Document Detail


Steroid-induced improvement of neurological signs in ataxia-telangiectasia patients.
MedLine Citation:
PMID:  18290844     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A recent clinical observation reported on a dramatic improvement of neurological symptoms following short-term betamethasone administration in a child affected with ataxia-teleangiectasia (A-T). The aim of this study was to extend this observation to additional A-T patients followed at a single Immunodeficiency Center. Six consecutive patients (three males; mean age 16.3 years, range 5-30 years) were enrolled into this monocentric before-after trial. A cycle of oral betamethasone at the dosage of 0.1 mg/kg/day was administered for 10 days. The neurological evaluation was performed through the Scale for the Assessment and Rating of Ataxia. Overall, five of the six patients exhibited a clear amelioration of the neurological performances. Only in two patients, a slight amelioration persisted 7 days after the therapy withdrawal, whilst in the other patients the score reached approximately the pre-treatment value at the end of the therapy. Twenty-eight of the 46 evaluated neurological items (60%) improved during therapy. The speech disturbance, finger chase and nose-finger test showed the more significant improvement. The clinical amelioration was inversely correlated with the level of cerebellum atrophy, as revealed by the magnetic resonance. Our data indicate that neurological signs in A-T are susceptible of beneficial pharmacological intervention even years after the disease onset.
Authors:
T Broccoletti; E Del Giudice; S Amorosi; I Russo; M Di Bonito; F Imperati; A Romano; C Pignata
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Publication Detail:
Type:  Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  European journal of neurology : the official journal of the European Federation of Neurological Societies     Volume:  15     ISSN:  1468-1331     ISO Abbreviation:  Eur. J. Neurol.     Publication Date:  2008 Mar 
Date Detail:
Created Date:  2008-02-22     Completed Date:  2008-04-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9506311     Medline TA:  Eur J Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  223-8     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Federico II University, Naples, Italy.
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MeSH Terms
Descriptor/Qualifier:
Administration, Oral
Adolescent
Adult
Ataxia Telangiectasia / drug therapy*,  pathology,  physiopathology*
Betamethasone / administration & dosage*
Brain / pathology
Child
Child, Preschool
Female
Glucocorticoids / administration & dosage*
Humans
Lymphocytes / drug effects
Magnetic Resonance Imaging / methods
Male
Monocytes / drug effects
Time Factors
Chemical
Reg. No./Substance:
0/Glucocorticoids; 378-44-9/Betamethasone

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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