Treatment of Ataxia-Telangiectasia

  • Susan L. Perlman
Part of the NATO ASI Series book series (volume 77)


The greatest areas of concern are pulmonary disease and malignancy. Recurrent sinopulmonary infections must be recognized and treated aggressively to lower the risk of developing chronic bronchiectasis. When chronic bronchiectatic changes are present, care must be taken with pulmonary toilet to remove secretions and protect the oxygenating capability of the lungs. Periodic pulmonary function studies can help monitor this, and assistance from respiratory therapists and pulmonary physicians is invaluable. Various immunotherapies have been tried to build up the patient’s resistance to infection. The most reliable remains the use of intravenous gamma globulin (0.6 – 0.8 ml perkg) IV q 3–4 weeks (when immunoglobulin levels are less than 200 mg per 100 ml). Increasing bulbar dysfunction in the later stages of disease predisposes to aspiration pneumonia. This may be helped by anticholinergic drugs to dry up secretions or by fluoxitine 10–20 mg q AM.


Valproic Acid Essential Tremor Episodic Ataxia Congenital Nystagmus Intravenous Gamma Globulin 
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Copyright information

© Springer-Verlag Berlin Heidelberg 1993

Authors and Affiliations

  • Susan L. Perlman
    • 1
  1. 1.Department of NeurologyUniversity of California at Los AngelesLos AngelesUSA

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