn again. Today she has started drawing again. She is a real fighter. We know we have a long journey ahead.  Our hope and the emergence of Amanda’s art keeps us going that days full of promise are ahead.


Donate to Support IAES and our Life Saving Mission


International Autoimmune Encephalitis Society (IAES) is a Family/Patient centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey.

Driven by the knowledge that “Education is Power”, International Autoimmune Encephalitis Society manages an educational support group for patients diagnosed with Autoimmune Encephalitis and their loved ones, empowering them to be strong self-advocates and advocates that will lead them to best outcomes and recovery. We are the premiere organization leading in these vital roles.

June is Aphasia Awareness Month

June is Aphasia Awareness Month

June 19, 2018 | Michelle Skimmons

What is aphasia?  Until recently, I had never heard the term before, however after John’s, my fiance’s, diagnosis of autoimmune encephalitis in 2016, I was now hearing some new and uncommon (to me) terms. This happened to be one of them.

Aphasia is simply put, an impairment of language and affects the comprehension of speech and the ability to read or write. Aphasia is always due to injury to the brain, from stroke, injury or neurological disease. In the case of AE this will be an ABI, acquired brain injury, due to the disease itself. Like a patient who has had a stroke, the damage will cause the patient to have difficulty in speech, trouble understanding or difficulty with word recall.

 

It can be frustrating for the patient, as well as for their loved ones, as it can create difficulty in daily function as well as relationships. I think knowledge is so very important and will assist with the communication and relationship issue potentially.

 

As a caregiver or loved one, it is very important not to “talk down” to the person suffering from aphasia. Give them time to complete their thought and please do not finish their sentences for them. This will only cause more frustration for them.  The inability to communicate as they once did is in no way a reflection on intelligence. It is simply the result of the injury. Learn to be a good listener and let your loved one express themselves fully. And in cases of very severe aphasia, keep it as simple as possible and let their response be a simple yes or no.

Ultimately patience is key, and you will learn a new way to communicate with one another. Once the underlying cause is treated, the main treatment for aphasia is speech therapy.

 

 

June is Aphasia awareness month. According to the National Aphasia Association, over 2 million people in the United States are affected by Aphasia yet 84.5 % of Americans state they have never heard the term aphasia.

Please be sure to help share and educate those around you, for the sake of your loved one and yourself.
#aphasiaawareness


Donate to Support IAES and our Life Saving Mission


International Autoimmune Encephalitis Society (IAES) is a Family/Patient centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey.

Driven by the knowledge that “Education is Power”, International Autoimmune Encephalitis Society manages an educational support group for patients diagnosed with Autoimmune Encephalitis and their loved ones, empowering them to be strong self-advocates and advocates that will lead them to best outcomes and recovery. We are the premiere organization leading in these vital roles.

Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY  provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society  cannot provide medical advice.


International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.

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