title: Steroid treatment in Ataxia-Telangiectasia induces alterations of functional magnetic resonance imaging during prono-supination task.
abstract: BACKGROUND
  Ataxia-Teleangiectasia (A-T) is a rare neurodegenerative disorder characterized by progressive cerebellar degeneration. Till few years ago only supportive care was available to improve the neurological function in A-T patients. Even though A-T remains an incurable disease, we recently demonstrated a drug dependent amelioration of neurological signs in A-T patients during a short-term treatment with oral betamethasone.
  
  
  OBJECTIVE
  The aim of this study is to evaluate whether the steroid induced motor performance changes in A-T are associated with functional magnetic resonance imaging (fMRI) modifications. This represents a preliminary pilot study, which requires a validation on a larger cohort of patients.
  
  
  METHODS
  Six A-T patients received a 10-days cycle of oral betamethasone at 0.03 mg/kg/day. fMRI studies were carried out at T0 and at the end of the cycle. The neurological evaluation was performed through the Scale for the Assessment and Rating of Ataxia (SARA) quantification. The fMRI protocol was a block design with alternating epochs of rest and prono-supination of the dominant (right) hand.
  
  
  RESULTS
  The voxel-based comparison showed a remarkable increase in the number of activated voxels within the motor cortex under the on-therapy condition as compared with the cortical activity under baseline condition in the 2 patients who completed the study protocol.
  
  
  CONCLUSIONS
  Changes in motor performance in A-T patients treated with betamethasone are coupled with an increase in the activation in relevant cortical areas, thus suggesting that in A-T patients steroid treatment could improve motor performance facilitating cortical compensatory mechanisms.
authors: Biostructure and Bioimaging Institute, National Research Council, Naples, Italy.
authors: Mario
authors: M
authors: Quarantelli
authors: Giuliana
authors: G
authors: Giardino
authors: Anna
authors: A
authors: Prinster
authors: Giuseppina
authors: G
authors: Aloj
authors: Barbara
authors: B
authors: Carotenuto
authors: Emilia
authors: E
authors: Cirillo
authors: Angela
authors: A
authors: Marsili
authors: Elena
authors: E
authors: Salvatore
authors: Ennio
authors: E
authors: Del Giudice
authors: Claudio
authors: C
authors: Pignata
mesh_terms: D000293:Adolescent; D001260:Ataxia Telangiectasia / Q000188:drug therapy*; D001623:Betamethasone / Q000627:therapeutic use*; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009044:Motor Cortex / Q000187:drug effects*; D010865:Pilot Projects; D011187:Posture; D011597:Psychomotor Performance / Q000187:drug effects
chemical_list: D005938:Glucocorticoids; D001623:Betamethasone