Treatment of Ataxia-Telangiectasia
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.
KeywordsValproic Acid Essential Tremor Episodic Ataxia Congenital Nystagmus Intravenous Gamma Globulin
Unable to display preview. Download preview PDF.
- Baloh, RW (1984) The Essentials of Neurotology. FA Davis Company, PhiladelphiaGoogle Scholar
- Baloh, RW; Winder, A (1991) Acetazolamide — Responsive Vestobulocerebellar Syndrome, clinical and oculographic features. Neur 41:429–33Google Scholar
- Perlman, SL; Kern, R (1986) Intensive Physical Therapy for Chronic Progressive Neuromuscular Disease. Muscle and Nerve 9:257 (#55)Google Scholar
- Peterson, PL; Saad, J; Nigro, MA (1988) The Treatment of Friedreich’s Ataxia with Amantidine Hydrochloride. Neur 38:1478–80Google Scholar
- Swift, M; Morrell, D; Massey, RB; Chase, CL (1991) Incidence of Cancer in 161 Families Affected by Ataxia-Telangiectasia, N Engl J Med 325:183136Google Scholar
- Yee, RD; Baloh, RW; Honrubia, V (1982) Effect of Baclofen on Congenital Nystagmus. Functional Basis of Ocular Motility Disorders Pergamon PressGoogle Scholar