Substantia Nigra Echogenicity Research Articles

Transcranial Sonography Characteristics of Cerebellar Neurodegenerative Ataxias.

Cerebellar neurodegenerative ataxias are a group of disorders affecting the cerebellum and its pathways with different neurological structures. Transcranial sonography (TCS) has been used for the evaluation of brain parenchymal structures in various diseases because of its fast and safe utilization, especially in neuropsychiatric and neurodegenerative diseases. The aim of our study was to investigate TCS characteristics of patients with neurodegenerative cerebellar ataxias. In our study, we included 74 patients with cerebellar degenerative ataxia; 36.5% had autosomal dominant onset, while 33.8% had sporadic onset. Standardized ultrasonographic planes were used for the identification of brain structures of interest. The SARA, INAS, neuropsychological and psychiatric scales were used for the further clinical evaluation of our study participants. The brainstem raphe was discontinued in 33.8% of the patients. The substantia nigra (SN) hyperechogenicity was identified in 79.7%. The third and fourth ventricle enlargement had 79.7% and 45.9% of patients, respectively. A positive and statistically significant correlation was found between SN hyperechogenicity with dystonia (p < 0.01), rigidity and dyskinesia (p < 0.05). The higher SARA total score is statistically significantly correlated with the larger diameter of the III (r = 0.373; p = 0.001) and IV ventricles (r = 0.324; p = 0.005). In such patients, the echogenicity of substantia nigra has been linked to extrapyramidal signs, and raphe discontinuity to depression. Furthermore, ataxia and its clinical subtypes have positively correlated with the IV ventricle diameter, indicating brain atrophy and brain mass reduction.

Clinical findings of hyperechoic substantia nigra in patients with Parkinson's disease.

This study aims to analyse hyperechoic substantia nigra (HSN) characteristics and the correlation of HSN with clinical features and blood biomarkers in patients with Parkinson's disease (PD). Transcranial sonography (TCS) evaluations of the substantia nigra (SN) were performed in 40 healthy controls and 71 patients with PD, including patients with SN hyperechogenicity (SN+) and those with normal SN echogenicity (SN-). Evaluation of motor and non-motor symptoms was assessed by a series of rating scales. The uricase method was used to determine serum uric acid (UA) levels, and enzyme-linked immunosorbent assay (ELISA) was used to measure plasma interleukin (IL)-1β levels. TCS showed 92.50% specificity and 61.97% sensitivity in differentiating PD patients from controls. The area of SN+ contralateral to the side of initial motor symptoms (SNcontra ) was larger than that ipsilateral to the side of initial motor symptoms (SNipsi ). The PDSN+ group had lower Argentine Hyposmia Rating Scale (AHRS) scores and UA levels than the PDSN- group. Binary logistic regression analysis revealed that AHRS scores and UA levels could be independent predictors for HSN. The larger SN echogenic area (SNL ) sizes positively correlated with plasma IL-1β levels in PD patients with SN+. The present study provides further evidence of the potential of SN echogenicity as an imaging biomarker for PD diagnosis. PD patients with HSN have more severe non-motor symptoms of hyposmia. HSN in PD patients is related to the mechanism of abnormal iron metabolism and microglial activation.

Transcranial sonography in neurodegeneration with brain iron accumulation disorders

BackgroundTranscranial Sonography is a non-invasive technique that has been used as a diagnostic tool for a variety of neurodegenerative disorders. However, the utility and potential application of this technique in NBIA disorders is scarce and inconclusive. MethodsIn this cross-sectional retrospective case-control study, the echogenicity of Substantia Nigra (SN), Lentiform Nucleus (LN), and Diameter of the Third Ventricle (DTV) were assessed by TCS in genetically confirmed NBIA patients referring to the movement disorder clinic. The normal echogenicity area of SN was defined based on the 90th percentile of an age-and-gender-matched control group. NBIA patients underwent neurologic examination at each visit, but their brain magnetic resonance imaging and demographics were extracted from electronic records. ResultsThirty-five NBIA patients of four subtypes with a mean disease duration of 10.54 years and 35 controls were enrolled. The normally defined SN echogenicity in controls was 0.23 cm2. DTV and SN echogenicity areas were significantly higher in patients compared to the controls (P = 0.002 and < 0.001, respectively). Around 85% and 63% of the patients showed LN and SN hyperechogenicity at least on one side, respectively. Disease duration was positively correlated with DTV (r = 0.422, p = 0.015). Cases with Pantothenate Kinase Associated Neurodegeneration (n = 23) also had significantly higher DTV and SN echogenicity area compared to the controls. ConclusionDespite most NBIA patients displayed increased DVT and higher SN and LN hyperechogenicity than healthy controls, the discriminatory role of TCS on different NBIA subtypes remains to be determined.

Substantia Nigra Echogenicity Signal Correlated with Clinical Features in Patients with Parkinson's Disease in Xinjiang.

Transcranial sonography (TCS) is a noninvasive test that can reveal structural changes in the substantia nigra (SN) in Parkinson's disease (PD). The purpose of this study was to investigate the relationship between SN signatures and clinical features in PD patients in a multiethnic region of China. A total of 147 patients with PD were included in the study, and all of whom had underwent a TCS examination. Clinical information was collected from PD patients, and motor and nonmotor symptoms were assessed using assessment scales. There were differences in the substantia nigra hyperechogenicity (SNH) area between age of onset, visual hallucinations (VH), and UPDRS3.0 II scores (P < 0.05), patients with late onset PD had a greater SNH area than early onset (0.326 ± 0.352 vs. 0.171 ± 0.194), and PD patients presenting with VH had a greater SNH area than those without hallucinations (0.508 ± 0.670 vs. 0.278 ± 0.659), and further multifactorial analysis showed that a high SNH area was an independent risk factor for development of VH. The area under the ROC curve for predicting VH from the SNH area in PD patients was 0.609 (95% CI: 0.444-0.774). There was a positive correlation between the SNH area and UPDRS3.0-II scores, but further multifactorial analysis showed that SNH was not an independent predictor of the UPDRS3.0-II score. A high SNH area is an independent risk factor for development of VH, there is a positive correlation between the SNH area and UPDRS3.0 II score, and TCS has guiding significance in predicting clinical VH symptoms and activities of daily living in PD patients.

Echogenicity and size of substantia nigra on transcranial sonography (TCS) in patients with attention-deficit/hyperactivity disorder and healthy children aged 6–12 years: a comparative study

BackgroundAttention-deficit/hyperactivity disorder (ADHD) is one of the most common neuropsychiatric disorders. Children with ADHD may experience significant functional problems, such as academic concerns at school, poor interpersonal relationships and low self-esteem. Current models of ADHD suggest that it is associated with functional impairments in dopamine and norepinephrine systems. The substantia nigra in the midbrain produces the largest amount of dopamine in the brain. The present study was conducted using TCS to compare the size and echogenicity of substantia nigra between ADHD and healthy children.ResultsThis cross-sectional, analytical study was conducted on 68 (34 ADHD and 34 healthy individuals) aged 6–12 years. Based on the results obtained, the hyper-echogenicity of SN in control and ADHD groups were 33.3% and 66.7% (P < 0.001) and hypo-echogenicity of thalamic nuclei were 55.2% and 44.8% (P < 0.05), respectively. Interestingly, the TCS results of healthy children with a positive family history of ADHD were similar to results for patients with the disorder.ConclusionsThe echogenicity of Substantia nigra and thalamus nucleus among children and adolescents with ADHD is significantly higher from that in healthy children.

Motor cortical excitability and pre-supplementary motor area neurochemistry in healthy adults with substantia nigra hyperechogenicity.

Substantia nigra (SN) hyperechogenicity, viewed with transcranial ultrasound, is a risk marker for Parkinson's disease. We hypothesized that SN hyperechogenicity in healthy adults aged 50-70 years is associated with reduced short-interval intracortical inhibition in primary motor cortex, and that the reduced intracortical inhibition is associated with neurochemical markers of activity in the pre-supplementary motor area (pre-SMA). Short-interval intracortical inhibition and intracortical facilitation in primary motor cortex was assessed with paired-pulse transcranial magnetic stimulation in 23 healthy adults with normal (n=14; 61 ± 7yrs) or abnormally enlarged (hyperechogenic; n=9; 60 ± 6yrs) area of SN echogenicity. Thirteen of these participants (7 SN- and 6 SN+) also underwent brain magnetic resonance spectroscopy to investigate pre-SMA neurochemistry. There was no relationship between area of SN echogenicity and short-interval intracortical inhibition in the ipsilateral primary motor cortex. There was a significant positive relationship, however, between area of echogenicity in the right SN and the magnitude of intracortical facilitation in the right (ipsilateral) primary motor cortex (p=.005; multivariate regression), evidenced by the amplitude of the conditioned motor evoked potential (MEP) at the 10-12 ms interstimulus interval. This relationship was not present on the left side. Pre-SMA glutamate did not predict primary motor cortex inhibition or facilitation. The results suggest that SN hyperechogenicity in healthy older adults may be associated with changes in excitability of motor cortical circuitry. The results advance understanding of brain changes in healthy older adults at risk of Parkinson's disease.

Clinical Features in Parkinson's Disease Patients with Hyperechogenicity in Substantia Nigra: A Cross-Sectional Study.

BackgroundTranscranial ultrasound (TCS) can be used to reveal structural changes in the substantia nigra (SN) and is a potential tool for the early diagnosis of Parkinson’s disease (PD). This study aimed to explore the relationship between substantia nigra hyperechogenicity (SNH) and the clinical features of PD patients.MethodsA total of 96 PD patients were included in our study. All patients were detected by TCS and divided into two groups: PD patients with SNH (PDSN+) and those with normal SN echogenicity (PDSN-). The Unified Parkinson’s Disease Rating Scale (UPDRS) and the Hoehn & Yahr stage were used to assess the extent of disease-related disability of the PD patients. Non-motor symptoms were evaluated by using several scales. The instrumented stand and walk test was performed on all subjects, and gait data were gathered using a JiBuEn gait analysis system.ResultsSeventy-five PD patients were successfully assessed by TCS. We found that SNH was associated with a higher UPDRS II scores (p = 0.028). In addition, compared with PDSN- group, the PDSN+ group exhibited more severe gait impairment, including increased variability in stride length (p = 0.042), decreased heel strike angle (p = 0.017), decreased range of motion of hip joints (p = 0.031), and a more asymmetrical walking pattern (p = 0.028).ConclusionOur study demonstrated that SNH significantly correlated with activities of daily living and gait impairment in Chinese patients with PD, suggesting the formation of SNH might be a dynamic biomarker reflecting disease severity.

Differentiating Parkinson's Disease from Essential Tremor Using Transcranial Sonography: A Systematic Review and Meta-Analysis.

Background:Essential tremor (ET) and the tremor of Parkinson’s disease (PD) are the most common tremors encountered in clinical practice. Especially in early disease stages, discrimination between the tremors of ET and PD can be challenging.Objective:The aim of this study was to evaluate the diagnostic accuracy of transcranial sonography (TCS) of the substantia nigra echogenicity for differential diagnosis of PD versus ET.Methods:A systematic PubMed search identified 512 studies. Sensitivity and specificity of substantia nigra hyperechogenicity was estimated. Data synthesis was carried applying a random effects bivariate binomial model. To assess study quality and risk of bias, the QUADAS-2 tool was used.Results:Eighteen studies were suitable for analysis including 1,264 PD and 824 ET patients. The meta analysis showed a pooled sensitivity and specificity for TCS in the differential diagnosis of PD versus ET of 84.6% (95% CI, 79.4–88.6%) and 83.9% (95% CI, 78.4–88.2%), respectively. Furthermore, we found nearly similar results in sensitivity and specificity comparing TCS and DaTSCAN in a subgroup-analysis of three studies using both diagnostic tools including 107 patients with PD and 62 patients with ET. The QUADAS-2 toolbox revealed a high risk of bias regarding the methodological quality of patient selection.Conclusion:Substantia nigra hyperechogenicity yield high diagnostic accuracy for the discrimination of PD from ET. TCS is a low cost, widely available, non-invasive marker without radiation Therefore, a diagnostic algorithm based on presence or absence of substantia nigra hyperechogenicity is highly warranted.

Correlation between systemic iron parameters and substantia nigra iron stores in restless legs syndrome

ObjectiveTo evaluate the relation between systemic iron parameters (SIP) and substantia nigra (SN) iron deposits, as assessed by transcranial sonography (TCS) in restless legs syndrome (RLS). MethodsWe conducted a cross-sectional study in RLS patients, from whom blood samples with SIP were obtained, consisting of total iron-binding capacity (TIBC), serum ferritin, hemoglobin, transferrin saturation (TSAT), serum iron, and serum transferrin. TCS was performed over the SN, and the substantia nigra echogenicity index (SNEI) was determined according to established methods. Symptom severity was evaluated using the international restless legs scale (IRLS). A Spearman correlation was performed. ResultsA total of 167 patients were studied. Correlations between SNEI and SIP were as follows: serum ferritin (R = 0.0422; n.s.), TSAT (R = 0.0883; n.s.), TIBC (R = −0.1091; n.s.), serum transferrin (R = −0.0420; n.s.), hemoglobin (R = 0.0185; n.s.), serum iron (R = 0.0389; n.s.). No correlation was found with age and IRLS (R = 0.1375; n.s. and R = 0.0880, n.s., respectively). ConclusionsSIP are not correlated with SN iron content in RLS, quantified by means of TCS. TCS of the SN might be a more valid estimate and could be useful in the evaluation of RLS patients.

Sonographic hypoechogenicity of brainstem raphe nucleus is correlated with electroencephalographic spike frequency in patients with epilepsy

BackgroundBrainstem raphe nucleus (BRN) hypoechogenicity in transcranial sonography (TCS) has been demonstrated in patients with major depression, possibly representing a sonographic manifestation of serotonergic dysfunction in depression. Most patients with epilepsy with comorbid depression exhibit hypoechogenic BRN in TCS. However, the role of BRN in the pathogenesis of epilepsy is unclear. This study aimed to evaluate the correlation of BRN echogenicity with epilepsy itself, and the echogenicity of other midbrain structures and the size of lateral ventricle (LV) will also be evaluated in patients with epilepsy. MethodsThirty-six patients with epilepsy without depression and 37 healthy controls were recruited. Sonographic echogenicity of BRN, caudate nucleus (CN), lentiform nucleus (LN), substantia nigra (SN), and the width of frontal horns of the lateral ventricles (LV) and the third ventricle (TV) were evaluated with TCS. The frequency of interictal epileptiform discharges (IEDs) was assessed with ambulatory electroencephalogram (AEEG). ResultsHypoechogenicity of BRN was depicted in 36.1% of patients with epilepsy and 18.9% of controls, showing no significant difference. Patients with epilepsy with BRN hypoechogenicity had higher epileptic discharge index (EDI) than those with normal BRN echogenecity. Especially, higher EDI in patients with BRN hypoechogenicity was observed during the sleep period but not during awake period. The width of TV was significantly larger in patients with epilepsy than that in controls. We did not find any difference between patients with epilepsy and controls in the echogenicity of CN, LN, and SN, as well as in the width of frontal horn of LV. ConclusionsHypoechogenic BRN is correlated with a high frequency of epileptic discharges in electroencephalogram (EEG), especially during sleep period but not during awake period, indicating that BRN alterations may play a potential role in the pathogenesis of epilepsy in association with sleep cycle.

Measurement of the adult human midbrain with transcranial ultrasound.

BackgroundTranscranial sonography is increasingly used to aid clinical diagnoses of movement disorders, for example, to identify an enlarged area of substantia nigra echogenicity in patients with Parkinson’s disease.ObjectiveThe current study investigated characteristics of the midbrain at the anatomical plane for quantification of substantia nigra echogenicity. METHODS: Area of substantia nigra echogenicity, cross-sectional area of the midbrain, and interpeduncular angle were quantified in two groups of adults aged 18–50 years: 47 healthy non-drug-using controls (control group) and 22 individuals with a history of methamphetamine use (methamphetamine group), a cohort with a high prevalence of enlarged substantia nigra echogenicity and thus risk of Parkinson’s disease.ResultsIn the control group, cross-sectional area of the midbrain (4.47±0.44 cm2) and interpeduncular angle were unaffected by age, sex, or image acquisition side. In the methamphetamine group, cross-sectional midbrain area (4.72±0.60 cm2) and area of substantia nigra echogenicity were enlarged compared to the control group, and the enlargement was sex-dependent (larger in males than females). Whole midbrain area and interpeduncular angle were found to be weak predictors of area of substantia nigra echogenicity after accounting for group and sex.ConclusionsHistory of methamphetamine use is associated with an enlarged midbrain and area of substantia nigra echogenicity, and the abnormality is more pronounced in males than females. Thus, males may be more susceptible to methamphetamine-induced changes to the brainstem, and risk of Parkinson’s disease, than females.

Segmentation of induced substantia nigra from transcranial ultrasound images using deep convolutional neural network

Parkinson's disease (PD) is an age-related neurodegenerative disorder, whose early diagnosis is challenging. PD neuropathology is characterized by a selective loss of dopaminergic neurons in the substantia nigra (SN). The echogenicity of SN is considered as an important biomarker for diagnosing PD. Since Ultrasound is well suited for measuring echogenicity, transcranial ultrasound images (TCUI) are used to diagnose PD and have become an industrial standard. But, ultrasound images usually have low resolution and are noisy compared to other medical imaging modalities. Thus, this whole method relies on the experience of the clinician to identify SN from the TCUI. To automate the process, we propose a deep convolutional neural network based on the U-Net architecture with a weighted binary cross-entropy (WBCE) loss function to do semantic segmentation of SN from the TCUI obtained. To alleviate the negative effects caused by noisy images, third-order Volterra filter is used for pre-processing. Furthermore, we compare the convergence rate of WBCE with standard BCE and other standard loss metrics, where WBCE outperforms others. We then, compare the results of the proposed U-Net architecture with a DenseNet based architecture and a U-Net and DenseNet based hybrid architecture, where we obtain better accuracy with the proposed architecture.

Substantia nigra echogenicity is associated with serum ferritin, gender and iron-related genes in Parkinson\u2019s disease

Substantia nigra (SN) hyperechogenicity is present in most Parkinson’s disease (PD) cases but is occasionally absent in some. To date, age, gender, disease severity, and other factors have been reported to be associated with SN hyperechogenicity in PD. Previous studies have discovered that excess iron deposition in the SN underlies its hyperechogenicity in PD, which may also indicate the involvement of genes associated with iron metabolism in hyperechogenicity. The objective of our study is to explore the potential associations between variants in iron metabolism-associated genes and SN echogenicity in Han Chinese PD. Demographic profiles, clinical data, SN echogenicity and genotypes were obtained from 221 Han Chinese PD individuals with a sufficient bone window. Serum ferritin levels were quantified in 92 of these individuals by immunochemical assay. We then compared factors between PD individuals with SN hyperechogenicity and those with SN hypoechogenicity to identify factors that predispose to SN hyperechogenicity. Of our 221 participants, 122 (55.2%) displayed SN hyperechogenicity, and 99 (44.8%) displayed SN hypoechogenicity. Gender and serum ferritin levels were found to be associated with SN hyperechogenicity. In total, 14 genes were included in the sequencing part. After data processing, 34 common single nucleotide polymorphisms were included in our further analyses. In our data, we also found a significantly higher frequency of PANK2 rs3737084 (genotype: OR = 2.07, P = 0.013; allele: OR = 2.51, P = 0.002) in the SN hyperechogenic group and a higher frequency of PLA2G6 rs731821 (genotype: OR = 0.45, P = 0.016; allele: OR = 0.44, P = 0.011) in the SN hypoechogenic group. However, neither of the two variants was found to be correlated with serum ferritin. This study demonstrated that genetic factors, serum ferritin level, and gender may explain the interindividual variability in SN echogenicity in PD. This is an explorative study, and further replication is warranted in larger samples and different populations.

Left-hemispheric predominance of nigrostriatal deficit in isolated REM sleep behavior disorder.

Unilateral onset of parkinsonism due to nigrostriatal damage of the contralateral hemisphere is frequent in Parkinson disease (PD). There is evidence for a left-hemispheric bias of motor asymmetry in right-handed patients with PD indicating a hemispheric dominance. Isolated REM sleep behavior disorder (IRBD) constitutes the prodromal stage of PD and other synucleinopathies. To test the hypothesis that right-handed patients with IRBD exhibit left-hemispheric predominance of subclinical nigrostriatal dysfunction, we evaluated this aspect using neuroimaging instruments. In 167 right-handed patients with IRBD without parkinsonism, we evaluated in each hemisphere the integrity of the striatal dopaminergic terminals by dopamine transporter (DAT)-SPECT and the substantia nigra echogenicity by transcranial sonography. DAT-SPECT showed lower specific binding ratio (SBR) in the left striatum and left caudate nucleus than in the right striatum and right caudate nucleus. The percentage of patients with lower SBR was greater in the left striatum and left caudate nucleus than in the right striatum and right caudate nucleus. In those who developed a synucleinopathy in <5 years from DAT-SPECT, there was a lower SBR in the left putamen and left caudate nucleus than in the right putamen and right caudate nucleus. Substantia nigra echogenic size was greater in the left than in the right side in patients with hyperechogenicity and among individuals who phenoconverted in <5 years from transcranial sonography. Right-handed patients with IRBD exhibit left-hemispheric predominance of subclinical nigrostriatal dysfunction. In premotor PD, the neurodegenerative process begins asymmetrically, initially impairing the nigrostriatal system of the dominant hemisphere.

Transcranial sonography imaging of brainstem raphe, substantia nigra and cerebral ventricles in patients with geriatric depression.

Geriatric depression is a special condition associated with a chronic course, treatment resistance and vascular processes. However, its neurobiology has not been fully elucidated. There is no study in geriatric depression evaluating deep brain structures with transcranial sonography (TCS) which is a low-cost, non-invasive and practical tool. The present study aimed to evaluate the changes in the echogenicity of brainstem raphe (BR), substantia nigra (SN) and ventricular diameters by TCS in association with cognitive dysfunctions in patients with geriatric depression. Echogenicity of BR and SN were assessed and transverse diameters of the third ventricle and frontal horns of the lateral ventricles were measured by TCS in 34 patients with DSM-5 major depression and 31 healthy volunteers aged 60 and older. Cognitive functions were evaluated by using Mini Mental State Examination, Montreal Cognitive Assessment Tool, Clock Drawing Test and Subjective Memory Complaints Questionnaire. Although depressed patients had more subjective memory complaints than controls, they had similar cognitive performances. Reduced echogenicity (interrupted/invisible echogenic line) of BR was found to be significantly higher and the ventricular diameters were larger in the depressed group. There was no difference between the groups in terms of SN echogenicity. There was no correlation between ventricular diameters and depression severity or cognitive functions. Results of the present study are important in terms of pointing out neurobiological changes related to geriatric depression which are in parallel with the results of the studies in younger patients with depression. However, long-term follow-up studies are required for accurate differentiation of neurocognitive disorders.

Comparative study of the substantia nigra echogenicity and 123I-Ioflupane SPECT in patients with synucleinopathies with and without REM sleep behavior disorder

ObjectivesHyperechogenicity of the substantia nigra (SN) and abnormal dopamine transporter-single-photon emission computed tomography (DAT-SPECT) are biomarkers commonly used in the assessment of prodromal synucleinopathy. Our goals were as follows: (1) to compare echogenicity of SN in idiopathic rapid eye movement (REM) behavior disorder (iRBD), Parkinson's disease (PD) without RBD (PD-noRBD), PD with RBD (PD + RBD), and control subjects; and (2) to examine association between SN degeneration assessed by DAT-SPECT and SN echogenicity. Patients/methodsA total of 61 subjects with confirmed iRBD were examined using Movement Disorders Society-unified PD rating scale (MDS-UPDRS), TCS (transcranial sonography) and DAT-SPECT. The results were compared with 44 patients with PD (25% PD + RBD) and with 120 age-matched healthy subjects. Results and conclusionThe abnormal SN area was found in 75.5% PD, 23% iRBD and 7.3% controls. Median SN echogenicity area in PD (0.27 ± 0.22 cm2) was higher compared to iRBD (0.07 ± 0.07 cm2; p < 0.0001) and controls (0.05 ± 0.03 cm2; p < 0.0001). SN echogenicity in PD + RBD was not significantly different from PD-noRBD (0.30 vs. 0.22, p = 0.15).Abnormal DAT-SPECT was found in 16 iRBD (25.4%) and 44 PD subjects (100%). No correlation between the larger SN area and corresponding putaminal binding index was found in iRBD (r = −0.13, p = 0.29), nor in PD (r = −0.19, p = 0.22).The results of our study showed that: (1) SN echogenicity area in iRBD was higher compared to controls, but the hyperechogenicity was present only in a minority of iRBD patients; (2) SN echogenicity and DAT-SPECT binding index did not correlate in either group; and (3) SN echogenicity does not differ between PD with/without RBD.

Third Ventricle Width Assessed by Transcranial Sonography as Predictor of Long-Term Cognitive Impairment

Non-invasive biomarkers of cognitive impairment are needed. We aim to evaluate transcranial sonographic markers as predictors of cognitive impairment in a prospective cohort. To study the changes in the third ventricle diameter and the SN echogenicity between the baseline and the control visit, as well as its association with cognitive performance and the diagnosis of cognitive impairment in a prospective cohort. From the longitudinal population-based Asymptomatic Intracranial Atherosclerosis Study, we selected those subjects that received a complete transcranial sonography (TCS) and extensive cognitive testing, both at baseline and follow-up. We evaluated third ventricle (IIIv) width, echogenicity of substantia nigra (SN), and temporal changes of these parameters. We included 289 participants with a median follow-up time of 7.16 years. Those subjects who developed cognitive decline (n = 23, 7.96%) had a larger IIIv at baseline than those who did not (0.54±0.14 cm versus 0.41±0.15 cm; p = 0.001). A cut-off point of 0.465 cm for the IIIv width was identified as an independent predictor of long-term cognitive impairment after adjustment for age, gender, educational level, and vascular risk score. Change in IIIv diameter after follow-up was not associated with diagnosis of cognitive impairment. The area of SN and the presence of hyperechogenicity of the SN remained stable over time and was not associated with the diagnosis of cognitive impairment. IIIv width assessed by TCS emerged as an independent predictor of long-term cognitive impairment.

Digitized Image Analysis of Insula Echogenicity Detected by TCS-MR Fusion Imaging in Wilson's and Early-Onset Parkinson's Diseases

Transcranial sonography (TCS) can reveal pathology in brain structures including insula. This study compared insula echogenicity among 22 patients with Wilson's disease (WD), 21 patients with early-onset Parkinson's disease (EO-PD) and 24 healthy patients. Echogenicity of predefined brain structures (insula, lentiform nucleus, caudate nucleus, substantia nigra and raphe nuclei) was evaluated using digitized analysis of TCS fusion imaging with magnetic resonance. Cortical, subcortical and cerebellar atrophy and ventricle diameters were determined from magnetic resonance images. The mean echogenicity index of insula did not differ between males and females (p = 0.92), but the echogenicity of insula was higher in patients with WD than in patients with EO-PD and healthy patients (p < 0.05). The substantia nigra echogenicity was higher in patients with EO-PD, and lentiform nucleus echogenicity was higher in patients with WD (p < 0.05). The echogenicity of insula correlated with lentiform nucleus echogenicity (r = 0.75) but not with age (r = –0.14), disease duration (r = –0.36), symptom severity (r = 0.28), cortical (r = 0.11) nor subcortical (r = 0.05) atrophy.

Increased substantia nigra echogenicity correlated with visual hallucinations in Parkinson\u2019s disease: a Chinese population-based study

As a noninvasive technique, transcranial sonography (TCS) of substantia nigra (SN) has gradually showed its effectiveness not only in diagnosis but also in understanding clinical features of Parkinson’s Disease (PD). This study aimed to further evaluate TCS for clinical diagnosis of PD, and to explore the association between sonographic manifestations and visual hallucinations (VH). A total of 226 subjects including 141 PD patients and 85 controls were recruited. All participants received TCS. A series of rating scales to evaluate motor and non-motor symptoms were performed in PD patients. Results showed that 172 subjects were successfully assessed by TCS. The area of SN was greater in PD patients than that in controls (P < 0.001). As receiver-operating characteristic (ROC) curve analysis showed, the best cutoff value for the larger SN echogenicity size was 23.5 mm2 (sensitivity 70.3%, specificity 77.0%). Patients with VH had larger SN area (P = 0.019), as well as higher Non-Motor Symptoms Scale (NMSS) scores (P = 0.018). Moreover, binary logistic regression analysis indicated that SN hyperechogenicity (odds ratio = 4.227, P = 0.012) and NMSS scores (odds ratio = 0.027, P = 0.042) could be the independent predictors for VH. In conclusion, TCS can be used as an auxiliary diagnostic tool for Parkinson’s disease. Increased SN echogenicity is correlated with VH in Parkinson’s disease, possibly because the brain stem is involved in the mechanism in the onset of VH. Further studies are needed to confirm these findings.

A transcranial sonography study of brainstem and its association with depression in idiopathic generalized epilepsy with tonic–clonic seizures

Brainstem raphe (BR) hypoechogenicity in transcranial sonography (TCS) has been depicted in patients with depression. But, up to date, the association of BR alterations in TCS with depression in patients with epilepsy has never been reported. This study was to investigate the possible role of BR examination via TCS in patients with idiopathic generalized epilepsy with tonic–clonic seizures (IGE-TCS) and depression. Forty-six patients with IGE-TCS and 45 healthy controls were recruited. Echogenicity of the caudate nuclei (CN), lentiform nuclei (LN), substantia nigra (SN), and BR and widths of the lateral ventricle (LV) frontal horns and the third ventricle (TV) were assessed via TCS. The determination of depression was based on the criteria of the Diagnostic and Statistical Manual of Mental Disorders IV (DSM-IV), and depression severity measured by Chinese version Neurological Disorders Depression Inventory for Epilepsy (C-NDDI-E) and Beck Depression Inventory—II (BDI-II). The width of TV in patients with epilepsy was found significantly larger than that in healthy controls (p = 0.001), but there was no significant difference in TV width between patients with IGE-TCS with and without depression. There were no significant differences between patients with IGE-TCS and healthy controls in LV frontal horn width, as well as in SN, CN, LN, and BR echogenicity. Here, it seems that patients with IGE-TCS were detected with smaller SN echogenic area compared with controls though they had no statistical significance. Patients with IGE-TCS with hypoechogenic BR had significantly higher C-NDDI-E and BDI-II scores than those with normal BR signal, and most patients with IGE-TCS with depression exhibited hypoechogenic BR, but few patients with IGE-TCS without depression exhibited hypoechogenic BR. In conclusion, BR echogenic signal alterations in TCS can be a biomarker for depression in epilepsy, but it might not be associated with epilepsy itself. The alterations of SN echogenic area and TV width in TCS may reflect a potential role of SN and diencephalon structure in the pathogenesis of epilepsy, which needs to be further elucidated.