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Arch Neurol. 2006 Oct;63(10):1479-82.

Betamethasone and improvement of neurological symptoms in ataxia-telangiectasia.

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1
Department of Pediatrics, University of Siena, Siena, Italy.

Abstract

BACKGROUND:

To our knowledge, there have been no reports on the control of central nervous system symptoms in patients with ataxia-telangiectasia.

OBJECTIVE:

To preliminarily determine the effectiveness of corticosteroid therapy on the central nervous system symptoms of a child with ataxia-telangiectasia in whom neurological signs improved when, occasionally, he was given betamethasone to treat asthmatic bronchitis attacks.

DESIGN:

Case report.

SETTING:

Tertiary care hospital. Patient A 3-year-old boy with the classic hallmarks and a proved molecular diagnosis of ataxia-telangiectasia.

INTERVENTIONS:

We used betamethasone, 0.1 mg/kg per 24 hours, divided every 12 hours, for 4 weeks to preliminarily determine its effectiveness on the child's central nervous system symptoms and its safety. Methylprednisolone, 2 mg/kg per 24 hours, divided every 12 hours, was then given in an attempt to perform a long-term treatment.

RESULTS:

There were improvements in the child's neurological symptoms 2 or 3 days after the beginning of the drug treatment. After 2 weeks of treatment, the improvement was dramatic: the disturbance of stance and gait was clearly reduced, and the control of the head and neck had increased, as had control of skilled movements. At 4 weeks of treatment, adverse effects mainly included increased appetite and body weight and moon face. No beneficial effect was obtained when, after 4 weeks, betamethasone was replaced with methylprednisolone. Six months later, without therapy, the child continued to experience severe signs of central nervous system impairment.

CONCLUSION:

Controlled studies to better understand the most appropriate drug and therapeutic schedule are required.

Comment in

PMID:
17030666
DOI:
10.1001/archneur.63.10.1479
[Indexed for MEDLINE]
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