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1.
Mark Obermann M. Küper O. Kastrup O. Yaldizli S. Esser J. Thiermann E. Koutsilieri G. Arendt H.-C. Diener M. Maschke 《Journal of neurology》2009,256(6):948-953
Dopaminergic dysfunction is thought to play a pivotal role in human immunodeficiency virus (HIV)-related dementia. Decreased
dopamine (DA) levels in the cerebrospinal fluid (CSF) and neuronal loss in the substantia nigra (SN) have been reported in
HIV-infected patients, suggesting nigrostriatal damage. Structural changes detectable as hyperechogenicity in transcranial
ultrasound (TCS) scans of the SN have been reported in patients with Parkinson’s disease (PD) and other neurological conditions.
In this study, we assessed the echomorphology of the SN in 40 HIV-positive patients compared to 40 age- and sex-matched healthy
controls and correlated these findings with CSF levels of DA and the metabolites homovanillic acid (HVA) and 3,4-dihydroxy
phenylacetic acid (DOPAC) and with neuropsychologic performance. We observed that the SN of HIV-infected patients was hyperechogenic
relative to that of controls (0.07 ± 0.05 vs. 0.04 ± 0.07 cm2; mean ± SEM; P < 0.001) and that this SN hyperechogenicity was correlated with decreased DA levels in the CSF, decreased CD4 cell count,
and an impaired performance in the psychopathology assessment scale (AMDP) subtest for drive and psychomobility. An association
to CDC stage, duration of HIV infection, or presence of HIV dementia was not observed. Our results indicate changes in the
nigrostriatal system in HIV-infected patients that are detectable as hyperechogenic SN precede prominent extrapyramidal symptoms
and cognitive dysfunction. 相似文献
2.
Heike Stockner Martin Sojer Klaus Seppi K Joerg Mueller Gregor K Wenning Christoph Schmidauer Werner Poewe 《Movement disorders》2007,22(3):414-417
Several studies have reported an increased risk to develop Parkinson's disease (PD) in essential tremor (ET) populations. Hyperechogenicity of the substantia nigra (SN) is a common transcranial sonography (TCS) finding in PD and has been suggested as a PD risk-marker in nonparkinsonian subjects. This study compared SN areas of 44 ET patients with 100 controls and 100 PD patients. Sixteen percent of ET patients had SN hyperechogenicity as compared with 3% of controls and 75% of PD patients. These findings might correspond to an increased risk of ET patients to develop PD. Long-term follow-up will show if those with hyperechogenic SN develop PD, while these without will not. 相似文献
3.
Transcranial sonography has become an important tool for the diagnosis of various movement disorders. In most patients with idiopathic Parkinson disease, a markedly hyperechogenic substantia nigra (SN) was detected on at least one side. We have highlighted the sonographic features that might help the differential diagnosis of PD and other movement disorders. Our investigation involved 30 patients (age 45–85 years) with idiopathic Parkinson disease, 2 multiple system atrophy, 3 progressive supranuclear palsy and 2 patients with restless legs syndrome. In accordance with several previous studies, we detected hyperechogenicity of the SN by TCS in 90 % of patients with idiopathic Parkinson disease. Subjects with a marked severity disease had a larger extent of the hyperechogenic SN signal. All progressive supranuclear palsy patients had an enlarged third ventricle and, in two cases, we observed the presence of hyperechoic areas in the lentiform nucleus. This last ultrasonographic feature was also seen in our patients with multiple system atrophy. TCS abnormalities of the SN, midbrain raphe and basal ganglia are characteristics of several movement and affective disorders. These features are less easily detected by other techniques, such as CT and MRI, which enable the exclusion of structural lesions, such as tumours and multi-infarct disease, because the physical principle differs from other imaging methods. 相似文献
4.
Panteha Fathinia Andreas Hermann Ulrike Reuner Jan Kassubek Alexander Storch Albert C. Ludolph 《Journal of neurology》2013,260(2):454-457
Clinical and neuroimaging data suggest impairment of the nigrostriatal system in amyotrophic lateral sclerosis (ALS). We thus hypothesized whether Parkinson’s disease (PD)-like midbrain sonography findings are also present in ALS. Eighty-six patients with the diagnosis of possible or definite ALS according to revised El Escorial criteria were examined by transcranial B-mode sonography compared to 76 age- and gender-matched controls and 33 PD patients. Hyperechogenic areas of the midbrain representing the substantia nigra were measured planimetrically using standard protocols. In subjects with sufficient temporal acoustic bone windows, mean midbrain hyperechogenic areas were significantly higher in ALS (0.251 ± 0.104 cm2) and PD patients (0.286 ± 0.078 cm2) compared to controls (0.091 ± 0.054 cm2) with no significant difference between ALS and PD patients (one-way ANOVA: F value = 94.3; P < 0.0001). Sixty-seven percent (95 % CI 57–78 %) of ALS patients and 84 % (95 % CI 71–97 %) of PD patients displayed abnormal midbrain hyperechogenic areas (P = 0.383 for group comparison, χ2 test). No correlations of hyperechogenic area sizes in ALS patients were found in regard to age, gender, ALS subtype (bulbar versus spinal form) or ALS-FRS-R score. In summary, we observed hyperechogenicity of the substantia nigra in patients with sporadic ALS with a frequency similar to that in PD and higher than in all other movement disorders. These findings are important for the diagnosis and differential diagnosis of PD and ALS alike. 相似文献
5.
Miyamoto M Miyamoto T Iwanami M Muramatsu S Asari S Nakano I Hirata K 《Sleep medicine》2012,13(1):102-106
Objectives
Transcranial sonography (TCS) has been shown to reveal hyperechogenicity of the substantia nigra (SN) in people with Parkinson’s disease and in approximately 10% of healthy subjects. It is hypothesized that SN hyperechogenicity in healthy subjects and patients with idiopathic rapid eye movement (REM) sleep behaviour disorder (iRBD) patients is a marker of vulnerability for Parkinson’s disease.Methods
TCS and positron emission tomography (PET) with 6-[18F] fluoro-meta-tyrosine (FMT), which can assess the level of the presynaptic dopaminergic nerve, were performed in 19 male patients with iRBD, mean age 66.4 (standard deviation [SD] 4.9) years, to assess nigrostriatal function.Results
Nine patients had pathological SN hyperechogenicity (mean age 66.8 [SD 3.9] years; 0.31 [SD 0.12] cm2) and 10 patients did not have SN hyperechogenicity (mean age 66.0 [SD 5.8] years; 0.11 [SD 0.06] cm2). FMT uptake at the putamen and caudate was significantly lower in iRBD patients with pathological SN hyperechogenicity compared with those without SN hyperechogenicity. However, no correlation was found between SN echogenicity and FMT uptake. This is in conflict with previous findings which showed that subjects with hyperechogenicity had lower FMT uptake in the striatum.Conclusion
Pathological hyperechogenic alterations in the SN in patients with iRBD may suggest the existence of preclinical SN dysfunction as determined by FMT-PET. 相似文献6.
Five‐year follow‐up of substantia nigra echogenicity in idiopathic REM sleep behavior disorder
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Mónica Serradell Bsc Klaus Seppi MD Francesc Valldeoriola MD Birgit Frauscher MD José Luis Molinuevo MD Isabel Vilaseca MD Thomas Mitterling MD Carles Gaig MD Dolores Vilas MD Joan Santamaria MD Birgit Högl MD Eduard Tolosa MD Werner Poewe MD 《Movement disorders》2014,29(14):1774-1780
Hyperechogenicity of the substantia nigra visualized by transcranial sonography occurs in most Parkinson's disease (PD) patients. Idiopathic rapid eye movement (REM) sleep behavior disorder (IRBD) subjects eventually develop PD and other synucleinopathies. This study was undertaken to evaluate whether in IRBD, transcranial sonography identifies subjects who convert to PD and other synucleinopathies, and whether substantia nigra echogenic size changes with time. It was a prospective study in which 55 IRBD patients underwent transcranial sonography at baseline and were invited to follow‐up after 5 years. Patients were assessed by the same experienced sonographer who was blinded to clinical data and baseline transcranial sonography results, and used the same equipment and adjustments. Twenty‐one (38.2%) subjects were diagnosed with a synucleinopathy (PD in 11, dementia with Lewy bodies in nine, and multiple system atrophy in one). Sensitivity of baseline substantia nigra hyperechogenicity for the development of a synucleinopathy was 42.1%, specificity 67.7%, positive predictive value 44.4%, negative predictive value 65.6%, and relative risk 1.29. No differences were detected between the first and second examination in mean size of the substantia nigra (0.20 ± 0.09 cm2 vs. 0.19 ± 0.07 cm2; P = 0.777) and in percentage of patients with substantia nigra hyperechogenicity (33.3% vs. 42.8%, P = 0.125). Transcranial sonography of the substantia nigra alone is not a useful tool to identify IRBD subjects at risk for the development of PD or a synucleinopathy after 5 years of follow‐up. In IRBD, transcranial sonography cannot be used to monitor the degenerative process in the substantia nigra, because echogenicity size remains stable over time. © 2014 International Parkinson and Movement Disorder Society 相似文献
7.
Nikola Kresojević Milija Mijajlović Stojan Perić Aleksandra Pavlović Marina Svetel Milena Janković Valerija Dobričić Ivana Novaković Milan B. Lakočević Christine Klein Vladimir S. Kostić 《Parkinsonism & related disorders》2013,19(4):431-435
ObjectivesThe aim of this study was to search for possible differences in the findings of transcranial sonography (TCS) between groups of patients with glucocerebrosidase (GBA)-associated Parkinson's disease (PD) (4 patients with Gaucher disease type 1 and parkinsonism [GD+PD+] and 18 PD patients with heterozygous GBA mutations; [GBA+PD+]) and groups of 12 patients with Gaucher disease type 1 and no signs of parkinsonism (GD+PD?), 9 asymptomatic carriers of heterozygous GBA mutations (GBA+PD?), 32 sporadic PD patients (sPD), and 43 healthy controls.ResultsIn all groups of patients, except asymptomatic carriers of heterozygous GBA mutations (mean ± SD: 0.16 ± 0.03 cm2), the maximal areas of substantia nigra hyperechogenicity (aSN-max) was higher (GD+PD+: 0.28 ± 0.15 cm2; GD+PD?: 0.18 ± 0.06 cm2; GBA+PD+: 0.27 ± 0.06 cm2; sPD: 0.28 ± 0.10 cm2) when compared to controls (0.12 ± 0.08 cm2) (p = 0.001). In GBA-associated PD (GD+PD+ and GBA+PD+) and sPD, aSNmax values were very similar. Moderate or marked SN hyperechogenicity was present in 87.5% of sPD patients and in 83% of PD patients with heterozygous GBA mutations, but in only 11.6% of controls, and in 22.2% and 33.3% of patients from GBA+PD? and GD+PD? groups, respectively (p < 0.001). The prevalence of interrupted or missing echogenicity of the brainstem raphe differed between the groups (p = 0.046), while no difference was observed in the diameter of the third ventricle.ConclusionsTCS findings in GBA-associated PD were consistent to those of patients with sporadic PD. 相似文献
8.
Hee Young Shin Eun Youn Joo Seong Tae Kim Hun-Jong Dhong Jin Whan Cho 《Neurological sciences》2013,34(6):935-940
Rapid eye movement (REM) sleep behavior disorder (RBD) is a preclinical feature of synucleinopathies, such as Parkinson’s disease (PD).This study aimed to investigate the presence of potential early manifestations of parkinsonism, such as olfactory dysfunction and substantia nigra (SN) hyperechogenicity, in idiopathic RBD (iRBD) patients, PD patients and normal controls. We performed an olfactory function test using the cross-cultural smell identification test (CC-SIT) and midbrain transcranial sonography (TCS) in 15 patients with iRBD as confirmed by polysomnography, 30 patients with PD, and 30 normal controls. The CC-SIT scores of the iRBD patients and PD patients were significantly lower than those of the normal controls and similar between iRBD and PD (mean ± SD, 7.1 ± 2.2 and 7.6 ± 2.4 vs. 10.4 ± 1.2, respectively, p < 0.01). The sum of bilateral SN echosignals in the iRBD patients was greater than that of the normal controls but lower than that of the PD patients (0.29 ± 0.47, 0.11 ± 0.17 and 0.72 ± 0.41 cm2, respectively, p < 0.01). In conclusion, we found that the concomitant abnormality of olfaction and increased SN echogenicity was more frequent in iRBD compared with normal control. Olfactory dysfunction and SN hyperechogenicity could be preclinical manifestations of parkinsonism in iRBD patients. 相似文献
9.
Background : Methamphetamine abuse has been linked to an increased risk of Parkinson's disease. Objective : The objective of this study was to investigate structural abnormality of the substantia nigra in past methamphetamine users using transcranial sonography. Methods: In a cross‐sectional, observational study, echogenicity of the substantia nigra was assessed in 59 past methamphetamine users and 59 matched controls. The frequencies of an abnormal spatial extension of the substantia nigra as well as the average sizes of left and right substantia nigra were evaluated. Results: The average echogenic size of the substantia nigra was larger in methamphetamine users (0.22 ± 0.06 cm2) when compared with controls (0.17 ± 0.05 cm2, P < .001). Furthermore, the frequency of enlarged, echogenic substantia nigra was increased in methamphetamine users (42% vs 12% in controls, P < .001). Partial correlation analysis revealed an association of echogenic substantia nigra size with estimated total lifetime intake of methamphetamine (r55 = 0.395, P = .002). Conclusions: Current data link methamphetamine abuse in humans to injury of substantia nigra neurons and an increased risk of Parkinson's disease. © 2017 International Parkinson and Movement Disorder Society 相似文献
10.
We describe a 78‐year‐old Japanese woman with early‐stage progressive supranuclear palsy (PSP). She had a 3‐week history of postural instability and gait disturbance. On examination, upper vertical gaze palsy, akinesia, hyperreflexia with pathological reflexes, hesitation, and postural instability were observed. Rigidity and resting tremors were not apparent. Brain MRI revealed atrophy of the frontotemporal lobes and dilatation of the third ventricle. A month later, she died of cerebral infarction. The total duration of her clinical course was approximately 2 months. The brain weighed 1180 g after fixation. Macroscopically, mild atrophy of the frontal lobes and mild depigmentation of the substantia nigra were observed. The conspicuous findings included degeneration confined to the subthalamic nucleus and substantia nigra and widespread but infrequent tau‐positive neurofibrillary tangles/pretangles and glial fibrillary tangles (tuft‐shaped astrocytes, coiled bodies and argyrophilic threads) in the brain. It has been reported that the most affected areas in PSP are the globus pallidus, subthalamic nucleus and substantia nigra. We suggest that degeneration in PSP would start with involvement of the substantia nigra and subthalamic nucleus. 相似文献
11.
IntroductionThe sonographic appearance of the substantia nigra is abnormally bright and enlarged (hyperechogenic) in young adults with a history of illicit stimulant use. The abnormality is a risk factor for Parkinson's disease. The aim of the current study was to identify the type of illicit stimulant drug associated with substantia nigra hyperechogenicity and to determine if individuals with a history of illicit stimulant use exhibit clinical signs of parkinsonism. We hypothesised that use of amphetamines (primarily methamphetamine) is associated with substantia nigra hyperechogenicity and clinical signs of parkinsonism.MethodsThe area of echogenic signal in the substantia nigra was measured in abstinent human amphetamine users (n = 27; 33 ± 8 years) and in three control groups comprising a) ‘ecstasy’ users (n = 19; 23 ± 3 years), b) cannabis users (n = 30; 26 ± 8 years), and c) non-drug users (n = 37; 25 ± 7 years). A subset of subjects (n = 55) also underwent a neurological examination comprising the third and fifth part of the Unified Parkinson's Disease Rating Scale.ResultsArea of substantia nigra echogenicity was significantly larger in the amphetamine group (0.276 ± 0.080 cm2) than in the control groups (0.200 ± 0.075, 0.190 ± 0.049, 0.191 ± 0.055 cm2, respectively; P < 0.002). The score on the clinical rating scale was also significantly higher in the amphetamine group (8.4 ± 8.1) than in pooled controls (3.3 ± 2.8; P = 0.002).ConclusionIllicit use of amphetamines is associated with abnormal substantia nigra morphology and subtle clinical signs of parkinsonism. The results support epidemiological findings linking use of amphetamines, particularly methamphetamine, with increased risk of developing Parkinson's disease later in life. 相似文献
12.
Echogenicity of the substantia nigra in Parkinson's disease and its relation to clinical findings 总被引:5,自引:0,他引:5
Recently an increased echogenicity of the substantia nigra (SN) in patients with Parkinson's disease (PD) was demonstrated
by transcranial ultrasound (TCS). In this study we set out to compare SN echogenicitiy with disease characteristics (time
of onset, duration, toxin exposure) in a large patients sample. Patients' history and exposure to toxins were recorded from
112 PD patients who underwent a thorough neurological examination including assessment of disease stage according to Hoehn
and Yahr and CURS (Columbia University Rating Scale). Personality was assessed according to the Freiburg Personality Inventory.
In all patients the area of SN echogenicity was encircled and measured by TCS. All except 9 patients had hyperechogenic SN
areas exceeding the mean plus standard deviation values of an age matched control group (0.19cm2). The age of disease onset was lower in patients who displayed an area of SN echogenicity above this value. The area of SN
echogenicity was larger contralateral to the side with more severe symptoms. None of the other characteristics correlated
with ultrasound findings. We conclude that SN hyperechogenicity is a typical finding in PD. The cause of hyperechogenicity
is so far unknown. Investigation of the underlying reason might disclose a pathogenic factor in PD.
Received: 13 November 2000 / Received in revised form: 24 January 2001 / Accepted: 19 February 2001 相似文献
13.
Gabrielle Todd Miranda Haberfield Patrick L. Faulkner Caroline Rae Michael Hayes Robert A. Wilcox Janet L. Taylor Simon C. Gandevia Jana Godau Daniela Berg Olivier Piguet Kay L. Double 《Journal of neural transmission (Vienna, Austria : 1996)》2014,121(11):1377-1386
Abnormal substantia nigra morphology in healthy individuals, viewed with transcranial ultrasound, is a significant risk factor for Parkinson’s disease. However, little is known about the functional consequences of this abnormality (termed ‘hyperechogenicity’) on movement. The aim of the current study was to investigate hand function in healthy older adults with (SN+) and without (SN?) substantia nigra hyperechogenicity during object manipulation. We hypothesised that SN+ subjects would exhibit increased grip force and a slower rate of force application compared to SN? subjects. Twenty-six healthy older adults (8 SN+ aged 58 ± 8 years, 18 SN? aged 57 ± 6 years) were asked to grip and lift a light-weight object with the dominant hand. Horizontal grip force, vertical lift force, acceleration, and first dorsal interosseus EMG were recorded during three trials. During the first trial, SN+ subjects exhibited a longer period between grip onset and lift onset (i.e. preload duration; 0.27 ± 0.25 s) than SN? subjects (0.13 ± 0.08 s; P = 0.046). They also exerted a greater downward force prior to lift off (?0.54 ± 0.42 N vs. ?0.21 ± 0.12 N; P = 0.005) and used a greater grip force to lift the object (19.5 ± 7.0 N vs. 14.0 ± 4.3 N; P = 0.022) than SN? subjects. No between group differences were observed in subsequent trials. SN+ subjects exhibit impaired planning for manipulation of new objects. SN+ individuals over-estimate the grip force required, despite a longer contact period prior to lifting the object. The pattern of impairment observed in SN+ subjects shares similarities with de novo Parkinson’s disease patients. 相似文献
14.
Masaoki Iwanami Tomoyuki Miyamoto Masayuki Miyamoto Koichi Hirata Etsuo Takada 《Sleep medicine》2010,11(4):361-365
BackgroundSubstantia nigra (SN) hyperechogenicity determined by transcranial sonography (TCS) and olfactory dysfunction are common findings in Parkinson disease (PD), which may reveal a prodromal synucleinopathy in idiopathic REM sleep behavior disorder (iRBD).MethodsTCS and the Odor Stick Identification Test for Japanese (OSIT-J) were performed in 34 consecutive patients with iRBD (67.9 ± 6.1 years), 17 consecutive patients with PD (66.4 ± 6.7 years), and 21 control group subjects (64.4 ± 5.8 years).ResultsThere was a significantly increased area of echogenicity in the SN in the iRBD group (0.20 ± 0.13 cm2) and PD group (0.22 ± 0.11 cm2) compared with the control group (0.06 ± 0.06 cm2). We found pathological SN hyperechogenicity (?0.20 cm2) in 41.2% of the iRBD group, 52.6% of the PD group, and 9.5% of the control group. Further, there were abnormal findings of both pathological SN hyperechogenicity (?0.20 cm2) and functional anosmia or hyposmia in 4 (11.8%) or 9 (26.5%) of the iRBD group subjects, respectively, and 7 (57.9%) or 2 (11.8%) of the PD group subjects, respectively.ConclusionPathological SN hyperechogenic abnormality and functional anosmia in iRBD may be a disease state in the transition to a neurodegenerative disease. 相似文献
15.
D Berg G Becker B Zeiler O Tucha E Hofmann M Preier P Benz W Jost K Reiners K W Lange 《Neurology》1999,53(5):1026-1031
OBJECTIVE: To assess the incidence of a hyperechogenic substantia nigra (SN) by transcranial sonography (TCS) in healthy people and to evaluate whether an enlarged hyperechogenic SN area is associated with functional impairment of the nigrostriatal system. BACKGROUND AND METHODS: Until now, preclinical impairment of the nigrostriatal system could be identified only by functional neuroimaging techniques such as PET in selected groups of patients. TCS is a new, noninvasive ultrasound technique that has demonstrated an increased echogenicity of the SN in patients with PD, whereas in most healthy individuals, the SN is either barely detectable or undetectable by TCS. RESULTS: Of 330 healthy volunteers, 8.6% exhibited an increased echogenicity of the SN. From these, 10 clinically healthy individuals with distinct unilateral or bilateral hyperechogenic signals in the SN region (SN area above 0.25 cm2) underwent comprehensive motor testing, neuropsychological assessment, MRI, and [18F]-dopa PET examination. With regard to motor functions, these individuals did not differ from 10 age- and sex-matched controls with a low echogenic SN and an area of echogenic signals below 0.2 cm2. Enlargement of hyperechogenic areas in the 10 healthy individuals was associated with a marked decrease in the accumulation of [15F]-dopa in the caudate nucleus and putamen. CONCLUSIONS: Substantia nigra hyperechogenicity appears to indicate a functional impairment of the nigrostriatal system. Transcranial sonography may be a suitable method of identifying persons at risk of nigrostriatal alterations, making possible the introduction of early neuroprotective therapy. 相似文献
16.
Stojan Peric MD Aleksandra Pavlovic MD PhD Vesna Ralic MS Valerija Dobricic PhD Ivana Basta MD PhD Dragana Lavrnic MD PhD Vidosava Rakocevic Stojanovic MD PhD 《Muscle & nerve》2014,50(2):278-282
Introduction: In this study we analyzed transcranial sonography (TCS) in patients with myotonic dystrophy type 1 (DM1). Methods: This cross‐sectional study included 66 DM1 patients and 55 matched healthy controls (HCs). Echogenicity of the brainstem raphe (BR) and substantia nigra (SN) and third ventricle width (DTV) were assessed by TCS. Results: BR hypoechogenicity was more common in DM1 patients than in HCs (37.7% vs. 7.8%, P < 0.01). Patients with depression or fatigue were more likely to have BR hypoechogenicity (80.0% vs. 29.4%, P < 0.01 and 51.9% vs. 24.2%, P < 0.05, respectively). Both hypoechogenicity and hyperechogenicity of SN were more frequent in DM1 patients than in controls (26.2% vs. 10.9% and 13.1% vs. 1.8%, respectively, P < 0.01). DTV was increased in DM1 patients compared with HCs (6.0 ± 1.4 vs. 4.9 ± 0.9 mm, P < 0.01). Conclusion: TCS can offer new insight into structural changes of several cerebral areas in patients with DM1. Muscle Nerve 50:278–282, 2014 相似文献
17.
《Sleep medicine》2020
ObjectiveTo analyze changes in substantia nigra (SN) iron deposits, assessed by quantitative transcranial sonography (TCS), to obtain and compare substantia nigra echogenicity indices (SNEI) at baseline and after intravenous (IV) iron therapy in patients with restless legs syndrome (RLS)/Willis−Ekbom disease (WED).MethodsA total of 30 consecutive subjects diagnosed with RLS/WED were recruited and underwent IV iron treatment. The SNEI, total daily dose of dopamine equivalents, and International Restless Legs Syndrome Rating Scale (IRLS) scores were obtained at baseline and following IV iron administration. Comparative statistics were performed by means of nonparametric testing.ResultsThe sample was stratified into two groups according to the median baseline SNEI and the grade of SN hypoechogenicity: severely hypoechogenic (HE) (n = 13) and moderately HE (n = 17). Following IV iron, the increase in SNEI among severely HE subjects was 19% (0.038 ± 0.046 cm2; P < 0.01), whereas in moderately HE subjects it was 10% (0.021 ± 0.069 cm2; P = 0.28). Among severely HE subjects, the average reduction in IRLS following IV iron was 10 ± 7.12 points (P < 0.01), in contrast to 1.85 ± 9.85 (not significant) among moderately HE subjects. Finally, we quantified the percentage of patients in each group who were able to reduce by ≥30% their total daily dopaminergic requirements (TDR) after IV iron, with a 57.14% reduction in the severely HE group vs 25% in the moderately HE group (P = 0.1). Three of 30 subjects (17%) were able to completely cease all dopaminergic treatment.ConclusionIntravenous iron caused changes in SNEI in both groups of patients, reflecting an increase in brain iron stores. However, the increase in SNEI was greater in patients previously defined as severely HE. Furthermore, RLS/WED symptoms also improved more in severely HE subjects, and there was a greater reduction in TDR. This study highlights the role of TCS in quantifying brain iron deposits and in predicting which patients will likely benefit from IV iron. 相似文献
18.
Armstrong RA Lantos PL Cairns NJ 《Journal of neural transmission (Vienna, Austria : 1996)》2007,114(12):1569-1577
Summary In eight cases of progressive supranuclear palsy (PSP), neurofibrillary tangles (NFT) were numerous in the substantia nigra
(SN), red nucleus (RN), locus caeruleus (LC), pontine nuclei (PN), and inferior olivary nucleus (ION) and abnormally enlarged
neurons (EN) in the ION, LC and PN. Loss of Purkinje cells was evident in the cerebellum. Tufted astrocytes (TA) were abundant
in the striatum, SN and RN and glial inclusions (‘coiled bodies’) (GI) in the midbrain (SN, RN) and pons (LC). Neuritic plaques
were frequent in one case. NFT, GI, and TA densities were uncorrelated in most areas. NFT and EN densities were positively
correlated in the midbrain and surviving neurons and disease duration in several areas. These results suggest: 1) predominantly
subcortical pathology in PSP with widespread NFT while TA and GI have a more localized distribution, 2) little correlation
between neuronal and glial pathologies, and 3) shorter duration cases may be more likely to develop cortical pathology. 相似文献
19.
We analyzed the results of transcranial sonography (TCS) on 110 Parkinson's disease (PD) patients, 30 essential tremor (ET) patients and 110 controls in a Chinese population and compared our findings to the previous literatures. The echo signal intensity of midbrain substantia nigra (SN) was measured and divided into grade I-V. If the high echo signal intensity (grade III, IV or V) was detected in either side of SN, it was measured as well as the whole area of midbrain and the ratio of both sides of SN hyperechogenicity to the whole area of midbrain (S/M) were calculated. In addition, the width of the third ventricle in the level of thalamus was also determined. There were more individuals with the grade of SN?≥?III in PD group (100/110, 85.45%) than these in ET group (4/30, 13.33%, x ( 2 )?=?58.38, P?0.001) and control group (11/110, 10%, x ( 2 )?=?125.51, P?0.001). The median and quartile range of SN hyperechogenicity area and S/M in PD patients were greater than those in ET patients and controls. Both the area of SN hyperechogenicity ≥0.2?cm(2) and the S/M?≥?0.07 were useful indexes to distinguish PD and ET in Chinese population. In conclusion, TCS is an effective and useful tool to detect PD and to distinguish PD from ET in Chinese patients. 相似文献
20.
Mesencephalic area measured by transcranial sonography in the differential diagnosis of parkinsonism
Isabel Sastre-Bataller Juan Francisco Vázquez Irene Martínez-Torres Patricia Sahuquillo Ignacio Rubio-Agustí Juan Andrés Burguera Jose Manuel Ferrer Caridad Valero Jose Ignacio Tembl 《Parkinsonism & related disorders》2013,19(8):732-736
BackgroundTranscranial B-mode sonography (TCS) has become an important tool in the differential diagnosis of parkinsonism given that current technology enables an adequate assessment of brain structures. In this study we aimed at evaluating the usefulness of midbrain area measured by TCS in the differential diagnosis between Parkinson's Disease (PD) and Progressive Supranuclear Palsy (PSP).MethodsPatients with a diagnosis of PD or PSP according to current clinical criteria were recruited. PSP patients were classified as Richardson's syndrome and PSP-parkinsonism. TCS was performed and the mesencephalic area and third ventricle width were measured offline by an examiner blinded to clinical diagnosis.ResultsTCS was performed in 60 patients (75% PD, 25% PSP). Eight patients (13,3%) had inadequate acoustic window. Patients with PSP had a smaller mesencephalic area (3.58 cm2 vs 5.28 cm2, p < 0.001). A mesencephalic area ≥4.27 cm2 discriminates PD from PSP with a positive predictive value 100%. Patients with PSP also had a higher third ventricle diameter (8.84 mm vs 5.11 mm, p < 0.001). Within the PSP group patients with Richardson's syndrome had a wider third ventricle than patients with PSP-Parkinsonism phenotype (9.57 mm vs 7 mm, p = 0.01), but no differences were found in the mesencephalic area between both phenotypes.ConclusionsMeasurement of the mesencephalic area and the third ventricle width by TCS is a non-invasive, easily accessible technique that is useful in the differential diagnosis between PD and PSP, at least in the late stages of the disease. 相似文献