phosphocreatine and Multiple-System-Atrophy

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.

Topics: Adult; Aged; Aspartic Acid; Atrophy; Cerebellar Diseases; Cerebellum; Choline; Creatine; Disease Progression; Female; Frontal Lobe; Humans; Hydrogen; Magnetic Resonance Spectroscopy; Male; Middle Aged; Multiple System Atrophy; Neurodegenerative Diseases; Phosphocreatine; Protons; Putamen

We performed in vivo phosphorus magnetic resonance spectroscopy on the occipital lobes of 15 patients with multiple system atrophy (MSA; eight with olivopontocerebellar atrophy [OPCA] and seven with the striatonigral degeneration variant [SND]), 13 patients with idiopathic Parkinson's disease (PD), and 16 age-matched healthy subjects. The MSA group showed significantly reduced phosphocreatine (PCr), increased inorganic phosphate (Pi), and unchanged cytosolic free [Mg2+], and pH. We did not find any significant difference between the OPCA and SND variants. However, patients with PD showed significantly increased content of Pi, decreased cytosolic free [Mg2+], and unchanged [PCr] and pH. Comparing the MSA and PD groups, [PCr] was significantly lower in MSA than in PD, whereas cytosolic free [Mg2+] was significantly lower in PD. Despite a certain degree of overlap of [PCr] and [Mg2+] values between the two groups, by considering both variables at the same time it was possible to classify correctly 93% of cases by discriminant analysis. We conclude that phosphorus magnetic resonance spectroscopy discloses abnormal phosphate metabolite and ion contents in both MSA and PD, respectively, and may provide noninvasive diagnostic help to differentiate MSA from PD.

Topics: Adult; Aged; Aged, 80 and over; Brain; Cytosol; Female; Humans; Hydrogen-Ion Concentration; Magnesium; Magnetic Resonance Spectroscopy; Male; Middle Aged; Multiple System Atrophy; Occipital Lobe; Parkinson Disease; Phosphates; Phosphocreatine; Phosphorus; Severity of Illness Index

Proton magnetic resonance spectroscopy ((1)H-MRS) was performed in patients with a clinical diagnosis of idiopathic Parkinson's disease (IPD), multiple system atrophy (MSA) or progressive supranuclear palsy (PSP) in order to assess metabolic differences between the three groups of patients. Single-volume (1)H-MRS, localized to the lentiform nucleus, was carried out in 19 IPD patients, 14 MSA patients, 11 PSP patients and 12 age-matched healthy subjects. The signals of N-acetylaspartate (NAA), choline-containing compounds (Cho) and creatine-phosphocreatine (Cr) were evaluated as peak area ratios. The NAA/Cho peak ratio was significantly reduced in MSA and in PSP patients compared to IPD patients and to controls. The NAA/Cr peak ratio was significantly reduced in MSA, in PSP and in IPD patients compared to controls, but only in MSA compared to IPD patients. The NAA reduction in the basal ganglia of MSA and PSP patients may reflect a neuronal loss or damage. Single-volume (1)H-MRS may be a useful tool in differentiating MSA and PSP from IPD patients.

Topics: Adult; Aged; Aspartic Acid; Brain; Choline; Creatine; Diagnosis, Differential; Disease Progression; Female; Humans; Magnetic Resonance Spectroscopy; Male; Middle Aged; Multiple System Atrophy; Parkinsonian Disorders; Phosphocreatine; Supranuclear Palsy, Progressive