Mast Cell Disease & Neurodegeneration: You Can (Literally) Lose Your Mind

The Disease / Thursday, September 7th, 2017 / no comments

One of the biggest challenges we face as patients is the feeling that we are crazy. Perhaps we tell ourselves that or maybe it’s coming from those around us who don’t understand our disease (or our paranoia makes us think they think we’re crazy!). Sometimes even ill-informed doctors can convince a mast cell patient that it’s “all in your head”.

And it is! But probably not the way they think it is.

I’ve been researching neuroinflammation/degeneration for awhile now because I honestly feel like I am losing my own mind — both figuratively and literally — as my disease progresses. I have a lot of brain inflammation and am seeing a neurologist (my second one) to consult with them about my fainting, seizures and also the cognitive and emotional effects brought on by the recurring inflammation.

A few days ago I found a great research paper on the subject. Here are some highlights:

Mast cells are present in variable quantities in all tissues and organs, especially along vessels, including the brain, which is rich in mast cells. In this organ, they are preferentially located near blood vessels at the level of the blood-brain barrier and also at the nerve endings of sensory and sympathetic fibers.
In addition, mast cells are found particularly and in high density in certain structures of the diencephalon, especially in the hypothalamus, which is involved in systems of stress response, emotion, and cognition, and also in amygdales, in the ventral portion of the median eminence, near the pituitary gland at these anterior and posterior sides as well as in the hippocampal formation and surrounding leptomeninges and meningeal spaces at the level of the olfactory bulb. A large number of mast cells reside in the thalamus, which lesions or stimulation of the dorsomedial portion and earlier kernels have been associated with changes in emotional reactivity and pain.


…several studies have found an association between mast cell burden and susceptibility to experimental autoimmune encephalitis.


That last highlighted bit — autoimmune encephalitis — was still fresh in (what is left of) my memory when I saw this piece on CNN today, entitled “Doctors thought she was psychotic, but her body was attacking her brain“.

That sounds a bit like me, to be honest! According to the article:

There are over 15 known types of autoimmune encephalitis, according to the Autoimmune Encephalitis Alliance. Meaning the immune system can attack other brain receptors, too.


I wonder if that is what’s happening with me so I’ll be bringing this up with my immunologist and neurologist. I also found more detailed information to share with him from another research paper.

Here are some highlights from that one:

Mast cells are immune cells of hematopoietic origin that regulate inflammation and upon activation release many proinflammatory mediators in systemic and central nervous system (CNS) inflammatory conditions. In addition, inflammatory mediators released from activated glial cells induce neurodegeneration in the brain. Systemic inflammation-derived proinflammatory cytokines/chemokines and other factors cause a breach in the blood brain-barrier (BBB) thereby allowing for the entry of immune/inflammatory cells including mast cell progenitors, mast cells and proinflammatory cytokines and chemokines into the brain.
Glial cells, mast cells and T-cells can reactivate each other in neuroinflammatory conditions in the brain and augment neuroinflammation. Further, inflammatory mediators from the brain can also enter into the peripheral system through defective BBB, recruit immune cells into the brain, and exacerbate neuroinflammation. We suggest that mast cell-associated inflammatory mediators from systemic inflammation and brain could augment neuroinflammation and neurodegeneration in the brain.


So again, it’s very clear that neuroinflammation can result in neurodegeneration and the scary part is, neuroinflammation is at the heart of some pretty scary diseases.

Diseases such as:

Neuroinflammation is the leading cause of devastating neurodegenerative diseases such as Parkinson’s disease (PD), Alzheimer’s disease (AD), Amyotrophic lateral sclerosis (ALS) and Multiple sclerosis (MS)/Experimental autoimmune encephalomyelitis.


You can read exactly what EAE (experimental autoimmune encephalomyelitis) is here. Also, keep in mind that encephalitis is inflammation of the brain where encephalomyelitis is inflammation of the brain and spinal cord.

The form of encephallitis noted in the CNN story I cited above is described as:

Anti-NMDA receptor encephalitis, also known as NMDA receptor antibody encephalitis, is an acute form of brain inflammation that is potentially lethal but has a high probability for recovery with treatment. It is caused by an immune system attack, primarily targeting the NR1 subunit of the NMDA receptor (N-methyl D-aspartate receptor).

So it definitely sounds like something I need to investigate based on my neuro symptoms.

Here are a few more facts about the role mast cells play in neuroinflammation/neurodegeneration:

  • mast cells are very powerful cells and even a few mast cells can release sufficient quantity of inflammatory mediators that can affect BBB
  • increased number of mast cell involvement and their activation is deleterious, since this could increase BBB permeability, activate astrocytes, oligodendrocytes, microglia and T cells in neuroinflammation
  • mast cells can recruit and activate other inflammatory cells and glial cells in the brain at the site of inflammation and induce vasodilation in neuroinflammation
  • mast cells are the major link between neurons and neuroinflammation

The research also unfortunately shows that this neuroinflammation/degeneration can be difficult to treat:

Previous studies have shown that non-steroidal anti-inflammatory drugs (NSAIDs), statins and other drugs provide some relief although they are ineffective for neurodegenerative diseases. This is because the NSAIDs do not efficiently cross the blood brain-barrier (BBB) and do not attain therapeutic levels that are necessary to overcome and reverse neuroinflammation. Therefore, therapeutic options like nasal NSAIDs have been suggested to improve its bioavailability and effectiveness in neuroinflammation by increasing the concentration in the brain.


I will definitely be following up on this topic once I consult with my doctors. In the meantime all I (we) can do is try our best to avoid triggers and find medicines that keep our mast cells as calm as possible.

Yeah, I know. 🙂

Remember, we are (literally!) in this mast cell craziness together!

Mother. Wife. Patient. Keeper of Huskies. MCAS blogger & advocate. Living life in the mast lane with the Grand Tetons & Yellowstone as my backyard. You can also find me blogging at Life In The Mast Lane and The Empty Nest Housewife.

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