We went for our regular appointment at the Movement Disorder Specialist at Medical University of South Carolina, and she changed John's diagnosis to a different kind of Atypical Parkinson's--Multiple Systems Atrophy (instead of Lewy Body Dementia). Her main argument was that he has not had a steep cognitive decline. I have thought he did have fluctuating cognition, one of the two essential symptoms of Lewy Body Dementia, because sometimes he can have a sophisticated conversation (about politics) and sometimes he is confused. But that is hard to define.
I'm shaken up by the new diagnosis for two reasons. One is that it makes me feel alone--I no longer fit in several of the communities that have been helpful to me. MSA is a much rarer disease. The other is what would we have done differently if we had had the correct diagnosis earlier? I don't think John would have had two surgeries for benign prostate enlargement, which gave little benefit. I took over more things because I expected more rapid progression to dementia, though I think my doing so has made his life more pleasant.
The new diagnosis does make sense. The key symptoms are rigidity, slowness, and problems with the autonomic nervous system such as postural hypotension, urinary retention, constipation, and both kinds of incontinence. Consider a list of red flags for MSA:
- Early instability--John has only started falling several times a month recently, but he has had balance problems from early on
- Rapid progression--not so much so, but if defined as in a wheelchair in 10 years (the Parkinson's disease definition) that is likely to fit
- Abnormal postures--he has something called disproportionate antecollis where his head is way forward
- Bulbar dysfunction--various voice issues, which he doesn't have except a little hoarseness
- Respiratory dysfunction--snoring and another noise breathing in while sleeping, but these were issues for many years. He often falls into a shallow rapid breathing pattern, though he reports no shortness of breath
- REM Sleep Behavior Disorder--this was an early symptom
- Emotional incontinence--not an issue
The relationship between the diseases is odd--most of these symptoms are seen in both diseases, the difference is which are predominant. Yet they show different kinds cell damage on autopsy. One article states: "Although the molecular mechanisms of misfolding, aggregation, and fibrillation of α-synuclein might partly overlap among the α-synucleinopathies, disease-specific cascades that are determined by genetic and environmental factors are likely to discriminate these." That is, Lewy Body and MSA may start with the same process that is damaging the brain, but the brain can fight that damage in several different ways, which define the different diseases.
Both diseases have treatment only for symptoms, no way to modify the course of the disease. Because of the change in diagnoses the doctor did increase John's Sinemet dose, in hopes of reducing his stiffness and balance issues. There doesn't seem to be anything more we can do about the constipation and incontinence issues. Life expectacy is similar for the two diseases, perhaps with fewer people with MSA living more than 10 years from diagnosis. With MSA, the body is likely to go before the brain goes completely, which is a good thing.
(The image is a road sign from Alaska sent to me by a friend)