Another question regarding anesthesia & dystonia

[Erin__0__0]

  : : Thanks so much for your answer - we are seeing Dr. Friedman for a
      follow-up next month.
  : : I have a more complicated question regarding this. Can general anesthesia
  : : cause dystonia (not temporary) which cannot be corrected with benedryl but
  : : responds to Sinemet? Or is generalized dystonia, which is dopa-responsive,
  : : definitely a hereditary form of dystonia? I am asking these questions to
  : : help us decide whether to pursue genetic testing for dystonia. Our daughter's
  : : severe onset of Dystonia began shortly after a tonsilectomy and this same
  : : surgery may need to be done for another daughter. Needless to say, we are hesitant about
  : : considering surgery for her. I understand how difficult dystonia is to
  : : diagnose and treat, let alone find the cause, but any input would be
  : : appreciated. There is a strong history of spinocerebellar degeneration
  : : in our family, but we have been told that the two conditions are not
  : : related.
  : : You provide a wonderful service here and have been more help than you
  : : know over the past two years that we have been using the forum. If it
  : : wasn't for this forum, our daughter may have never been diagnosed correctly.
  : : It was one of the forum doc's encouragement to have our daughter seen at
  : : CCF that finally got us a correct diagnoses last Spring.
  : =
  : You may be aware of cases of oculogyric crises precipitated by anesthesia in people who have idiopathic torsion dystonia. The dystonic syndrome is already present (genetic) and the episodes are triggered by anesthesia and resolve with treatment (anticholinergic medications).
  : There are many other syndromes of dystonia. Dystonia is not a single disease, but rather an expression of impaired posture control in the brain (structures within the basal ganglia are involved). A variety of processes can impair basal ganglia function, including medications, toxins, trauma, oxygen deprivation, genetic abnormalities, etc. Of course, the same insults can cause other problems as well, not just dystonia. That's part of the complexity of neurology.
  : Dopa responsive dystonia is a syndrome different than the idiopathic torsion dystonia. There are recognized genetic biochemical abnormalities which produce this, and in fact there is not just a single form of dopa-responsive dystonia.
  : Dystonia can appear suddenly (or with some delay) after "medical" events, such as trauma or surgery. The cause and effect relationship is not clear. I am not aware of any persistent dystonia definitely caused by anesthesia. It may be true that your daughter had the predisposition to develop dystonia, and the surgical procedure (or anesthesia) was just enough to tip the balance and bring out symptoms then, rather than later. If your daughter's dystonia is dopa responsive, then anesthesia is not a cause per se, but may have been the "last straw."
  : In this forum, it is impossible to advise you whether to proceed with surgery, but in general I'd say that if something is medically necessary, you have to weigh the risks and benefits, even if all options are less than perfect.
  : The family history of spinocerebellar degeneration is puzzling but may provide a clue. If you have not seen a movement disorder specialist, I would arrange consultation with one (that service is available at CCF). The exact nature of the family disease(s) and your child's problems should at least be defined before any link could be established. There may be some clues and a unifying diagnosis could pop up, allowing you to be better prepared for what to expect and possibly suggesting some treatment.
  : Interesting question. I hope this helps. CCF MD mdf.
Dear Erin:
I am another doctor answering your request.  I agree with everything thus far stated.  The answer to your question is yes.  It would be a rare occurrance but it can happen.  If there is a metabolic disorder such as a mitochondrial disease, this might happen.  I hope that you get your questions answered by Dr Freidman, he is a very good doctor.
Sincerely,
CCF Neuro[P] MD, RPS