MRS findings in a patient with juvenile-onset Alexander's leukodystrophy.
J La State Med Soc
; 165(1): 14-7, 2013.
Article
en En
| MEDLINE
| ID: mdl-23550391
ABSTRACT
Alexander's leukodystrophy is a rare cerebral white matter disorder with an onset that can be infantile, juvenile, or occur in the adult years. It is thought to be demyelinative, but the pathogenesis is ill-defined. We report a 24-year-old woman with juvenile-onset Alexander disease, of 12 years duration, who underwent magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) serially as part of her care. The patient's latest MRI showed periventricular-increased signal intensity on T2 and fluid attenuation and inversion recovery sequences, which appeared stable when compared to her first study seven years ago. MRS revealed an elevated choline/creatine ratio with relative suppression of the n-acetyl aspartate peak, also similar to her previous MRS findings. MRS also showed elevation of myoinositol levels, best demonstrated with the short echo-time spectra. These findings support the primarily demyelinative characteristics of this leukodystrophy and may provide a surrogate marker of disease progression, as well as a potential response to therapeutic intervention when this becomes available.
Buscar en Google
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedad de Alexander
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
J La State Med Soc
Año:
2013
Tipo del documento:
Article
País de afiliación:
Estados Unidos
Pais de publicación:
EEUU
/
ESTADOS UNIDOS
/
ESTADOS UNIDOS DA AMERICA
/
EUA
/
UNITED STATES
/
UNITED STATES OF AMERICA
/
US
/
USA