Question Title: Conflicting diagnoses; what to do?

Forum: Neurology Forum
Topic: Shy Drager

Her symptoms: She failed the tilt table test (on the third time she was lifted up with the addition of that drug that similates adrenalin). During the tilt table test we observed a very erratic heart beat even as she was horizontal. She has difficulty keeping her balance (has resorted to a walker) and has spells/episodes that seem stroke-like or like she is going to faint--she doesn't faint and these episodes seem independent of posture. She seems to have greater difficulty expressing herself--finding the words and her voice volume is reduced. She does have double vision. She was in great health a liitle more than a year ago, and now has discontinued driving and reduced her activity quite dramatically. She appears quite normal, except that she is somewhat fragile and is unsteady on her feet. No Parkinsonion symptoms--shaking, etc. Her handwriting has become increasingly difficult to read, she has lessened use of her right side (not obvious, but when she sits in the car, she lifts her right leg with her hands). A head CT revealed some atrophy and she is mildly forgetful--(when we were at the cemetary, claimed that her parents weren't buried there, but in another town), but recognizes everyone.

The neurologist, on the other hand, diagnosed her as having neurocardiagenic syncope (sp). But to eliminate the possibility of stroke, he prescribed a blood-thinner (Kumodon), but her "spells" did not decrease, so that was discontinued.

So now we are at an impasse, and I'm not certain where to proceed. Should we get a second opinion from a neurologist? Why isn't a pacemaker warranted? Is this what early MSA looks like?

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This isn't so easy to figure out. I do have several comments, though.

First, MSA (multiple system atrophy) is in fact a more modern term encompassing what was once thought to be three diseases: Shy Drager Syndrome (SDS), striato-nigral degeneration (SND), and sporadic olivo-ponto-cerebellar atrophy (OPCA). Note, however, that hereditary forms of OPCA have a different new nomenclature. The unifying feature of all the "diseases" that are now MSA is the presence of certain microscopic features at autopsy (or perhaps biopsy, though that would be rarely done).

The diagnosis of MSA, as with Parkinson's disease itself and the other Parkinson look-alikes (including progressive supranuclear palsy PSP and diffuse Lewy Body disease DLBD) is clinical. That is, the experienced doctor (usually it takes a movement disorders specialist) asks the right questions and performs a comprehensive neurologic examination. Sometimes tests like MRI may help, but rarely. Often, the areas of the brain which are affected look perfectly normal on MRI, so you can't rely on it for diagnosis.

It is interesting that a cardiologist diagnosed MSA, especially the cerebellar type (formerly OPCA). It is possible that you have a cardiologist especially experienced in autonomic nervous system disorders, so he/she may have seen a few before. Ordinarily, a neurologist would diagnose it. On the other hand, I personally would hesitate before diagnosing neurocardiogenic syncope without having a cardiologist see the patient. In other words, it seems that the cardiologist rendered the neurologist's diagnosis and vice versa.

MSA is a disorder of the 50s and 60s. It is possible, though rare, to diagnose it in the 40s or the 70s. Unlike Parkinson's disease, which can be diagnosed for the first time anywhere from the 20s to the 90s, I would be very hesitant to apply the diagnosis of MSA (any form) to a patient in his/her 80s, unless it was clear that the disorder had already been present for 10 or more years. That does not apply in your case.

My suspicion is that this is an entirely different diagnosis, possibly DLBD, because of the cognitive problems (rare in MSA) and the presence of autonomic dysfunction (prominent in MSA, but that's not the only cause). The double vision could conceivably represent MSA, but fits DLBD better.

The blood thinner Coumadin (warfarin) is reasonable if there is documented vascular disease under certain circumstances. I am not convinced that that is so, from the story. Coumadin is a good drug, if you need it, but it isn't something I'd want if it's not warranged.

I have presented the above at the risk of being out on a limb. It is not possible to provide a diagnosis without in-person interview and examination as well as review of the other available data. I hope you are able to take this information back to her doctor(s) and ask for his/her opinion about the possibility of DLBD.

Most general neurologists are not movement disorders specialists, so if you get a blank stare or an unsatisfying answer, seek a second opinion from such a specialist. If you are close to Cleveland, we'd be happy to see you here in the movement disorders clinic - call 800 223-2273 and ask for neurology appointments at 4-5559. Otherwise, there are a few centers of excellence around the country, usually attached to the better academic medical centers/schools.

Sorry about the longwinded response. I hope this helps. CCF MD mdf.

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