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Progressive Cognitive Difficulties in Adult Patients with Mitochondrial Disease

Discussion in 'Cognition' started by BeautifulDay, Oct 5, 2017.

  1. BeautifulDay

    BeautifulDay Senior Member

    Progressive Cognitive Difficulties in Adult Patients with Mitochondrial Disease

    Association of British Neurologists (ABN) joint meeting with the Royal College of Physicians (RCP), London, 23–24 October 2013

    "Conclusion: These findings demonstrate widespread cognitive difficulties that can undermine independence and quality of life in patients with mitochondrial disease, and emphasise the need to incorporate cognitive testing into clinical practice, in order to ensure that appropriate care is provided. These findings serve as an informative guide for assessing the true nature and severity of cognitive disability in patients with mitochondrial disease and preface future studies assessing speed of progression and final cognitive outcome, whilst providing clues to the role of mitochondrial dysfunction in other neurodegenerative conditions and age–related cognitive decline."

    A decade ago, I started mixing up words. For example, meeting a new person I once said "Nice to foot you". Then it started happening all the time. I mentioned it to my neurologist and was told to decrease my stress and get more sleep.

    Then I started forgetting things. I'd lose focus. I'd enter a room and forget why I was there. I'd ask the same question over and over again and forget that I was already told the answer. I owned my own business at the time and met a client one day and over the weekend we were at an event and the client came over to talk business and I didn't remember anything about her or meeting her previously. She thought I was nuts. My husband took me over to the emergency stairs where I had my first cry of "Oh My God, I'm losing my mind".

    My business went under because I couldn't remember anything or what I was doing. I went into the business because it was supposed to be just something I could do in spare time and work around my Chronic Fatigue.

    Then I started forgetting my kids names, my husband, and I went back to the neurologist and said I'm forgetting things. Major things. Again she said reduce stress and get more sleep.

    Not once did she mention cognitive testing. Instead she assumed fatigue and memory lapses and my odd symptoms meant hypochondriac. So I went to a different city, New York City, where I underwent cognitive testing at a top university. They found severe cognitive loss.

    My doctor explained that people with high cognitive reserve who have memory loss often aren't taken seriously until they are far along. So while I looked well (with the exception of my malar butterfly mask) and I could pretend to keep up with a conversation - ask me about that conversation 15 minutes later and it was gone.

    I learned to use cognitive helpers from a 5 foot calendar in the kitchen, notes everywhere, alarms on my phone, my husband GPS'd my phone, etc.... I was given a prescription of Donepezil. I started doing research and found the MIND diet. It really helped. Now I can have music on in the house and keep up with conversations (well some of the time).

    But I still have bouts of words getting mixed up. I still need to use the calendar, etc.... I have severe amnesia episodes regularly where I can't remember an event. Luckily people fill the events in for me.

    If I write something here, it has probably taken me 5 times as long as you think because I have to keep rereading what I wrote to remember the path I'm taking here.

    My doctor calls these episodes as being due to "attention". It's not that I'm not paying attention. It's that we have these buckets of short term memories happening and they get dumped into long term memory buckets. He says my short term memory bucket has developed holes. I'm doing a horrible job of explaining it the way he does.

    Since the memory issues developed I also have ADHD and OCD. I can't focus on anything my brain doesn't want to focus on. When my brain wants to focus on something, it's very hard to walk away. I've walked away from conversations with friends without explaining because it's just too painful to be subject to whatever the conversation was (despite the fact that it's rude and something that actually should be important to me). I was in church the other day watching our 8 year old daughter sing in the choir. It was so difficult to sit there during the sermon and readings. I was clinging by a string from walking out. It's a little karma getting me back for forcing our son (who later we learned was high functioning autistic spectrum with ADHD and OCD) to sit in church for a few years.

    In the last year, when we were diagnosed with Mitochondrial Disease, it all made sense.

    Going back to cognitive testing. Anyone who thinks they are having cognitive loss should get tested. My memory loss neurologist can't believe my past neurologists didn't recommend cognitive testing for me earlier when I mentioned losing my memory. He said that's the time to have a test so that continued memory loss can be documented by comparing test results over time.
    Sancar, Maya24, Chezboo and 13 others like this.
  2. Chocolove

    Chocolove Tournament of the Phoenix - Rise Again

    Wonderful! You may be needing a lot of nutrients, or certain forms of nutrients. Have you run your genetics? It might help decipher nutrient wise what you and possibly what your son needs. Otherwise look for common dietary deficiencies in your population or due to drug depletion. etc. I think you are really on to something here. Mitochondrial functions depend on certain nutrients and co-factors.

    Remember that most doctors are not trained in nutrition, thus they will tend to prescribe drugs which will complicate matters if problems are due to nutrient deficiencies. A good nutritionist might be able to help.

    Thank you for taking the time and effort to post. :hug:
    BeautifulDay, merylg and pattismith like this.
  3. Alvin2

    Alvin2 If humans were rational...

    I'm so sorry to hear about this, i also have cognitive issues but not at the level you do. Did the donepezil help at all?
    Sancar, BeautifulDay and merylg like this.
  4. pattismith

    pattismith Senior Member

    You do explain all these things so well!:)

    I can identify to many of your troubles....:thumbsup:
    Forgetting why I went into one or another room. Forgetting to bring something my husband asked me about just a few minutes before. Forgetting an idea that crossed my brain one second before...

    Cognitive functions are a concern too now for some years...

    And I too wasn't taken seriously by my neurologist 10 years ago, I wish to find some energy to go back to doctors, but they were so helpless to me for so long...:confused:
  5. Cinders66

    Cinders66 Senior Member

    Thanks. Cognitive problems usually increase with ME severity but aren't usually tested or taken seriously, it's just written off as fatigue in the way that the problems bring active isn't just written off as fatigue but researched.
  6. BeautifulDay

    BeautifulDay Senior Member

    Thanks @Chocolove I don't have time to re-read my notes below, so please forgive incomplete thoughts.

    It ends up being complex, one of the symptoms I have is aminoaciduria (from my body not processing things correctly). For me, this means I'm starving for some things and yet my urine is full of them. Adding more will just overburden my body. So it's a matter of how to get whatever it is that I need into my body, without it accumulating it in my brain in a bad way or it going out in urine.

    For example, I can't take iron pills or anything with copper. The first few days I start to feel drunk, then I can't feel the top of my head, then I act completely intoxicated, and then I feel like I'm poisoned and dying by the 4th day. So for me, basic low dose iron/copper goes straight to toxic level. Yet, I can have a huge bowl of spinach every day, and it doesn't overburden my body and keeps my iron level up. I'm, big on listening to the body, when science has yet to explain symptoms.

    For me, eating clean (MIND diet), the spinach, blueberries, whole grain crackers/bread/cereal, and having little junk food), and being careful to try not to dive into hypoglycemia, works the best for me. It seems the less I overburden my body, the more it's able to deal with the issues that come up from Mitochondrial Disease. There are still tons of issues, but I'm definitely better when I'm clean eating. My cognitive issues aren't as bad (and I decline more slowly), my allergies are kept to a minimum, my body is no longer overreacting to chemicals/smells/etc... I still have intermittent foot drop, severe fatigue, intermittent gastroparesis, etc...... The hardest part is not eating the junk food. I'm not perfect (or even close). :bang-head:

    I ended up on the MIND diet because I liked that they took the studies on Alzheimer's/dementia and added what worked for some people into a combined program. A little of this, a little of that, ..... Then they put it to the test. Then they published the results and explained what they did so that anyone can do it.

    That's completely opposite of the MEND diet, where part of it was published, but never giving all the details. They added in things like, regular dental checkups (which is known to slow memory loss) -- which is a good thing. But then those related to conducting the study have been involved in selling use of the program to those selling outrageously expensive conferences and programs. I believe their latest is this -- https://museslabs.com/physicians/

    It's good for people who can't do the research on their own and have the money to do it.

    However, I have much more respect for the researchers doing the studies for the right reasons and not promoting them by selling supplements or programs.I believe that's a complete conflict of interest. In my mind, I take less seriously the researchers or those that paid for a study who then profit from it through alternative means.

    MIND diet associated with reduced incidence of Alzheimer's disease

    MIND diet slows cognitive decline with aging

    Last edited: Oct 7, 2017
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  7. BeautifulDay

    BeautifulDay Senior Member

    @Alvin2 I wish it had. I have the genetic mutation for Pseudocholinesterase Deficiency carrier (heterozygous for rs28933390). Usually, that just means carrier for most people. Those who are homozygous are considered to have Pseudocholinesterase Deficiency.

    According to the NIH:
    "Pseudocholinesterase deficiency can be caused by mutations in the BCHE gene. This gene provides instructions for making the pseudocholinesterase enzyme, also known as butyrylcholinesterase, which is produced by the liver and circulates in the blood. The pseudocholinesterase enzyme is involved in the breakdown of choline ester drugs. It is likely that the enzyme has other functions in the body, but these functions are not well understood. Studies suggest that the enzyme may be involved in the transmission of nerve signals."

    But couple that heterozygous mutation with my Mitochondrial Disease mutation, and things that shouldn't impact me as a heterozygote, do. My first experience was when 18 years old with boney impacted wisdom teeth, where they put me under in the hospital. I reacted by having the muscle relaxant for the throat hold for too long and couldn't breathe after the procedure and was wheezing when waking up. It sounded like I was trying to breathe through the tiniest closed airway and it made a squeak sound when trying to breathe (like a balloon when you let the air out and hold the ends tight).

    Therefore, I'm treated as being Pseudocholinesterase Deficency (not just carrier).

    In addition, everyone in our family with the MitoD gene mutation also have severe and odd reactions to meds (even those without the Psedocholinesterase Deficiency mutation). http://www.mitoaction.org/blog/medication-exposures-mitochondrial-toxicity

    Donepezil (Aricept) acts as a centrally acting reversible acetylcholinesterase inhibitor. I'm not sure what that all means, but because of these three things: 1) my pseudocholinesterase deficiency, 2) my stomach issues (constipation, intermittent gastroparesis, huge long colon), and 3) the fact that Donepezil has many side effects and has been proven to be not effective to slightly effective in some people -- my pharmacist and I decided that it just wasn't worth the slight upside for all the potential risks.

    I mentioned the Donepezil prescription as an indication of how seriously my New York neurologists now take my memory loss in my 40's. Yet, do my NY cognitive docs know anything about Mitochondrial Disease? Nope. We end up still having to be on top of the research and do what's in our best interest. It helps to have the cognitive study data on me to compare from year to year, yet, the cure is not there yet.

    I occasionally go to the Early Alzheimer's (memory loss) Group meetings when I'm having severe issues. I've only met one other young person at the meetings. Yet, when I mention issues that I might be having at the time, such as when I tell my husband to slow down his driving to 20 miles per hour in a 60 mile per hour zone because my brain can't keep up and it looks like warp speed in Star Wars -- they all understand.
    Alvin2, realturbo, Chocolove and 2 others like this.
  8. BeautifulDay

    BeautifulDay Senior Member

    @Cinders66 I agree

    Study: "Neuroinflammation in Patients with Chronic Fatigue Syndrome/Myalgic Encephalomyelitis: An 11C-(R)-PK11195 PET Study"

    "Results: The BPND values of 11C-(R)-PK11195 in the cingulate cortex, hippocampus, amygdala, thalamus, midbrain, and pons were 45%–199% higher in CFS/ME patients than in healthy controls. In CFS/ME patients, the BPND values of 11C-(R)-PK11195 in the amygdala, thalamus, and midbrain positively correlated with cognitive impairment score, the BPND values in the cingulate cortex and thalamus positively correlated with pain score, and the BPND value in the hippocampus positively correlated with depression score. Conclusion: Neuroinflammation is present in widespread brain areas in CFS/ME patients and was associated with the severity of neuropsychologic symptoms. Evaluation of neuroinflammation in CFS/ME patients may be essential for understanding the core pathophysiology and for developing objective diagnostic criteria and effective medical treatments."
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  9. Eastman

    Eastman Senior Member

  10. Cinders66

    Cinders66 Senior Member


    Yes that study was good but small. Fortunately that same team is now attempting replicating it on a much larger scale, Shame no one else replicated it in the three years gap.
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  11. panckage

    panckage Senior Member

    Vancouver, BC
    @BeautifulDay those cognitive symptoms sound like my own. I think my ME brain fog can be classed as severe most of the time... I can do by reflex, but cannot think on feet at all 90% of the time

    I do find notes are the best option when I am well but are almost completely useless on my worse days. I have posters on the wall with what i need to do. I read them but I can't process them at all. In such a state notes are almost worthless...
    Fogbuster, rosie26 and Chocolove like this.
  12. Chocolove

    Chocolove Tournament of the Phoenix - Rise Again

    I'm with you there. We'll be in a whole lot of pain if we don't listen to the body.

    That's a bowl of fresh - not cooked spinach? ...Looks like you're proving an old theory wrong:


    This form of iron is not absorbed as efficiently as heme iron, the iron found in meat and animal sources. Additionally, the oxalic acid in spinach binds with iron, which further inhibits iron absorption. You can improve the absorption of iron from spinach by eating it with foods that enhance iron absorption.
    Does the Oxalic Acid in Spinach Inhibit the Absorption of Iron ...

    But this finding shows that:
    Eur J Clin Nutr. 2008 Mar;62(3):336-41. Epub 2007 Apr 18.
    Oxalic acid does not influence nonhaem iron absorption in humans: a comparison of kale and spinach meals.
    genannt Bonsmann SS1, Walczyk T, Renggli S, Hurrell RF.
    Author information
    Institute of Food Science and Nutrition, ETH Zurich, Zurich, Switzerland. stefan.storcksdieck@ilw.agrl.ethz.ch
    To evaluate the influence of oxalic acid (OA) on nonhaem iron absorption in humans.

    Two randomized crossover stable iron isotope absorption studies.

    Zurich, Switzerland.

    Sixteen apparently healthy women (18-45 years, <60 kg body weight), recruited by poster advertizing from the staff and student populations of the ETH, University and University Hospital of Zurich, Switzerland. Thirteen subjects completed both studies.

    Iron absorption was measured based on erythrocyte incorporation of (57)Fe or (58)Fe 14 days after the administration of labelled meals. In study I, test meals consisted of two wheat bread rolls (100 g) and either 150 g spinach with a native OA content of 1.27 g (reference meal) or 150 g kale with a native OA content of 0.01 g. In study II, 150 g kale given with a potassium oxalate drink to obtain a total OA content of 1.27 g was compared to the spinach meal.

    After normalization for the spinach reference meal absorption, geometric mean iron absorption from wheat bread rolls with kale (10.7%) did not differ significantly from wheat rolls with kale plus 1.26 g OA added as potassium oxalate (11.5%, P=0.86). Spinach was significantly higher in calcium and polyphenols than kale and absorption from the spinach meal was 24% lower compared to the kale meal without added OA, but the difference did not reach statistical significance (P>0.16).

    Potassium oxalate did not influence iron absorption in humans from a kale meal and our findings strongly suggest that OA in fruits and vegetables is of minor relevance in iron nutrition.


    Sounds rather like the iron and copper overload problems in diseases discussed here....


    Wow. Thanks for sharing. You've given me a whole lot to digest. :thumbsup:
  13. Wonko

    Wonko Senior Member

    The other side.
    Q. Why is such complicated stuff in a thread aimed at those with cognitive difficulties?
  14. Chocolove

    Chocolove Tournament of the Phoenix - Rise Again

    @Wonko Well here is a mass media overview of the MIND diet...which as we know can lead to gross misinterpretation upon occasion:


    @BeautifulDay Just off the bat, the avoidance of cheese, fermented cheeses that is, could prevent consumption of any vitamin K2 in many. Vitamin K2 is an essential nutrient in sending calcium into bones rather than soft tissues like skin, arteries, heart and brain. Calcified heart disease is still the #1 killer in the U.S. evidencing a major deficiency of vitamin K2 confirmed by dietary intake studies. (Vitamin K is totally different from K2 and converts very poorly to K2.)

    The easy way around that is to consume natto instead: fermented soy beans, which contains loads of vitamin K2... if you can manage the taste and texture without aversion. It can be obtained through Amazon.
    pattismith likes this.

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