Magnetic Resonance Spectroscopy in Ataxias

Research output: Chapter in Book/Report/Conference proceedingChapter

2 Scopus citations


Ataxia refers to deficits in coordination of movement and balance. This chapter focuses on recessively and dominantly inherited as well as sporadic degenerative ataxias. These diseases are characterized by neurodegeneration in the cerebellar system, including the cerebellum and its afferent and efferent connections, and frequently also in other brain regions such as the pontine nuclei. They display great pathological diversity, as well as phenotypic variability; thus there is a great need for imaging biomarkers that reflect the underlying pathology and that can be used for diagnostic, prognostic, and treatment monitoring purposes. Despite technical challenges of magnetic resonance spectroscopy (MRS) in the cerebellum and brainstem, MRS has been shown to be sensitive to neurochemical alterations in various degenerative ataxias. Namely, early neurochemical abnormalities have been detected by MRS in ataxias prior to the structural atrophy detectable by conventional MRI and prior to symptoms. Correlations with clinical status and pathological severity were demonstrated in clinical and animal model studies, respectively. MRS was also shown to distinguish different ataxia subtypes, with potential utility in differential diagnosis, especially valuable for sporadic ataxias in the absence of genetic testing. Finally, a few studies have utilized MRS for treatment monitoring in clinical trials of recessive ataxias, and a great need exists in this area for all degenerative ataxias. More longitudinal investigations and standardization of advanced MRS methodology for multi-site trials will be critical in this respect.

Original languageEnglish (US)
Title of host publicationContemporary Clinical Neuroscience
PublisherSpringer Nature
Number of pages22
StatePublished - 2016

Publication series

NameContemporary Clinical Neuroscience
ISSN (Print)2627-535X
ISSN (Electronic)2627-5341

Bibliographical note

Publisher Copyright:
© 2016, Springer International Publishing Switzerland.


  • Ataxia with oculomotor apraxia
  • Ataxia-telangiectasia
  • Autosomal recessive spastic ataxia of Charlevoix-Saguenay
  • Friedreich’s ataxia
  • Multiple system atrophy
  • Spinocerebellar ataxia


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