Non-motor symptoms and the quality of life in multiple system atrophy with different subtypes

BackgroundThe differences in non-motor symptoms (NMS) and quality of life (QOL) between MSA patients with different subtypes remain unknown, so do the determinants of poor QOL in both subtypes.MethodsA total of 172 MSA patients were enrolled in the study. NMS of patients with MSA were assessed using the non-motor symptoms scale (NMSS) and Parkinson's Disease Questionnaire-39 item version (PDQ-39) was used to evaluate the QOL of patients with MSA.ResultsThe most prevalent NMS domain was urinary (91.3%) in both subtypes. The mood/apathy domain was more severe in MSA-P than MSA-C patients (P < 0.05). Drooling, constipation, and pain symptoms were more prevalent and severe in the MSA-P patients compared to the MSA-C patients (P < 0.05). We found that the MSA-C patients had a higher score of mobility than the MSA-P patients (P = 0.002); However, the MSA-P patients had a higher score of bodily discomfort than the MSA-C patients (P = 0.036). There were close correlations between NMS and PDQ-39 in both subtypes. Disease severity, cardiovascular symptoms, sleep/fatigue symptoms and gastrointestinal symptoms were determinants of poor QOL in MSA-P patients. While in MSA-C patients, longer disease duration, disease severity and mood/apathy symptoms were determinants of poor QOL.ConclusionNMS are more severe and prevalent in MSA-P patients, especially for mood/apathy and gastrointestinal symptoms. There is a close relationship between NMS and QOL in both MSA subtypes. Disease severity, longer disease duration and severe NMS are determinants of poor QOL in MSA.     

Cognitive function in multiple system atrophy of the cerebellar type.

Dementia represents an exclusion criterion for the diagnosis of multiple system atrophy (MSA), but there have been reports of fronto-executive dysfunction in patients with MSA of the striatonigral type (MSA-P). To study the cognitive profile of MSA, 20 patients with MSA of the cerebellar type (MSA-C) were subjected to an extensive neuropsychological test battery comprising tests for IQ, attention, verbal and visuospatial memory, as well as executive function. There was evidence for impaired verbal memory and verbal fluency. Test performance was not related to the severity of motor disability. Regarding the similar cognitive syndrome of MSA-P, the otherwise subclinical problems in MSA-C result from subcortical rather than from cerebellar dysfunction.     

Cognitive profiling in relation to short latency afferent inhibition of frontal cortex in multiple system atrophy

BackgroundCognitive dysfunction occurs in multiple system atrophy (MSA) more frequently than previously known. As a type of synucleinopathy, pathology spreads widely in cortical and subcortical areas as the disease advances. The exact anatomical and imaging substrates, and electrophysiological or biochemical indicators of cognitive impairment in MSA are not yet clear. Diminished short-latency afferent inhibition (SAI) of motor cortex was shown to be an electrophysiological correlate of dementia and mild cognitive impairment associated to Parkinson's disease (PD). We hypothesize that it can also be electrophysiological correlate of cognitive impairment in MSA.MethodsWe studied SAI and a neuropsychological test battery in 19 non-demented MSA patients (11 MSA-P and 8 MSA-C), 10 non-demented PD patients and 10 healthy controls. Neuropsychological test scores were grouped in four main cognitive domains (attention, memory, executive and visuo-spatial functions) and were analyzed by factor analysis.ResultsAll subject groups were matched for age. Moreover, the MSA-P, MSA-C, and PD groups were matched for disease duration. Scores of cognitive domains were similar in MSA and PD cases, while scores in attention, executive and visuo-spatial domains were worse in MSA than controls (p < 0.05). SAI was normal in PD but decreased in MSA patients by reaching statistical significance in MSA-C subtype. SAI response was correlated with cognitive performances measured by factor scores of neuropsychological test battery in all study subjects.ConclusionsThese results show that cognitive functions are impaired in MSA patients compared to controls as well as a parallel reduction in SAI response.     

Evolution of neuropsychological profile in motor subtypes of multiple system atrophy

IntroductionCognitive deficits and neuropsychiatric symptoms occur in parkinsonian and cerebellar subtypes of Multiple System Atrophy (MSA-P and MSA-C). These symptoms have been investigated mainly in cross-sectional studies. The present 1-year follow-up study aimed at evaluating the evolution of cognitive and neuropsychiatric profile in patients with MSA-C and MSA-P.MethodsTwenty-nine patients with MSA-P, 21 with MSA-C and 30 healthy subjects (HCs) underwent a neuropsychological battery and questionnaires assessing depression and apathy (T0). After 1 year (T1), patients with MSA-C and MSA-P underwent the same neuropsychological and neuropsychiatric tools employed at T0.ResultsAt T0, MSA-P and MSA-C groups were more depressed and apathetic and performed worse on tests assessing repetition abilities, executive and attentive functions than HCs. MSA-P and MSA-C groups did not differ on cognitive variables and neuropsychiatric scales. At T1, a significant worsening in spatial planning and psychomotor speed in MSA-C group and a significant worsening in memory, spatial planning, repetition abilities and functional autonomy in MSA-P group were found. The prevalence of apathy increased in both subtypes, whereas the prevalence of depression was reduced in MSA-C and relatively consistent in MSA-P.ConclusionsThe finding revealed a wide-ranging worsening of cognitive functions in MSA-P and a significant decline in processing speed in MSA-C. These results underline the relevance of evaluating cognitive and psychiatric features of MSA over the course of the disease in the daily clinical practice.     

3-Hz postural tremor in multiple system atrophy cerebellar type (MSA-C)—a static posturography study

The objective of the study is to evaluate postural dysfunction of multiple system atrophy-parkinsonian type (MSA-P) and cerebellar type (MSA-C) by static posturography exam. A total of 29 MSA-P patients, 40 MSA-C patients, and 23 healthy controls (HC) were recruited and engaged in a sensory organization test (SOT). The amplitude of the postural sway was measured and transformed into energy value by Fourier analyzer. SOT scores, frequency of falls and typical 3-Hz postural tremors during the four stance tasks, and energy value in three different frequency bands were recorded and compared. Compared with HC, SOT scores were significantly lower in MSA groups (P < 0.01). Compared with MSA-P, the vestibular scores were further reduced in MSA-C patients (P < 0.05). Falls were more frequent in MSA groups, especially in SOT4 task (foam surface with eyes closed) or in MSA-C group (P < 0.05). Typical 3-Hz postural tremor was observed in 97.5% MSA-C patients, in 24.1% MSA-P patients but in none of the HC (P < 0.05). Compared with HC, much more energy was consumed in every task, every direction, and nearly every frequency band in MSA groups. Energy value of MSA-C group was significantly higher than that of MSA-P, especially in higher frequency band (2 ~ 20 Hz) or in more difficult stance tasks (SOT 3 ~ 4, foam surface with eyes open or closed) (P < 0.05). Both MSA-P and MSA-C were characterized by severe static postural dysfunction. However, typical 3-Hz postural tremor was predominant in MSA-C and was very useful in the differential diagnosis between MSA-P and MSA-C.     

Vocal cord electromyographic correlates of stridor in multiple system atrophy phenotypes

IntroductionMultiple system atrophy (MSA) is a neurodegenerative disorder characterized by dysautonomia in combination with parkinsonian and cerebellar signs. Stridor may also occur and it is associated with life-threatening events and poor prognosis. The pathophysiology of stridor in MSA is still debated.ObjectiveTo define correlations between diurnal electromyographic (EMG) abnormalities of vocal cord muscles and stridor in MSA phenotypes.MethodsWe recruited 60 patients with “probable” MSA (45 with parkinsonian [MSA-P] and 15 with cerebellar phenotype [MSA-C]). Nocturnal stridor was detected with video-polysomnography, whereas diurnal stridor was clinically noted when present. A diurnal kinesiologic EMG study of the adductor thyroarytenoid and the abductor posterior cricoarytenoid muscles was also performed.ResultsAmong subjects with nocturnal stridor, MSA-P patients predominantly showed a paradoxical burst-like activation of the adductor thyroarytenoid muscle during inspiration. This dystonic pattern was associated with nocturnal stridor in MSA-P (odds ratio [OR] = 23.64, 95% confidence interval [CI] 3.42–70.77, p < 0.001). Conversely, MSA-C patients with nocturnal stridor mainly had additional neurogenic findings of vocal cord muscles. This dystonic-plus pattern correlated with nocturnal stridor in MSA-C (OR = 17.21, 95% CI 4.17–74.92, p < 0.01). The findings of diurnal stridor paralleled the observations for nocturnal stridor.ConclusionsThe pathophysiology of stridor may differ between MSA phenotypes, possibly related to dysfunctional supranuclear mechanisms in MSA-P (dystonic pattern) and to additional nuclear damage in MSA-C (dystonic-plus pattern).     

Differentiating Parkinson's disease from multiple system atrophy by [123I] meta-iodobenzylguanidine myocardial scintigraphy and olfactory test

We aimed to study whether either [¹²³l] myocardial meta-iodobenzylguanidine (MIBG) myocardial scintigraphy or the odor stick identification test for Japanese (OSIT-J) is effective in differentiating Parkinson’s disease (PD) from multiple system atrophy (MSA). We compared the MIBG accumulation and olfactory score between 42 PD and 42 MSA (19 MSA-P and 23 MSA-C) patients in the early stages. [¹²³l] MIBG myocardial scintigraphy showed higher sensitivity and the olfactory test higher specificity in differentiating PD from MSA. There were significant differences between PD and MSA-C (p = 0.0019) instead of MSA-P (p > 0.05) in the MIBG accumulation, while there were significant differences between PD and MSA-P (p = 0.0003) or MSA-C (p = 0.0003) in the OSIT-J score. Our data suggest that the olfactory test can be useful as a clinical tool with its higher specificity in differentiating PD from MSA in the early stages and, moreover, support the discrimination of PD from MSA-P.     

多系统萎缩的认知功能障碍特点分析

目的通过对多系统萎缩患者(MSA)进行认知功能评估,明确其是否存在认知功能障碍,并分析两种亚型(MSA-C型和MSA-P型)的认知功能障碍特点,以期为临床诊断提供参考。方法采用简易智能量表(MMSE)、蒙特利尔认知评估量表(Mo CA)和阿尔茨海默病评定量表-认知分量表(ADAS-cog)分别测评23例MSA患者(MSA-C型13例;MSA-P型10例)和25例健康志愿者的认知功能。结果 MSA组MMSE和Mo CA评分均低于对照组,差异有统计学意义(P0.05);ADAS-cog评分高于对照组,差异有统计学意义(P0.05)。在视空间/执行能力、注意力、语言、抽象思维和延迟记忆方面,MSA组的Mo CA评分低于对照组,差异均有统计学意义(P0.05);在记忆、语言和视空间/执行能力方面,MSA组的ADAS-cog评分高于对照组,差异均有统计学意义(P0.05)。MSA-P型Mo CA评分低于MSA-C型,差异有统计学意义(P0.05);MSA-P型ADAS-cog评分高于MSA-C型,差异有统计学意义(P0.05);MSA-P型在抽象思维和延迟记忆两项目的评分低于MSA-C型,差异有统计学意义(P0.05);MSA-P型在记忆和视空间/执行能力方面的评分高于MSA-C型,差异有统计学意义(P0.05)。结论 MSA患者存在一定程度的认知功能障碍;MSA-P型认知损害较MSA-C型更加广泛和严重。     

Skin temperature of the hand in multiple system atrophy and Parkinson's disease

AimA previous study on a small number of patients showed that low skin temperature of the hands, the so called “cold hands sign”, may be useful for distinguishing multiple system atrophy (MSA) from Parkinson's disease (PD). We have further investigated skin temperature of the hand in a larger number of patients.MethodsSkin temperature on the palm was measured in 50 MSA (11 MSA-P and 39 MSA-C patients) and 50 PD patients, and 25 normal healthy subjects.ResultsPalm skin temperature was significantly lower in MSA patients (32.0 ± 2.7 °C) than in controls (34.1 ± 0.9 °C, p = 0.0002), but was not different compared with the PD group (32.9 ± 1.8 °C, p = 0.06). Temperatures of <28 °C were observed in 3 MSA patients (6%) and none of the PD patients and controls. There was no significant difference in palm skin temperature between patients with and without orthostatic hypotension for each patient group, or between MSA-P and MSA-C patients.ConclusionThe cold hand (<28 °C) is a useful marker for distinguishing MSA from PD, but it is not common in MSA patients, and its sensitivity may be low for differentiating between MSA and PD.     

Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities,differences and survival

Multiple system atrophy (MSA) is a neurodegenerative disease with two motor phenotypes: parkinsonian (MSA-P) and cerebellar (MSA-C). To elucidate whether in addition to the motor abnormalities there are other significant differences between these phenotypes, we performed a retrospective review of 100 patients (61 males, 39 females) with a diagnosis of possible (12 %), or probable (88 %) MSA. Four patients eventually had post-mortem confirmation (i.e., definite MSA). Sixty percent were classified as having MSA-P and 40 % as MSA-C. MSA-C and MSA-P patients had similar male prevalence (60 %), age of onset (56 ± 9 years), and frequency of OH (69 %). Brain MRI abnormalities were more frequent in MSA-C patients (p < 0.001). Mean survival was 8 ± 3 years for MSA-C and 9 ± 4 years for MSA-P patients (p = 0.22). Disease onset before 55 years predicted longer survival in both phenotypes. Initial autonomic involvement did not influence survival. We conclude that patients with both motor phenotypes have mostly similar survivals and demographic distributions. The differences here identified could help counseling of patients with MSA.     

Cognitive deficits in multiple system atrophy correlate with frontal atrophy and disease duration

Background and purpose:  Dementia remains an exclusion criterion in diagnosing multiple system atrophy (MSA). This study aimed to determine the cognitive changes and brain atrophy patterns in the Parkinsonian (MSA-P) and cerebellar (MSA-C) variants of MSA.
Methods:  Voxel-based morphometry (VBM) of magnetic resonance imaging (MRI) and neuro-psychological tests were applied to 10 MSA-C and 13 MSA-P patients, and compared to 37 age-matched controls. Correlation analyses were performed between cognitive test results and morphometric data extracted from the VBM data.
Results:  In neuro-psychological testing, the 23 MSA patients scored lower in the Stroop interference test and took longer in the trail-making test as compared with the controls, whereas MSA-C performed worse than MSA-P in the memory scores, Stroop test, and time to complete the trail-making test. MSA, as a group, showed atrophy in the cerebellum, insular cortex, fusiform gyrus, inferior orbito-frontal gyrus, superior temporal gyrus, and caudate nucleus. Memory scores correlated well with pre-frontal lobe atrophy but not in the insular area.
Conclusion:  In conclusion, although dementia is not a typical presenting feature of MSA and is regarded as a sub-cortical movement disorder, frontal atrophy, cognitive changes, and dementia are identifiable as MSA progresses.     

Primary Sjogren's syndrome: cognitive symptoms, mood, and cognitive performance

Segal BM, Pogatchnik B, Holker E, Liu H, Sloan J, Rhodus N, Moser KL. Primary Sjogren’s syndrome: cognitive symptoms, mood, and cognitive performance.
Acta Neurol Scand: 2012: 125: 272–278.
© 2011 John Wiley & Sons A/S. Objective – To investigate the relationships between self‐reported cognitive abilities, psychological symptoms and neuropsychological outcomes in PSS. Methods – Patients with Primary Sjogren’s syndrome (PSS) and healthy controls completed a comprehensive neuropsychometric battery and questionnaires: the Centers for Epidemiological Scale‐Depression, the Profile of Fatigue‐mental domain (Prof‐M) for cognitive symptoms, Fatigue Severity Scale, and the Short‐Form McGill Pain Questionnaire. Results – Female patients with PSS (N = 39) were similar to controls (N = 17) in estimated premorbid intellectual function, age and education. Depression (P = 0.002), cognitive symptoms (P = 0.001), fatigue (P = 0.000003), and pain (P = 0.024) scores were greater in the patient group. Patients with PSS demonstrated inferior performance relative to controls in psychomotor processing (P = 0.027) and verbal reasoning (P = 0.007). Patients with PSS with and without depression had similar performance on multiple tests, but depressed patients had significantly lower scores for executive function (P = 0.041). Cognitive symptoms correlated with verbal memory (P = 0.048), whereas pain correlated with executive function measures (Stroop, P = 0.017) and working memory (Trails B, P = 0.036). In the regression model, depression and verbal memory were independent predictors that accounted for 61% of the variance in cognitive symptoms. Conclusion – The Prof‐M is a simple self‐report measure which could be useful in screening PSS subjects who may benefit from detailed psychometric evaluation. Our results are consistent with the hypothesis that depression and verbal memory impairment are overlapping but independent aspects of neural involvement in PSS. While pain and depression are significant confounders of cognitive function in PSS, this study suggests that impaired verbal reasoning ability in PSS is not attributable to pain or depression.     

Characterization and diagnostic potential of diffusion tractography in multiple system atrophy

IntroductionMicrostructural integrity of the middle cerebellar peduncle (MCP) and the putamen captured by diffusion-tensor imaging (DTI) is differentially affected in the parkinsonian and cerebellar variants of multiple system atrophy (MSA-P, MSA-C) compared to Parkinson's disease (PD). The current study applied DTI and tractography in order to 1) characterize the distribution of DTI metrics along the tracts of the MCP and from the putamen in MSA variants, and 2) evaluate the usefulness of combining these measures for the differential diagnosis of MSA-P against PD in the clinical setting.MethodsTwenty-nine MSA patients (MSA-C, n = 10; MSA-P, n = 19), with a mean disease duration of 2.8 ± 1.7 years, 19 PD patients, and 27 healthy controls (HC) were included in the study. Automatized tractography with a masking procedure was employed to isolate the MCP tracts. DTI measures along the tracts of the MCP and within the putamen were acquired and jointly used to classify MSA vs. PD, and MSA-P vs. PD. Putamen volume was additionally tested as classification feature in post hoc analyses.ResultsDTI measures within the MCP and putamen showed significant alterations in MSA variants compared to HC and PD. Classification accuracy for MSA vs. PD and MSA-P vs PD using diffusion measures was 91.7% and 89.5%, respectively. When replacing the putaminal DTI measure by a normalized measure of putamen volume classification accuracy improved to 95.8% and 94.7%, respectively.ConclusionMultimodal information from MCP tractography and putamen volume yields excellent diagnostic accuracy to discriminate between early-to-moderately advanced patients with MSA and PD.     

The relationship between impairment of voluntary movements and cognitive impairment in Huntington’s disease

The relationship between motor symptoms and cognitive impairment in Huntington’s disease (HD) is still discussed. We analysed 45 HD patients in various stages using Unified Huntington’s Disease Rating Scale motor subscale (voluntary and involuntary components were evaluated separately), verbal memory and executive functions tests. Partial correlations controlling for HD duration and age were used to estimate the relationships among factor scores for motor and cognitive impairment. Voluntary components of motor performance were found to be significantly correlated with verbal short-term memory disturbances (r = −0.361, P = 0.03), with tests of executive functions more dependent on motor performance (r = 0.640, P < 0.01) and also with tests of executive functions less dependent on motor performance (r = 0.461, P < 0.01). Involuntary components did not correlate significantly with any part of cognitive performance.     

The Cognition and Emotional Well-being indices of the Parkinson's disease questionnaire-39: What do they really measure?

IntroductionThe Parkinson's disease questionnaire-39 (PDQ-39) is a common measure of health related quality of life (HRQoL) that is widely used with Parkinson disease (PD) patients. Previous evidence suggests that the PDQ-39 reflects at least 8 dimensions (i.e., Emotion, Cognitions, Mobility, etc). To date, little research has examined the external/convergent validity of the Cognitions and Emotional Well-being domains of the PDQ-39.MethodsA convenience sample of 303 PD patients underwent a comprehensive multi-domain neuropsychological evaluation, including tests of execution function, episodic verbal memory, processing speed, language and working memory, as well as completing measures of depression, apathy, state and trait anxiety and HRQoL (PDQ-39). Hierarchical regressions were conducted in order to examine the relationship between scores on neuropsychological tests and the Cognitions index, as well as mood measures and the Emotional Well-being index of the PDQ-39.ResultsNeuropsychological test performance did not account for a significant amount of variance in the PDQ-39 Cognitions index scores. Instead, it was depression that significantly contributed to the Cognitions index, above and beyond neuropsychological performance. The PDQ-39 Emotional Well-being index was also related to mood measures, primarily depression and trait anxiety.ConclusionsThe PDQ-39 Cognition index may be more related to mood functioning, as opposed to cognitive functioning, and should not be considered a “proxy” for cognitive functioning. Future studies are needed to better explain the construct of this index.     

Orthostatic Hypotension Is Differentially Associated with the Cerebellar Versus the Parkinsonian Variant of Multiple System Atrophy: a Comparative Study

Orthostatic hypotension (OH) is a cardinal feature of autonomic failure in multiple system atrophy (MSA); however, there are few comparative data on OH in the motor subtypes of MSA. In the present retrospective study, postural blood pressure drop after 3 min of standing was determined in 16 patients with the cerebellar variant of MSA (MSA-C) and in 17 patients with the Parkinson variant (MSA-P). Twenty idiopathic Parkinson’s disease (IPD) patients matched for age, sex, disease duration and dopaminergic therapy served as control group. OH frequency and severity were more pronounced in MSA-C followed by MSA-P and IPD. Differences in brainstem pathology are likely to account for the tight association of MSA-C and OH. A simple standing test should be obligatory in the work-up of patients with sporadic late-onset ataxias.     

The effect of natalizumab on cognitive function in patients with relapsing-remitting multiple sclerosis: preliminary results of a 1-year follow-up study

The objective of the study was to assess the natalizumab effect on the course of cognitive impairment in patients with relapsing-remitting multiple sclerosis (MS). Patients with active relapsing-remitting MS (n = 17) were treated with natalizumab for 1 year. The quasi control group included patients (n = 7) with clinically stable MS. Assessment of disease course [expanded disability status scale (EDSS); number of relapses] and neuropsychological impairment [Wisconsin card sorting test (WCST); controlled oral word associations; verbal/non-verbal memory tests; paced auditory serial addition test] was conducted at baseline and after 1 year. Natalizumab-treated patients experienced significantly fewer relapses compared with the previous year (P < 0.05). At 1-year follow-up, EDSS score was unchanged and neuropsychological assessments of memory/executive functions showed a significant improvement in natalizumab-treated patients (all P < 0.05). No changes were observed in the quasi control group. This preliminary study suggests that natalizumab could be effective in ameliorating cognitive functions in patients with active relapsing-remitting MS, over 1-year follow-up.     

Visual event-related potential changes in two subtypes of multiple system atrophy,MSA-C and MSA-P

We investigated the visual event-related potentials (ERPs) in two subtypes of multisystem atrophy (MSA) in 15 MSA-C patients, 12 MSA-P patients, and 21 normal control (NC) subjects. We used a visual oddball task to elicit ERPs. No significant changes were seen in N1 or N2 latency, in either MSA-C or MSA-P, compared with the NC group. An early stage of visual information process related to N1 and a visual discrimination process related to N2 might be preserved in both MSA-C and MSA-P. The P3a peak was more frequently undetectable in MSA than in the NC group. Significant P3a amplitude reduction in both MSA-C and MSA-P suggests impairment of the automatic cognitive processing in both MSA-C and MSA-P. Significant difference was found in P3b latency and P3b amplitude only in MSA-C, compared with the NC group. The result suggests the impairment of the controlled cognitive processing after the visual discrimination process in the MSA-C group. We further investigated the correlation between visual ERP changes and magnetic resonance imaging (MRI) data. Quantitative MRI measurements showed reduced size of the pons, cerebellum, perisylvian cerebral area, and deep cerebral gray matter in both MSA-C and MSA-P, and of the corpus callosum only in MSA-P, as compared to NC group. In both MSA-C and MSA-P, P3b latency was significantly correlated with the size on MRI of the pons and the cerebellum. P3b latency in the whole MSA group was also significantly correlated with the size of the pons and the cerebellum. These results indicate that P3b latency changes in parallel with the volume of the pons and the cerebellum in both MSA-C and MSA-P. Received: 28 August 2001 Received in revised form: 22 January 2002 Accepted: 25 January 2002     

Nerve conduction studies in multiple system atrophy

To study the frequency and severity of peripheral neuropathy in multiple system atrophy (MSA), we performed nerve conduction studies in 42 MSA patients suffering from either cerebellar MSA (MSA-C) or parkinsonian MSA (MSA-P). Abnormal nerve conduction was present in 24% of the patients. Abnormalities were significantly more frequent in MSA-P (43%) compared to MSA-C (14%). Motor nerve conduction velocities were reduced in 4% of the MSA-C and in 7% of the MSA-P patients. Abnormal compound muscle action potentials were more frequent in MSA-P (29% versus 7% in MSA-C) pointing to a more pronounced loss of motor axons in this subgroup. Sensory nerve conduction velocities were abnormal in 4% of the MSA-C and 14% of the MSA-P patients, and mean sensory nerve action potentials were normal in all MSA-C and reduced in 7% of the MSA-P patients. The data provide evidence that the peripheral nervous system is differentially affected in MSA-C and MSA-P.     

多系统萎缩不同亚型间的临床特征及脑葡萄糖代谢差异分析

目的分析多系统萎缩-帕金森亚型和多系统萎缩-小脑共济失调亚型患者的临床特征以及18氟-脱氧葡萄糖正电子发射断层(l8F-FDG PET)显像的特征差异。方法收集8例多系统萎缩-帕金森亚型患者和17例多系统萎缩-小脑共济失调亚型患者的临床资料,回顾性分析包括统一帕金森病评定量表-运动部分(UPDRS Ⅲ)评分和Hoehn-Yahr分级的运动症状评估,认知功能、抑郁、嗅觉、快速眼动期睡眠行为紊乱等非运动症状评估,以及基于l8F-FDG PET脑葡萄糖代谢显像的特征差异。结果多系统萎缩-帕金森亚型与多系统萎缩-小脑共济失调亚型患者UPDRS Ⅲ评分(P=0.004)及Hoehn-Yahr分级(P<0.001)比较,差异有统计学意义,而非运动症状组间比较,差异无统计学意义(P>0.05)。多系统萎缩-帕金森亚型在基底节(尤其是后壳核)脑葡萄糖代谢(P<0.001)、小脑及顶枕叶呈低代谢,多系统萎缩-小脑共济失调亚型基底节脑葡萄糖代谢未见减低,仅在小脑和枕叶呈低代谢。结论多系统萎缩的临床特征异质性大,l8F-FDG PET脑葡萄糖代谢显像特征差异可为深入研究多系统萎缩的发病机制、开发更精准治疗奠定基础。