Early Multiple System Atrophy Research Articles

18F-FDG PET imaging in early diagnosis of multiple system atrophy

Objective To evaluate the feasibility of 18F-FDG PET/CT imaging in the early diagnosis of multiple system atrophy (MSA) and differentiation from early PD.Methods Five patients with MSA were included in this study.These patients had a disease duration of less than 2 years,predominantly with cerebellar dysfunction (MSA-C).Ten clinically confirmed PD patients with disease duration less than 3 years were also enrolled.All patients underwent 18F-FDG PET/CT imaging.To compare the metabolic features of these two diseases,SPM 5 software and t-test were used for image data analysis.The PD related pattern (PDRP) expression was calculated in all patients and the efficacy of the PDRP score in distinguishing early MSA from PD was evaluated.Results At the t-test level of P =0.01,all the 5 MSA patients displayed cerebellar hypometabolism ( t:-13.04 to -3.75 ) and 3 of them also had decreased metabolism in the putamen (t:-5.14 to -3.21 ).On the contrary,all PD patients showed cerebellar hypermetabolism (t:4.16 to 11.72) and normal metabolism in putamen.The PDRP scores in the PD group were significantly higher than those in the MSA group(ll.9±4.96vs -6.25±5.97,t=6.27,P＜0.01).Conclusions Cerebellar hypometabolism is the predominant FDG-PET feature of MSA-C,which may be used to distinguish it from early PD.Striatum may be involved in the early phase of MSA-C,displaying hypometabolism in putamen.The PDRP scores are also helpful in differentiating early phase MSA-C from early PD. Key words: Multiple system atrophy; Tomography, emission-computed ; Tomography,X-ray computed; Diagnosis, differential; Deoxyglucose

A case of early multiple system atrophy unveiled by antidepressant administration

A case of early multiple system atrophy unveiled by antidepressant administration

The value of apparent diffusion coefficient values of cerebellar and the middle cerebellar peduncles in differential diagnosis of multiple system atrophy and Parkinson disease

Objective To evaluate the apparant diffusion coefficient (ADC) values of cerebellar and the middle cerebellar peduncles in the differential diagnosis of multiple system atrophy (MSA) and Parkinson disease (PD). Methods Conventional MRI and DWI were performed in 18 clinically proved MSA patients with 7 cases of early cases (early-stage MSA group), 19 PD patients (PD group) and 18 agematched normal controls (the control group). DWI was performed using a single shot-spin echo-echo planar imaging sequences, and ADC values were measured in the ROIs (0. 16 cm2) of the bilateral cerebellum, the middle cerebellar peduncles and cerebral white matter. Then one way ANOVA test was used for statistical analysis. Results Of the 18 MSA patients, 11 had MR abnormalities, 8 had hot-cross bun sign in the pens on T2-weighted images, 11 patients had pontine, cerebellar and medulla oblongata atrophy, 10 patients had atrophy of the middle cerebellar peduncles, 2 patients had hyperintense rim of the putamen and putaminal atrophy on T2-weighted images. The ADC values in the middle cerebellar peduncles were significantly increased in the MSA group[ (0. 98 ±0. 07) × 103 mm2/s] and early-stnge MSA group [ (0. 95 ±0. 05) ×103 mm2/s] as compared to PD group [ (0. 77 ±0. 04) × 103 mm2/s] and control group[ (0. 78 ±0. 04) ×103 mm2/s]. There was statistical significant difference among them (F = 91.049,55. 301, P ＜ 0.01 ).There was no overlap in the distribution of ADC values of the middle cerebellar peduncles among the MSA group [ (0.86-1.13 ) × 103 mm2/s ], early-stage MSA group [ (0. 86-1.02 ) × 103 mm2/s ] and PD group [ (0. 68-0. 84) × 103 mm2/s] and the control group [ (0. 69-0. 82) × 103 mm2/s]. The ADC values in the cerebellum were significantly increased in the MSA group[ (0. 95 ±0. 09) × 103 mm2/s] and early-stage MSA group [ (0. 92 ±0. 07) × 103 mm2/s] as compared to PD group [ (0. 78 ±0. 05) × 103 mm2/s] and control group[ (0. 79 ± 0. 05 ) × 103 mm2/s ]. Statistically significant difference was found among them (F =39. 274,18. 623 ,P ＜0. 01 ). There was overlap in the distribution of ADC values of the cerebellum [ MSAgroup(0. 80-1.10) × 103 mm2/s,early stage MSA group (0. 80-0. 99) × 103 mm2/s,PD group(0. 72-0. 90) × 103 mm2/s,control group (0. 71-0. 87) × 103 mm2/s]. There was no significant difference among the ADC values of MSA group, MSA group(early stages) and PD group and the control group in the cerebral white matter( P ＞ 0. 05 ). Conclusions ADC values in the cerebellum and the middle cerebellar peduncles have very important significance in differential diagnosis between MSA and PD. Key words: Multiple system atrophy; Parkinson disease; Diffusion magnetic resonance imaging

Fractional anisotropy values detect pyramidal tract involvement in multiple system atrophy

Pathological studies have shown remarkable pyramidal tract involvement in multiple system atrophy (MSA), while clinical pyramidal signs are relatively rare. We investigated the fractional anisotropy (FA) values to assess the degree of pyramidal tract involvement in MSA, in comparison with amyotrophic lateral sclerosis (ALS) and controls. Furthermore, we compared FA values between MSA patients with or without clinical pyramidal signs and controls, and between MSA patients with or without positive conventional MRI findings and controls. We evaluated FA values in the internal capsule, corona radiate and whole pyramidal tract using visualized tractography of 65 subjects (20 probable MSA patients, 28 age-matched ALS patients, and 17 age-matched healthy controls) using a 3.0T magnetic resonance system. The FA values in the internal capsule, corona radiate, and whole pyramidal tract were significantly lower in MSA patients than in controls and were at a level similar to those of ALS patients. In addition, low FA values were prominent in MSA patients, even in those with short duration of illness, lacking precentral gyrus hyperintensity in FLAIR images, and without pyramidal signs. FA values could identify pyramidal tract degeneration even in patients with early phase MSA and those without clinical pyramidal signs or abnormal MRI findings. More extensive degeneration of the pyramidal tract occurs in MSA than so far believed.

Serum prolactin levels in Parkinson's disease and multiple system atrophy.

The hypothalamic-pituitary axis (HPA) may be involved early in multiple system atrophy (MSA), whereas in idiopathic Parkinson's disease (IPD) its impairment seems to be correlated with motor disability. The release of prolactin (PRL) is mediated through the HPA and an increase in PRL levels is documented during stress. In this study, we investigated basal and erect PRL levels to assess whether basal PRL or changes in PRL levels after 60 degrees head-up tilt (HUT; orthostatic stress) could distinguish between MSA and IPD patients. We studied five patients with MSA on levodopa treatment, five levodopa-naive MSA patients, nine IPD patients on dopaminergic treatment, six drug-naive IPD patients and six normal individuals. PRL levels were measured in the supine position after 30 minutes rest and during 60 degrees HUT after 5 and 15 minutes. Baseline PRL values were significantly lower for patients with IPD than for those with MSA, both for levodopa-treated and naive patients ( p < 0.004, estimated decrease 55.1 %, 95 % CI from 29.4 % to 71.52 %). After orthostatic stress PRL levels were increased in healthy individuals after 15 minutes of HUT ( p = 0.044, estimated increase 11.5 %, 95 % CI from 0.4 % to 23.8 %), whereas there was no evidence for a change of PRL levels in patients with MSA or IPD after 5 and 15 minutes of HUT. We also did not find any evidence for a difference in PRL change after HUT between MSA and IPD patients. Baseline PRL levels might differentiate between early MSA and IPD, being higher in MSA. However, orthostatic stress using HUT appears unable to differentiate between MSA and IPD.

Cerebral metabolic changes in early multiple system atrophy: a PET study

Previous positron emission tomography (PET) studies have shown widespread hypometabolism in the brain of advanced MSA but the time course of these metabolic abnormalities is largely unknown. In order to clarify the principal disease processes in multiple system atrophy (MSA) in the early stage, we investigated regional cerebral glucose metabolism (rCMGglc) and nigral dopaminergic function in nine patients with early stage of MSA using [ 18F]fluorodeoxyglucose (FDG) and 6- l-[ 18F]fluorodopa ( 18F-Dopa) positron emission tomography (PET) (two men and seven women; age, 59.3±5.4 years; disease duration, 29.7±14.6 months). The rCMRglc in the early MSA patients significantly decreased in the cerebellum, brainstem, and striatum compared with that in nine normal subjects. A significant correlation was found between the severity of autonomic dysfunction and rCMRglc within the brainstem. The severity of extrapyramidal signs also correlated with the decline of F-Dopa uptake but not that of rCMRglc within the striatum. The degree of atrophy on MRI has correlated with neither the clinical symptoms nor rCMRglc at the cerebellum and the brainstem. Our PET studies demonstrated widespread metabolic abnormalities except for the cerebral cortex in the brain of MSA even in the early stage. The hypometabolism in the brainstem was tightly linked to the autonomic dysfunction. Not the striatal dysfunction but the nigral damage may be responsible for the extrapyramidal symptoms in early MSA.

Levodopa Efficacy and Pathological Basis of Parkinson Syndrome

Levodopa is the most effective drug for symptomatic control of Parkinson syndrome (PS). We report a 22-year clinicopathological study of 59 PS cases. Of the entire group, 37 (63%) had an adequate trial on levodopa. Some improvement was noted on that drug in 24 (65%) cases. Improvement was seen in 94% of idiopathic Parkinson's disease cases as well as in all cases in which the pathology was characterized by neuronal loss in the substantia nigra without Lewy body inclusions. Improvement was also noted in 60% of patients with the dual pathology of idiopathic Parkinson's disease and Alzheimer's disease, and in one-third of early multiple system atrophy cases. We conclude that improvement on levodopa is a strong indication that the pathological basis of the parkinsonism is the damage to substantia nigra neurons. A favorable response to levodopa, however, is not an indication of idiopathic (Lewy body) Parkinson's disease.