Proton Magnetic Resonance Spectroscopy (¹H MRS) in Patients with Sporadic Cerebellar Degeneration

Abstract

The authors studied 23 patients with cerebellar degeneration including multiple systemic atrophy (MSA) and cerebellar cortical atrophy (CCA) by proton magnetic resonance spectroscopy (1H-MRS). 1H-MRS allowed noninvasive measurement of the signal intensities derived from N-acetylaspartate (NAA), creatine + phosphocreatine (CRE), and choline-containing compounds (CHO). There was significant reduction of the NAA/CRE level in the frontal cortex, putamen, cerebellar hemisphere and cerebellar vermis of patients with MSA, and in the frontal cortex, cerebellar hemisphere and cerebellar vermis of patients with CCA as compared with those of normal controls. There was significant reduction of the NAA/CRE level also in the putamen of patients with MSA as compared with that of patients with CCA. These results indicated the presence of a degenerative process and/or functional impairment in the frontal cortex and putamen of patients with MSA and in the frontal cortex of patients with CCA, in addition to a degenerative process in the cerebellum. There was a significant correlation between the NAA/CRE level and the severity of clinical signs. 1H-MRS is valuable in providing information regarding the pathophysiology and the progress of cerebellar degenerative diseases.

Differentiation of SCA2 from MSA‐C using proton magnetic resonance spectroscopic imaging

“…However, the location of their voxel in the dentate and peridentate region may not be representative of NAA/Cr loss, since the dentate nucleus is spared in SCA2. In accordance with a previous study that reported a significant correlation between the NAA/Cr level and the severity of clinical signs (26), the association between findings from MR volumetry/spectroscopy and cerebellar disability scores detected in the present study argues in favor of NAA as a valid noninvasive in vivo biomarker for neuronal dysfunction and/or atrophy in SCA2 and MSA‐C.…”

Distinct Neurochemical Profiles of Spinocerebellar Ataxias 1, 2, 6, and Cerebellar Multiple System Atrophy

“…Low cerebellar and pontine NAA levels and NAA/Cr ratios in SCA2 and MSA-C relative to controls were reported previously [9, 11, 28] and indicate neuronal dysfunction/loss in these brain regions [29]. In addition to this well-established biomarker, we detected differences in myo -inositol, tCr (both putative markers of gliosis [30, 31]), glutamate, glutamine (potentially reflecting disruptions in glutamatergic neurotransmission [21]), and glutathione and ascorbate (potentially indicating oxidative stress) in patients with SCA2 and MSA-C relative to controls (Fig.…”

Brainstem metabolites in multiple system atrophy of cerebellar type: 3.0‐T magnetic resonance spectroscopy study

“…NAA can be more easily accessible than MI because the methyl group of NAA can be obtained by the 1 H‐MRS both using long TE and short TE. In addition, several authors have reported the usefulness of NAA/Cr and NAA for the evaluation of MSA pathology 5–8. However, the previous study about AD and MCI19 and our data supported the superior utility of MI/Cr ratio as a diagnostic tool in evaluating the severity of neurodegenerative disorders compared to NAA and NAA/Cr.…”

“…Both compounds can be observed by 1 H‐MRS, and hence may be considered biomarkers of neuronal integrity and gliosis. Although both NAA and MI are important from the pathological point of view in MSA,3, 4 there was limited information about the role of MI in MSA 5–8…”