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Shy Drager vs. Pure Autonomic Failure
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Shy Drager vs. Pure Autonomic Failure


  My father has never officially been diagnosed with Shy Drager, but definitely has autonomic failure symptoms.  He has orthostatic hypotension (controlled now by Florinef and Promatine), dizziness (rarely), vision difficulties, neck and upper back discomfort, fainting (if BP drops too quickly), impotence, decreased sweat production, nasal stuffiness, constipation, bladder dysfunction, and anemia.  He does not, as yet, exhibit gait disturbances, tremors, no difficulty with speech, writing, or walking (except if BP is too low).  My mother feels he also has sleep apnea as he snores loudly then appears to stop breathing for a few minutes and then loudly inhales as if catching his breath.
  Based on these symptoms, do you feel he more likely has Pure Autonomic Failure or Shy Drager?  I realize you'd only be making an educated guess as you can't actually see and test him in "real life", but he refuses to go to another doctor (He's been to MANY and had many tests), and I know the prognosis for each disorder is different.
  Thanks in advance for any response.
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Your father certainly has what sounds like widespread autonomic dysfunction. Making the diagnosis of MSA vs PAF is (as you guessed) impossible in this format.
Part of the necessary information is the time course. Sometimes the autonomic failure may precede the development of parkinsonism or cerebellar symptoms. However, if the autonomic failure has been present for 10 years, then it's unlikely to be MSA.
Incidentally, MSA (multiple system atrophy) is the newer term encompassing what was once thought to be three diseases: Shy Drager Syndrome, striatonigral degeneration, and (non-familial) olivopontocerebellar atrophy (OPCA). There are specific features under the microscope which unify all these as one disease with different patterns of expression.
An experienced neurologist (preferably a movement disorders specialist) should see your dad and examine him thoroughly for signs of parkinsonism or cerebellar dysfunction. This would help you with a diagnosis.
In the mean time, until your dad gets fed up enough to actually consent to see another doctor, follow the advice given for management of autonomic symptoms.
Gait disturbance and falling would be an indicator that this is not just pure autonomic failure.
I hope this helps. Of course, this forum doesn't substitute for medical advice provided by your doctor after appropriate evaluation. If you are interested in evaluation at CCF, call 800 223-2273 and ask for neurology appointments at 4-5559. Tell them you want movement disorders specialist.
CCF MD mdf.





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