Life Accelerated

There is a physical, neurological component to ALS that imitates an emotional problem called the Pseudobulbar Affect or PBA. Like so many other things with this infernal illness, PBA mimics and expands on a normal life process, that of emotional expression and depression. It clouds itself within the very real feelings of sadness which are inherent in dealing with this illness. PBA is yet another part of the great challenge of this disease.

PBA seems to be related to degeneration of motor neuron pathways from the upper brain to the lower, or bulbar, part of the brain. As with everything in this illness, the actual cause and mechanism is, as yet, unknown. We know so little about the human brain and how it works. With PBA, the neurological pathways which normally manage emotional expression, keeping emotions appropriate, become “disconnected” as ALS progresses, creating out-of-context emotional outbursts. The term pseudo refers to the fact that the problem isn’t in the bulbar neurons themselves, but in their loss of connection to neurons elsewhere in the brain. So things that would normally not bother you make you cry, or laugh, all out of proportion to the cause. PBA can start early in some ALS cases, late in others, and in slightly over half the cases it doesn't happen at all. Nobody knows why.

On top of this gift of tears and laughter, this part of ALS can also occur with something called Frontotemporal Lobar Dementia. This is not a dementia in the classic sense; you don't lose your mind. Instead FTD can cause an ALS patient to become more easily confused and agitated, can cause issues with decision making, and can be linked with aphasia, the ability to find words in your intellectual lexicon and to express those words verbally.

Once again the problem is symptom imitation. The symptoms of FTD can easily be confused with the simple effects of aging. ALS is most common in people over 55, a time when most people begin to exhibit the typical symptoms of aging, including things like aphasia and agitation. Once again ALS imitates life, only moreso. And as with so many things in this disease, neither FTD nor PBA have viable treatments. The best so far is a drug to control emotional outbursts, a drug that treats the symptoms, not the disease.

So if I seem more easily agitated, more easily confused, prone to tears for what you would think is something silly and pointless, it's not me, it's ALS. No, it's not simply getting older. No, it's not emotions driven by my diagnosis. No, it's not the stress of having a terminal illness. Sure, those issues may be part of the problem. They are a part of life, only in ALS they are life accelerated.