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Ruckenmarklasionen ziehen haufig schwere klinisch-neurologische Ausfalle nach sich. Diese ausern sich durch spezifische Symptome und Syndrome. Zur Einordnung ist eine grundliche Kenntnis der spinalen Neuroanatomie wichtig. Spinale Anatomie, Untersuchungsablauf und klassische spinale Syndrome werden dargestellt. Wichtige spinale Syndrome sind das Hinterstrangsyndrom, Vorderseitenstrangsyndrom, Pyramidenbahnsyndrom, zentromedullare Syndrom, Querschnitt- und Brown-Sequard-Syndrom sowie kombinierte Syndrome. Die klinische Untersuchung erlaubt die Erfassung und anatomische Einordnung spinaler Syndrome und gezielte Untersuchung des Ruckenmarks mittels Zusatzdiagnostik.

Background The prehospital identification of stroke patients with large-vessel occlusion (LVO), that should be immediately transported to a thrombectomy capable centre is an unsolved problem. Our aim was to determine whether implementation of a state-wide standard operating procedure (SOP) using the Los Angeles Motor Scale (LAMS) is feasible and enables correct triage of stroke patients to hospitals offering (comprehensive stroke centres, CSCs) or not offering (primary stroke centres, PSCs) thrombectomy. Methods Prospective study involving all patients with suspected acute stroke treated in a 4-month period in a state-wide network of all stroke-treating hospitals (eight PSCs and two CSCs). Primary endpoint was accuracy of the triage SOP in correctly transferring patients to CSCs or PSCs. Additional endpoints included the number of secondary transfers, the accuracy of the LAMS for detection of LVO, apart from stroke management metrics. Results In 1123 patients, use of a triage SOP based on the LAMS allowed triage decisions according to LVO status with a sensitivity of 69.2% (95% confidence interval (95%-CI): 59.0–79.5%) and a specificity of 84.9% (95%-CI: 82.6–87.3%). This was more favourable than the conventional approach of transferring every patient to the nearest stroke-treating hospital, as determined by geocoding for each patient (sensitivity, 17.9% (95%-CI: 9.4–26.5%); specificity, 100% (95%-CI: 100–100%)). Secondary transfers were required for 14 of the 78 (17.9%) LVO patients. Regarding the score itself, LAMS detected LVO with a sensitivity of 67.5% (95%-CI: 57.1–78.0%) and a specificity of 83.5% (95%-CI: 81.0–86.0%). Conclusions State-wide implementation of a triage SOP requesting use of the LAMS tool is feasible and improves triage decision-making in acute stroke regarding the most appropriate target hospital.

Spinal cord injuries are frequently associated with severe clinical-neurological deficits. These are evident with specific symptoms and syndromes. Hereby, a thorough knowledge of spinal neuroanatomy is essential.Spinal anatomy, examination procedures and classical spinal syndromes are presented.Important spinal syndromes comprise the dorsal cord syndrome, spinothalamic tract syndrome, pyramidal tract syndrome, central cord syndrome, transversal and Brown-Séquard syndrome as well as combined syndromes.Clinical examination allows assessment and anatomical classification of spinal syndromes and targeted examination of the spinal cord using additional diagnostic methods.HINTERGRUND: Rückenmarkläsionen ziehen häufig schwere klinisch-neurologische Ausfälle nach sich. Diese äußern sich durch spezifische Symptome und Syndrome. Zur Einordnung ist eine gründliche Kenntnis der spinalen Neuroanatomie wichtig.Spinale Anatomie, Untersuchungsablauf und klassische spinale Syndrome werden dargestellt.Wichtige spinale Syndrome sind das Hinterstrangsyndrom, Vorderseitenstrangsyndrom, Pyramidenbahnsyndrom, zentromedulläre Syndrom, Querschnitt- und Brown-Séquard-Syndrom sowie kombinierte Syndrome.Die klinische Untersuchung erlaubt die Erfassung und anatomische Einordnung spinaler Syndrome und gezielte Untersuchung des Rückenmarks mittels Zusatzdiagnostik.

Cardiac dysautonomia is a potentially life-threatening complication of Guillain-Barré syndrome (GBS). Proper and prompt recognition of patients at risk and subsequent intensive care unit (ICU) monitoring are mandatory to prevent fatal outcome. Eyeball pressure testing (EP) has been suggested as an easy applicable bedside test for vagal overreactivity in GBS and thus identifying patients at risk. Yet, there is only sparse follow-up data concerning the course of EP findings in GBS. We report a 25 years-old male patient with GBS who underwent consecutive EP (n = 11) during his ICU stay over a period of 11 weeks. The series of tests performed in this patient (and corresponding clinical events) show that EP data might represent an approximation of vagal dysfunction and vagal recovery in GBS. Interestingly, we observed a much longer duration of pathological EP compared to a previous report. The tenacious cardiac dysautonomia in this patient necessitated long-term application of a transvenous temporary pacemaker.

To assess the value of optic nerve sheath diameter (ONSD) measurements at different time points to predict the malignant evolution in middle cerebral artery (MCA) infarction and to investigate the relationship between ONSD and infarct volume on follow-up computed tomography (CT). In a single-center prospective observational study, we recruited patients with MCA infarction and age- and sex-matched controls. Clinical characteristics including NationaI Institutes of Health Stroke Scale (NIHSS) and ONSD measurement were assessed during the first five days after symptom onset. Volumetric analysis of the infarction was performed by a neuroradiologist, who was blinded to results of ONSD measurement and clinical examinations, based on CT scans. We enrolled 29 patients with MCA infarction, including 10 with malignant MCA (mMCA) infarction and 14 controls. Mean ONSD on admission was already larger in patients who had developed an mMCA (5.99 ± 0.32 mm) compared to patients with MCA infarction (4.98 ± 0.53 mm; P = 0.003), and to control patients (4.57 ± 0.29 mm; P

Background and purpose Optic nerve sheath diameter (ONSD) is used for the estimation of intracranial pressure (ICP). But there are still doubts about the quality of the images and the lateral resolution. Our aim is to investigate the system suitability and best lateral resolution of different ultrasound systems for acoustic ONSD measurement. Methods First, we calculated the theoretically lateral imaging resolution at increasing frequencies: 6.6, 10, and 15 MHz using two different ultrasound systems. Second, we created two phantoms consisting of copper wires or polyvinylchloride (PVC) strips and tested the best lateral resolution at different frequencies with the two ultrasound systems. Using the same ultrasound systems, we evaluated the anatomy of optic nerve at increasing transmission frequencies. Finally, the two probes were tested in two patients with different neurological conditions affected by an increase of ICP. Results Theoretical resolutions were .63, .43, and .41 mm, respectively, with a frequency of 6.6, 10, and 15 MHz. We found a similar lateral resolution in both phantoms: copper wire; .56 mm at 6.6 MHz, .46 mm at 10 MHz, and .44 mm at 15 MHz; and PVC strips .6 mm at 6.6 MHz, .47 mm at 10 MHz, and .40 mm at 15 MHz in accordance with experimental resolution. The ONSD thickening could be clearly displayed at frequencies higher than 7.5 MHz using the two linear probes and the two patients with an increase of ICP showed thickening of ONSD. Conclusion According to our study, both systems are suitable for ultrasound OSND measurement.

Background and Purpose— A new generation of carotid artery stents that uses a second micromesh layer to reduce embolic events during carotid artery stenting has recently been introduced. The purpose of this study was to compare acute occlusion rates of these new dual-layer stents with those of single-layer stents in the setting of emergency carotid artery stenting with intracranial mechanical thrombectomy in acute ischemic stroke. Methods— Consecutive patients with acute tandem (intra- and extracranial) lesions of the anterior circulation who were endovascularly treated at our institution were identified from our registry of neuroendovascular interventions. Clinical, angiographic, and neuroimaging data were analyzed. End points included acute occlusions of the carotid stents (within 72 hours after stenting) and symptomatic intracerebral hemorrhage. Results— Forty-seven patients were included. Dual-layer stents (n=20) had a significantly higher rate of acute occlusions than single-layer stents (n=27; 45% versus 3.7%; P =0.001; odds ratio, 21.3; 95% confidence interval, 2.4–188.4). There were no significant differences in the rates of patients who had any antiplatelet or dual antiplatelet medication before admission, in the rates of postinterventional symptomatic intracerebral hemorrhage, the mean National Institutes of Health Stroke Scale scores at admission, or the modified Rankin Scale scores at discharge. Conclusions— The recently introduced dual-layer stents have a higher risk of acute occlusion compared with single-layer stents in the treatment of acute stroke.

Cognitive impairment and dementia are common in PD; however, no stable marker of cognitive dysfunction is available. Transcranial sonography can evaluate global and focal brain atrophy and has been widely used in the differential diagnosis of parkinsonism.225 consecutive PD patients were recruited in a two-center cross sectional study and underwent a standardized sonographic protocol assessing the third ventricle's width and substantia nigra hyperechogenicity. All subjects were evaluated with an extensive motor and cognitive battery.222 PD patients were included and classified as PD with normal cognition (PDNC; n = 130), mild cognitive impairment (PD-MCI; n = 61) and dementia (PDD; n = 31). Ventricular width correlated strongly with cognitive performance in all cognitive domains (p 0.001) while SN size did not. PDD patients had significantly wider ventricles than PD patients without dementia (p 0.001) while differences between PD-MCI and PDNC or PDD were less strong (p 0.05). There were no group differences in SN size. ROC analyses resulted in age-related cut-offs of third ventricular diameter for the prediction of PDD (6.0 and 7.5 mm for subjects and ≥70 years of age, respectively). These cut-offs significantly differentiated PDD from PDNC (p 0.001) and from all patients without dementia (PDNC + PD-MCI; p 0.001).The third ventricular diameter correlated with cognitive performance in all domains and was able to differentiate PDD patients from those without dementia. Longitudinal studies are warranted to evaluate whether transcranial sonography could identify PD patients at risk for a rapid cognitive decline.

Optic nerve sheath diameter (ONSD) is used for the estimation of intracranial pressure (ICP). But there are still doubts about the quality of the images and the lateral resolution. Our aim is to investigate the system suitability and best lateral resolution of different ultrasound systems for acoustic ONSD measurement.First, we calculated the theoretically lateral imaging resolution at increasing frequencies: 6.6, 10, and 15 MHz using two different ultrasound systems. Second, we created two phantoms consisting of copper wires or polyvinylchloride (PVC) strips and tested the best lateral resolution at different frequencies with the two ultrasound systems. Using the same ultrasound systems, we evaluated the anatomy of optic nerve at increasing transmission frequencies. Finally, the two probes were tested in two patients with different neurological conditions affected by an increase of ICP.Theoretical resolutions were .63, .43, and .41 mm, respectively, with a frequency of 6.6, 10, and 15 MHz. We found a similar lateral resolution in both phantoms: copper wire; .56 mm at 6.6 MHz, .46 mm at 10 MHz, and .44 mm at 15 MHz; and PVC strips .6 mm at 6.6 MHz, .47 mm at 10 MHz, and .40 mm at 15 MHz in accordance with experimental resolution. The ONSD thickening could be clearly displayed at frequencies higher than 7.5 MHz using the two linear probes and the two patients with an increase of ICP showed thickening of ONSD.According to our study, both systems are suitable for ultrasound OSND measurement.

Endovascular treatment represents a well-established therapeutic option in patients with vertebral artery origin (VAO) stenosis. Our aim was to determine which factors affect short- and long-term restenosis rates after endovascular VAO therapy. We conducted a single center analysis of 52 patients (36 men; age 64 ± 9.54 years) who underwent 55 endovascular procedures (27 balloon-assisted angioplasty [BAA], 28 stent-assisted angioplasty [SAA]) between 2005–2015. We collected data on patients clinical characteristics, medication and post-interventional follow-up visits. Overall, 15 of 55 vessels (27%) showed ≥70% restenosis at 1 year (short-term follow-up) and 18 after a mean follow-up of 52.9 ± 31.8 months (long-term). BMI ≥ 25 kg/m2 was associated with ≥70% restenosis in short-term (P = 0.014) and long-term (P = 0.003) follow-up. Other risk factors, namely, hypertension, ischemic heart disease, smoking, diabetes mellitus, hypercholesterolemia, atrial fibrillation, CRP (>5 mg/l) or pre-treatment antiplatelet administration, statin intake and platelet count, were not associated with restenosis risk in the entire cohort or in patients in the BAA group (all P > 0.05). BMI (P = 0.003) and ischemic heart disease (P = 0.041) were, in turn, associated with restenosis risk in the long-term follow-up in the SAA group. Patients undergoing BAA developed less frequently (P = 0.032) restenosis (18%) during long-term follow-up as compared to patients treated by stenting (46%). BMI ≥ 25 kg/m2 increases the odds for ≥70% restenosis of VAO while ischemic heart disease represents an additional risk factor in stented patients. Further studies are required to established therapeutic strategies lowering the restenosis rates in overweight individuals after VAO therapy, especially ones undergoing stenting.

Purpose: In animal studies intravitreal injection of tetrodotoxin (TTX) results in mfERG waveform changes similar to those observed in glaucoma. As TTX blocks amacrine as well as ganglion cells, there is still a question regarding the underlying cell population responsible for these changes in waveform. In an attempt to assess the contribution of the amacrine cells to these changes, a mfERG was obtained from patients with Parkinson's disease as some amacrine cells are mediated by dopamine, a substance lacking in Parkinson's. Methods: Eight patients with early Parkinson's disease underwent ophthalmologic examination, testing of contrast sensitivity and electrophysiological examination according to ISCEV standard at least 12h following their last medication with Dopamine. A slow stimulation mfERG was obtained with a stimulus base interval of 53.3ms and with a stimulus base interval of 106.6ms. During MF-ERG recordings 103 hexagons stimulated the central 50deg of the retina simultaneously and independently (m-sequence 213, Lmax: 200cd/m2, ~100% contrast). Results: Contrast sensitivity and ISCEV standard electrophysiological testing was unremarkable. When the mfERG was analyzed, only four patients had an adequate signal-to-noise ratio to allow further data analysis - one of whom was diagnosed with a multi system atrophy in retrospect. The first order response component was analyzed at a filter setting of 10-300Hz and at 100-300Hz (OPs) and compared to mfERGs of a control group. On average, in patients, the amplitude of N1P1 was slightly lower in the central and nasal response averages. When the three OPs at a latency of 72-89ms were analyzed in the 53.3ms base interval recording, the most marked difference in amplitude was observed in the superior nasal response average of the first OP. Here a mean amplitude of 1.3nV/deg2 in patients compared to a mean amplitude of 1.9nV/deg2 in the control group (P: 0.08). Discussion: In contrast to our previous findings in NTG, there was a consistent presence of three OPs. Under the stimulus conditions applied, we did not find an influence of dopaminergic amacrine cells on the mfERG in our patients with moderate stages of Parkinsion's. The difficulties in obtaining an adequate signal-to noise ratio due to e.g. muscle artifacts even in Parkinson patients of moderate disease stages render a success of mfERG recording in patients with more advanced stages unlikely. The question of the influence of dopaminergic amacrine cells on the mfERG could possibly be addressed using MPDT in animal research

Background: Decompressive hemicraniectomy (DHC) after space-occupying strokes among patients older than 60 years has been shown to reduce mortality rates but at the cost of severe disability. There is an ongoing debate about what could be considered an acceptable outcome for these patients. Data about retrospective consent to the procedure after lengthy time periods are lacking. Methods: This study included 79 consecutive patients who underwent DHC during a 7.75-year period. Surviving patients were assessed for functional and psychological outcome, quality of life (QoL) and retrospective consent for the procedure. Patients younger than 60 years were compared with older patients. Results: Of our 79 patients, 44 were younger than 60 years (median 50 years, interquartile range (IQR) 19-59 years) and 35 were older (median 68 years, interquartile range 60-87 years). The 30-day mortality rate was higher for the older group, but the difference was not statistically significant. Functional outcome was significantly better in the younger group: 31% of the patients in this group vs. 10% in the older group had a modified Rankin Scale score of 0-3 (p = 0.046). The mean National Institutes of Health Stroke Scale score was 17 ± 14 for the younger group and 29 ± 15 for the older group (p = 0.002). On the 36-Item Short Form Health Survey, with the exception of the item ‘General health', the older group reported higher values for all items, with statistically significant differences between the 2 groups on the items ‘Role limitation emotional' (p = 0.0007) and ‘Vitality' (p = 0.02). In the younger group, 29% of patients retrospectively declined consent for DHC opposed to 0% of patients in the older group (p = 0.07). Conclusions: Despite impaired functional outcome after DHC, indicators of QoL and retrospective consent are higher for patients older than 60 years over the long term. This finding should be taken into account by those who counsel patients and caregivers with regard to this serious procedure.

Principal Component Analysis (PCA) is a method to estimate the relation between data points. We used PCA to analyse movements of the upper and lower extremities during treadmill walking in healthy subjects and two groups of Parkinsonian patients. Healthy subjects ( n = 35) showed a typical pattern with high values of PC1 and low values in a descending order of PC2–PC4. Increase of speed resulted in a significant increase of PC1 and a significant decrease of the following PC's. In more severely affected patients ( n = 19, UPDRS > 20), PC1 was significantly decreased and PC2–PC4 were significantly increased compared to healthy subjects. Speed could be increased only within a small range without corresponding changes of the PC's. In less severely affected patients ( n = 17), significant differences of the PC's were only found with fast pace. Separate analysis of arms and legs revealed that these changes are only due to altered movements of the arm. Analysis of the pattern of PC's in response to changes of gait velocities reveal alterations even in less severely affected Parkinsonian patients. The changes of the PC's with higher gait velocities in healthy subjects are suggestive of an increase of intersegmental coordination. This is impaired even in less severely affected Parkinsonian patients.

In order to assess nigrostriatal function over time in healthy subjects with substantia nigra hyperechogenicity (SN+) believed to be at higher risk for Parkinson's disease (PD), ten healthy SN+ subjects underwent [(18)Fluoro] Dopa positron emission tomography (PET) twice-at baseline and after a mean of 7.3 years. Neurological examination took place at study inclusion followed by a structured telephone interview focusing on early Parkinsonian symptoms at the time point of second PET study and 3.5 years later. The [(18)Fluoro] Dopa uptake remained unchanged in eight of ten participants. In the other two subjects marked unilateral reduction of putaminal [(18)Fluoro] Dopa uptake ratios appeared over the time, followed by complaints of a clinically manifest PD in one. We suggest that the progressive pathological PET findings in 20 % and PD conversion in 10 % of our cohort may be in accordance with the presumed proportion of SN+ individuals eventually developing PD based on SN+ prevalence of 10 % within the healthy population, being ten times higher than PD prevalence in the age of over 60 years. Our findings hint towards a significance of SN+ indicating a high risk for PD in some extrapyramidally healthy SN+ individuals.

Objectives This study sought to assess the feasibility and safety of a recently described technique of mechanical recanalization with the help of a stent-like device. Background In the special group of acute stroke patients with an intracranial large vessel occlusion, intravenous tissue-type plasminogen activator on its own leads to a good clinical outcome (mRS ≤2) in only 15% to 25% of cases. The aforementioned technique of mechanical recanalization showed very promising clinical results. Methods Forty patients presenting within 6 h from stroke symptom onset were enrolled. Mechanical recanalization was performed using a Solitaire FR revascularization device. The primary endpoint of the study was the clinical outcome rated with the help of the modified Rankin Scale (mRS) after 90 days. Results Twenty-four patients (60%) showed a good clinical outcome (mRS ≤2) at 90 days. One symptomatic hemorrhage was detected on follow-up computed tomography. The death rate was 12.5% (5 patients). Successful recanalization (Thrombolysis In Cerebral Infarction score ≥2b) of the target vessel was achieved in 95% of the patients with a mean of 1.8 runs with the device. Conclusions The ReFlow (Mechanical Recanalization With Flow Restoration in Acute Ischemic Stroke) study shows that mechanical recanalization with flow restoration is highly effective in stroke patients with a large intracranial vessel occlusion presenting within 4.5 h after symptom onset. (Mechanical Recanalization With Flow Restoration in Acute Ischemic Stroke [ReFlow]; NCT01210729 )

Background SN hyperechogenicity (SN+), determined by transcranial sonography, has been proposed as a risk factor for Parkinson's disease (PD). Recently, we reported a 17.4-fold increased risk for PD in individuals with SN+ older than 50 years within 3 years. Methods This is the second follow-up of a prospective, longitudinal, three-center observational study after 5 years. Of the initial 1,847 at baseline PD-free participants 50 years or older, 1,271 underwent the 5-year reassessment. Results Within 5 years, 21 individuals developed incident PD. Participants with SN+ at baseline had a more than 20.6 times increased risk to develop PD in this time span than those without this echo feature. Conclusion We thus confirm our finding of the 3-year follow-up examination in a longer observation time and higher number of individuals with incident PD and suggest SN+ as an important risk marker for PD. © 2012 Movement Disorder Society

A hyperechogenicity of the (SN+) in transcranial sonography corroborates the diagnosis of idiopathic Parkinson's disease (iPD). Although it is thought to represent a biomarker of the disease that is independent of disease severity and progression, differing results have been reported describing a positive correlation of the size and advancing clinical stage. In 50 parkinsonian patients, transcranial ultrasound and clinical examination was performed twice with a mean time interval of 6.4 years. SN+ did not change in size significantly between the first and second examination, whereas clinical parkinsonian symptoms—as determined by the motor part of the UPDRS—significantly worsened (P < 0.001). We found a highly significant intraindividual correlation in SN+ sizes between both examinations (P < 0.001). The size of SN+ did not correlate with the UPDRS part III at the time of first or second ultrasound examination. Progression of motor symptoms between the first and second investigation did not correlate with the size of SN+ at baseline. Furthermore, even in the subgroup of patients with an interval of ≥8 years between examinations, there was no significant change in SN+ size. SN+ represents a largely stable biomarker in iPD and does not reflect disease progression. The size of SN+ does not predict the further course of the disease. © 2012 Movement Disorder Society

Much effort has been put in the identification of risk factors and pre-motor markers for Parkinson's disease (PD). In contrast to many of the pre-motor markers, SN hyperechogenicity (SN+) assessed by transcranial sonography (TCS) has been found to be conclusive for vulnerability for PD. In two centers in Germany 1204 individuals ≥50 years without the diagnosis of PD were recruited and the prevalence and relation of SN+ to a range of pre-motor markers was evaluated. SN+ was detected in 193 (16.0%) of 1204 subjects. Hyposmia (25.4%) was the most frequent sign in the cohort, followed by the occurrence of slight motor deficits. Male gender, positive family history of PD as possible risk factors and the pre-motor markers slight parkinsonian signs, one-sided reduced arm swing, and hyposmia were found to be significantly associated with SN+. The number of subjects who had more than one marker was significantly larger in the SN+ subgroup than in the non-hyperechogenic group (9.2% vs. 2.1%). Most of the discussed markers for PD seem to be unspecific with older age, but related to SN+. Co-occurrence of these markers is more probable in SN+ subjects. These findings may have implications for the design of high-risk cohorts for PD.

The metaiodobenzylguanidine (MIBG) scintigraphy is a well established nuclear medicine method to support the clinical diagnosis of Parkinson's disease. In this study we examined the predictive value of the MIBG scintigraphy concerning the severity and progression of the parkinsonian motor symptoms.This study included 40 patients with idiopathic Parkinson's disease (age 56 ± 9 years, Hoehn and Yahr stage 1.4 ± 0.7, mean ± standard deviation). All patients underwent a baseline visit and a follow-up visit 3-8 years (5.3 ± 1.6 years) after the baseline visit. (123)I-MIBG scintigraphy was performed only once at the baseline visit. At both visits the motor symptoms bradykinesia, rigidity, resting tremor, postural tremor and axial symptoms were quantified by means of the motor part of the Unified Parkinson's disease rating scale.The myocardial MIBG uptake correlated significantly with the annual progress of rigidity (r=-0.41, p0.05; Pearson's correlation) and axial symptoms (r=-0.49, p0.01). There was no significant correlation (p0.05) between the initial myocardial MIBG uptake and the annual progress of the other motor symptoms.The MIBG scintigraphy may predict the velocity of progress of rigidity and axial symptoms in the following 3-8 years. Such a prediction is not possible for the other motor symptoms resting tremor, postural tremor and bradykinesia.

Ventricular width and its enlargement over time are discussed as promising markers for preclinical brain atrophy. The aim of our study was to define whether brain atrophy can reliably be monitored by transcranial ultrasound (TCS). In a prospective longitudinal trial over 5years, 500 healthy persons were examined by a standardized protocol with TCS in addition to an extensive cognitive testing using the Consortium to Establish a Registry of Alzheimer's Disease - Neuropsychological Testing (CERAD-NP). TCS displayed the third ventricle in 96% of all cases at the follow-up with a high intra-individual reproducibility and excellent inter-rater coefficient (0.992). The mean diameter of the third ventricle in subjects with a cognitive decline was significantly wider (6mm±2) than in subjects with normal cognitive testing results (4.6mm±1.8). We demonstrated that the width of the third ventricle, as a marker of brain atrophy can reliably be monitored by using TCS as a non-invasive, time- and cost-effective method. We provide evidence that the assessed width of the third ventricle can differentiate between subjects with a normal cognitive performance and subjects with a cognitive decline. TCS may be a useful screening tool in the early diagnosis of cognitive decline.

Background/Aims: FP-CIT (fluoropropyl-2β-carbomethoxy-3β-4-iodophenyl-nortroptane) SPECT is a well-established nuclear medicine method to support the clinical diagnosis of Parkinson’s disease (PD). In this study, we examined the prognostic value of FP-CIT SPECT concerning the PD motor symptoms. Methods: All 38 PD patients (age 57 ± 7 years, Hoehn & Yahr stage 1.6 ± 0.8, mean ± SD) underwent a baseline visit and a follow-up visit 3–7 years (5.2 ± 1.3 years) after the baseline visit. Cerebral [123I]FP-CIT SPECT was performed only once at the baseline visit. At both visits the motor symptoms bradykinesia, rigidity, resting tremor, postural tremor and axial symptoms were quantified by means of the UPDRS motor scale. Results: There was no significant correlation between the initial striatal FP-CIT uptake and the annual progress of any motor symptom (= difference [(motor symptom at follow-up visit) – (motor symptom at baseline visit)]/time (in years) between assessments). Conclusion: The initial striatal FP-CIT SPECT does not predict the velocity of progress of PD motor symptoms within an interval of 3–7 years.

Department of Neurology, University Hospital, Homburg Saar, GermanyAbstract: Enlarged substantia nigra hyperechogenicity(SNþ) assessed by transcranial sonography (TCS) may beassociated with Parkinson’s disease (PD) risk markers suchas impaired motor performance and hyposmia. The aim ofthis multicenter cross-sectional study was to define the asso-ciation between SNþ and these risk markers in a large popu-lation older than 50 years without the diagnosis of PD. Inthree centers (Tuebingen, Homburg, and Innsbruck), 1,839individuals were examined. The echostatus of the SN wasassessed by TCS, motor performance by the Unified Parkin-son’s Disease Rating Scale (UPDRS) motor score, and olfac-tory function with Sniffin’ Sticks. From the 1,603 subjectsincluded in the analysis, 16.2% were SNþ, 23.0% scoredabove zero in the UPDRS motor section, and 28.0% werehyposmic as defined by less than 75% correctly classifiedSniffin’ Sticks. SNþ was associated with a UPDRS motorscore above zero (OR 1.45, 95% CI 1.08–1.96) and with alower odor identification capability (OR 1.48, 95% CI 1.12–1.96). The combination of these two features (OR 1.98, 95%CI 1.25–3.15) and UPDRS motor scores 3 lead to higherOR. It is concluded that SNþ, impaired motor performance,and hyposmia are frequently observed in the elderly and inisolation are unspecific and of limited use to predict a sub-ject’s risk for PD. Whether the association of SNþ with bothimpaired motor performance and hyposmia as seen in thisstudy predicts an increased risk for the development of PDneeds to be evaluated in the follow-up inves-tigations. 2010 Movement Disorder SocietyKey words: cohort studies; Parkinson’s disease/parkinson-ism; ultrasound

Transcranial ultrasound (TCS) has been shown to reveal hyperechogenicity of the substantia nigra (SN) in Parkinsonian patients and in about 10% of healthy controls. It is hypothesized that SN hyperechogenicity in healthy subjects is a vulnerability marker for idiopathic Parkinson's disease (IPD). Although there is strong evidence that the echomarker results from increased local iron content, the exact pathophysiological mechanisms remain incompletely understood. Thus, prognostic impact can only be estimated. We examined 14 subjects with SN hyperechogenicity (SN+) (7 IPD patients and 7 controls) and 7 healthy controls without the echomarker (SN−) by a magnetic resonance imaging method (MRI; T2 relaxation times) known to reveal tissue inhomogeneity following abnormal iron content and by F-Dopa PET to assess nigrostriatal function.

The present paper summarizes recommendations on transcranial sonography (TCS) application in neurodegenerative diseases, resulting from a consensus meeting of the European Society of Neurosonology and Cerebral Hemodynamics. TCS of distinct infra- and supratentorial brain structures detects characteristic changes in several movement disorders, such as abnormal hyperechogenicity of substantia nigra (SN) in Parkinson's disease and of lenticular nucleus in dystonia, Wilson's disease and atypical Parkinsonian disorders. In healthy adults, the TCS finding of marked SN hyperechogenicity indicates a subclinical functional impairment of the nigrostriatal dopaminergic system. The finding of marked SN hyperechogenicity in combination with normal lenticular-nucleus echogenicity discriminates idiopathic Parkinson's disease from multiple-system atrophy and progressive supranuclear palsy with a positive predictive value of more than 90%. As TCS is a quick and noninvasive method, using the same duplex-ultrasound machines as for investigation of intracranial vessels, applicable even in agitated patients, this method has a great potential to be more widely used.

There is evidence that nigrostriatal pathology may at least partly underlie mild Parkinsonian signs. We evaluated whether an increase in the Unified Parkinson's Disease Rating Scale part III (UPDRS-III) could be predicted by the presence of risk and prodromal markers for neurodegenerative diseases in elderly individuals without those diseases. Therefore, we analyzed the UPDRS-III score and various risk and prodromal markers known to antecede neurodegenerative diseases in a population-based cohort comprising 807 individuals free of neurodegenerative diseases at baseline. After 5 years, eight persons (1.0 %) were diagnosed with Parkinson's Disease (PD). Of those, seven (87.5 %) had motor worsening ≥3 points on the UPDRS-III from baseline to follow-up, one had two points increase. Of the 788 people without PD, 568 (72.1 %) showed no increase in the UPDRS-III scale, 220 (27.9 %) had ≥1 point increase and out of these 104 (13.2 %) had an increase of ≥3 points in the UPDRS-III score after 5 years. We identified an age >60 years (relative risk, RR = 1.7; confidence interval, CI 1.3-2.1) and the occurrence of ≥2 risk factors (RR = 1.5; CI 1.2-1.9) as possible predictors of motor progression. After 5 years, individuals with an increase in the UPDRS-III score had more often a one-sided reduced arm swing (p < 0.001) and identified less odors in the Sniffin' sticks test (p < 0.041) than persons with stable motor performance. Our data support the assumption that progression of Parkinsonian signs assessed by the UPDRS-III parallels the development of prodromal markers for neurodegenerative diseases in the elderly population.

Background: Enormous effort is being put into the identification and characterization of symptoms that may be used as predictive and progression markers in Parkinson's disease (PD). An impressive number of PD patients and individuals at risk for or in the prodromal stage of PD are currently followed in longitudinal studies; however, there does not exist an overview on the kind of markers evaluated and the assessments used. Methods: Information on the design, sample size, evaluated markers and assessments of 21 studies of the Joint Programme - Neurodegenerative Disease Research BioLoC-PD working group were collected by questionnaire. The studies were classified into at risk/prodromal or clinical PD cohorts. The assessments were grouped into quantitative assessments, investigator-rated assessments, investigator interviews, patient-rated questionnaires and caregiver-rated questionnaires. Results: Compilation of these data revealed an interesting consensus on evaluated markers, but there was an enormous variability of assessments. Furthermore, there is a remarkable similarity in the markers assessed and evaluation methods applied in the risk/prodromal and clinical PD cohorts. Conclusions: The inventory of the longitudinal cohorts that are part of the BioLoC-PD consortium reveals that there is a growing consensus on the markers that should be assessed in longitudinal cohort studies in PD. However, controversy still exists on the specific type of assessment. To allow comparison of data and common analyses it will be essential to harmonize scales and assessment outcomes.

Iron mediated oxidative stress is known to contribute to the neurodegenerative process in Parkinson's disease (PD). Although there are hints that genes involved in brain iron metabolism might be involved in the pathogenesis of PD in some instances, it is still not known whether the increase in brain iron content constitutes a primary or secondary event in the disease cascade. Recent studies on the role of hemochromatosis gene (HFE) mutations in PD vary from a protective effect of C282Y heterozygosity, no effect of the C282Y or H63D mutation to an increased risk for PD in C282Y mutation carriers. In this study, analyzing the whole coding region of the HFE gene by dHPLC in 278 PD patients, priorly characterized by transcranial sonography for increased iron content of the substantia nigra (SN), we did not find an association of the common HFE mutations and PD. However, we identified two novel variants (K92N and I217T) each in a single PD patient. These variations were not found in any of the controls. Future studies are necessary to reveal a possible functional relevance of these mutations for PD. Our results indicate that mutations in the HFE gene are not a common cause for PD with increased iron levels of the SN.

Hyperechogenic signal of substantia nigra (SN) in transcranial sonography (TCS) and reduced striatal uptake in FP-CIT SPECT are common findings in idiopathic Parkinson's disease (PD). But so far it is unknown whether the extent of SN hyperechogenicity represents a correlate for the degeneration of presynaptic dopaminergic neurons in PD. We performed TCS and 123I-labelled N -(3-fluoropropyl)-2s-carbomethoxy-3s-(4-iodophenyl)nortropane ([123I]FP-CIT) SPECT in 53 patients with PD. Striatal FP-CIT uptake was quantified by measuring the striatal/posterior lobe binding of [123I]FP-CIT. SN echogenicity was quantified by planimetric measurement of the maximum extension of hyperechogenic signals. We found no correlation between striatal FP-CIT uptake and echogenicity of the SN, neither contralateral to the clinically more affected body side ( r = +0.08, P = 0.57; Pearson's correlation) nor ipsilateral ( r = +0.01; P = 0.92). Our data show that the extent of SN hyperechogenicity does not correlate with the degeneration of presynaptic dopaminergic nerve terminals. Obviously SN hyperechogenicity and degeneration of presynaptic dopaminergic nerve terminals exist independently from each other and may be based on different pathomechanisms.

Identification of risk factors and prodromal markers for Parkinson's disease (PD) and the understanding of the point in time of first occurrence is essential for the early detection of incident PD. In this three-center longitudinal, observational study, we evaluated the specific risk for PD associated with single or combinations of risk factors and prodromal markers. In addition, we evaluated which risk factors and prodromal markers emerge at which time before the diagnosis of PD. Of the 1,847 at-baseline PD-free individuals ≥ 50 years, 1,260 underwent the 5-year follow-up assessment. There were 21 cases of incident PD during the study period. Enlarged hyperechogenic substantia nigra was the most frequent baseline sign in individuals developing PD after 3 years (80.0 %) and 5 years (85.7 %) compared to healthy controls (17.5 %) followed by the occurrence of mild parkinsonian signs and hyposmia. Evaluation of the signs at the first follow-up assessment showed that individuals developing PD after two additional years showed the same pattern of signs as individuals who developed PD 3 years after baseline assessment.

Mechanical thrombectomy with stent retrievers in acute stroke has emerged as a promising new technique with the highest recanalization rate of the therapeutic procedures available so far. However, endovascular treatment is also associated with the risk of specific complications. One of those is the occurrence of peri-interventional subarachnoid hemorrhage (SAH), which has been reported in 5-16 % of the cases. Interestingly, this rate is higher than that of angiographically detectable perforations (0-3 %), leaving the majority of peri-interventional SAH to be due to angiographically occult perforations. Little is known about the influence of this finding on clinical outcome. The purpose of this study was to investigate the clinical relevance of SAH due to occult perforations during thrombectomy with stent retrievers.Postinterventional computed tomography (CT) scans of 217 consecutive patients with acute occlusions of intracerebral arteries who were treated with stent retrievers in our department between October 2009 and October 2012 were retrospectively analyzed.SAH was found on postinterventional CT scans in 5.5 % of the cases. Seven cases were included for further analysis and matched to controls by the following characteristics: (1) site of occlusion, (2) result of the recanalization procedure according to the modified thrombolysis in cerebral infarction score, (3) administration of intravenous recombinant tissue plasminogen activator, (4) presence of proximal extracranial occlusion, (5) age, and (6) sex. Comparison of the angiographic data of the two cohorts showed no significant difference in the length of the procedures or the number of maneuvers needed for recanalization, nor were there significant differences in clinical outcomes as measured by NIHSS and mRS scores. Secondary symptomatic ICH occurred in one case in either cohort and led to death in both cases. The rate of asymptomatic ICH within the first 24 h after recanalization was significantly higher in the group with peri-interventional SAH (57 vs. 0 %, P = 0.018).This small retrospective case-control study did not reveal a significant influence of peri-interventional SAH due to angiographically occult perforations on neurologic outcome of patients treated with stent retrievers.

Patients with idiopathic Parkinson’s disease (IPD) have a reduced myocardial MIBG uptake in MIBG scintigraphy, indicating myocardial sympathetic denervation. We were interested whether this myocardial sympathetic denervation coincides with clinical symptoms of autonomic impairment in IPD patients. We performed MIBG scintigraphy, the SCOPA-AUT scale, a standardized medical history (developed in our clinic) and autonomic nervous system testing in 47 IPD patients (21 female, 26 male patients). We correlated myocardial MIBG uptake with the results of the SCOPA-AUT scale, the standardized medical history and the autonomic nervous system testing through the use of Spearman’s correlation. Myocardial MIBG uptake correlated significantly (p < 0.05) with several items of the SCOPA-AUT scale (in female patients: perspiration during the night, in male patients: sum score, saliva dribbling of the mouth, difficulty swallowing, fainting, constipation), of the standardized medical history (in male patients: swollen ankles) and of the autonomic nervous system testing (all patients: sum score, Ewing orthostasis test). Remarkably, we found more significant correlations in male than in female patients. Reduced myocardial sympathetic innervation—as revealed by MIBG scintigraphy—is associated with clinical symptoms of autonomic impairment. This association is more pronounced in male than in female patients. The cause for this gender-specific phenomenon is unclear.

Background: Bradydiadochokinesia is one main clinical symptom in idiopathic Parkinson's disease (IPD). The pathogenesis of bradydiadochokinesia is not completely clear. Methods: Fifteen patients with IPD and 15 age-matched healthy volunteers had to perform rhythmic alternating flexion and extension movements in the elbow joint. The rhythm was provided auditorily by a click tone stimulator. Six maneuvers (spatial extents of 48 and 83° at frequencies of 0.45, 0.75 and 1.25 Hz) had to be absolved. The potentiometer converted the horizontal forearm movements into a variable voltage. Results: The duration of single movements varied more significantly in patients than in controls (p < 0.05; Mann-Whitney U test). Patients executed all conditions more slowly than controls, but this difference was only significant at the most difficult condition (83° at 1.25 Hz; p < 0.01). The movement amplitudes or their variability were not significantly different at any condition. No parameter correlated significantly with the motor part of the Unified Parkinson's Disease Rating Scale (UPDRS) or with the duration of disease. Conclusion: An insufficient temporal coordination contributes to bradydiadochokinesia in IPD. This deficit occurs independently of other parkinsonian cardinal motor symptoms.

In order to assess nigrostriatal function over time in healthy subjects with substantia nigra hyperechogenicity (SN+) believed to be at higher risk for Parkinson's disease (PD), ten healthy SN+ subjects underwent [(18)Fluoro] Dopa positron emission tomography (PET) twice-at baseline and after a mean of 7.3 years. Neurological examination took place at study inclusion followed by a structured telephone interview focusing on early Parkinsonian symptoms at the time point of second PET study and 3.5 years later. The [(18)Fluoro] Dopa uptake remained unchanged in eight of ten participants. In the other two subjects marked unilateral reduction of putaminal [(18)Fluoro] Dopa uptake ratios appeared over the time, followed by complaints of a clinically manifest PD in one. We suggest that the progressive pathological PET findings in 20 % and PD conversion in 10 % of our cohort may be in accordance with the presumed proportion of SN+ individuals eventually developing PD based on SN+ prevalence of 10 % within the healthy population, being ten times higher than PD prevalence in the age of over 60 years. Our findings hint towards a significance of SN+ indicating a high risk for PD in some extrapyramidally healthy SN+ individuals.

Since the first description of changes in the B-mode ultrasound signal of the brain in patients with Parkinson disease in 1995, the use of transcranial sonography (TCS) to assess brainstem and subcortical brain structures has become an important tool for the diagnosis and differential diagnosis of various movement disorders. Today it is applied in the clinical routine and for research purposes all over the world. Because of the underlying physical principles, TCS displays alterations associated with movement disorders differently compared to other neuroimaging methods. Thus information obtained by TCS may be regarded as supplementary. The most widely recognized finding for movement disorders has been an increase in echogenicity of the substantia nigra (SN) in idiopathic Parkinson disease (iPD). This finding enables the reliable diagnosis of iPD with high predictive values. Other sonographic features, such as hypoechogenicity of the brainstem midline and hyperechogenicity of the lentiform nucleus, may help in the differential diagnosis of iPD and other movement disorders. Here we review the method, advantages, and limitations of this neuroimaging tool, and include guidelines for the scanning procedure. Examples for application are illustrated. This review substantiates that TCS is a reliable, noninvasive, commonly available, easily applicable, and inexpensive method, which provides new information about the morphology of the brain to support the diagnosis of various movement disorders.

The diagnosis of idiopathic Parkinson's disease (IPD) is based on clinical criteria. In the last two decades several neuroimaging methods using transcranial sonography (TCS) or radiolabelled tracers such as the myocardial MIBG scintigraphy were applied to support diagnosis of IPD. They have been used independently of each other and their interrelation is not yet clear. In the present study we analyzed the relation between findings of TCS, MIBG scintigraphy, and clinical presentation in 42 patients with IPD who were clinically diagnosed and underwent clinical follow-up over ≥3 years in order to confirm IPD diagnosis throughout the clinical course. The extent of substantia nigra hyperechogenicity (SN+) contralateral to the clinically more affected body side (SN(contra)) was compared to myocardial (123)I-MIBG uptake. SN(contra) did not correlate with the myocardial MIBG uptake (r = -0.10; p = 0.52). Both myocardial MIBG uptake and TCS did not correlate significantly with Hoehn and Yahr stage (r = -0.03; p = 0.87 and r = -0.10; p = 0.54, respectively). Sensitivity of TCS in the diagnosis of IPD was 79%, of MIBG scintigraphy 81%. The combination of both measurements reached a sensitivity of 95%. TCS and MIBG scintigraphy may disclose complementary aspects of IPD. The combined use of both neuroimaging methods might improve the diagnostic sensitivity regarding IPD.

This study sought to assess the feasibility and safety of a recently described technique of mechanical recanalization with the help of a stent-like device.In the special group of acute stroke patients with an intracranial large vessel occlusion, intravenous tissue-type plasminogen activator on its own leads to a good clinical outcome (mRS ≤ 2) in only 15% to 25% of cases. The aforementioned technique of mechanical recanalization showed very promising clinical results.Forty patients presenting within 6 h from stroke symptom onset were enrolled. Mechanical recanalization was performed using a Solitaire FR revascularization device. The primary endpoint of the study was the clinical outcome rated with the help of the modified Rankin Scale (mRS) after 90 days.Twenty-four patients (60%) showed a good clinical outcome (mRS ≤ 2) at 90 days. One symptomatic hemorrhage was detected on follow-up computed tomography. The death rate was 12.5% (5 patients). Successful recanalization (Thrombolysis In Cerebral Infarction score ≥ 2b) of the target vessel was achieved in 95% of the patients with a mean of 1.8 runs with the device.The ReFlow (Mechanical Recanalization With Flow Restoration in Acute Ischemic Stroke) study shows that mechanical recanalization with flow restoration is highly effective in stroke patients with a large intracranial vessel occlusion presenting within 4.5 h after symptom onset. (Mechanical Recanalization With Flow Restoration in Acute Ischemic Stroke [ReFlow]; NCT01210729).

Objectives The purpose of this study is to demonstrate the technical success of carotid artery stenting in acute extracranial internal carotid artery (ICA) occlusion as well as the benefit in clinical outcome. Background Stroke caused by acute occlusion of the ICA is associated with a significant level of morbidity and mortality. For this type of lesion, treatment with standard intravenous thrombolysis alone leads to a good clinical outcome in only 17% of the cases, with a death rate as high as 55%. Recanalization of the occluded ICA can lead to an improvement in acute symptoms of stroke, prevent possible deterioration, and reduce long-term stroke risk. At present, there is no consensus treatment for patients with acute ischemic stroke presenting with severe clinical symptoms due to atherosclerotic occlusion of the extracranial ICA. Methods Carotid artery stenting was performed in 22 patients with acute atherosclerotic extracranial ICA occlusion within 6 h of stroke symptom onset. In 18 patients, there was an additional intracranial occlusion at the level of the terminal segment of the ICA (n = 4) and at the level of the middle cerebral artery (n = 14). Intracranial occlusions were either treated with the Penumbra system or the Solitaire stent–based recanalization system, or a combination of mechanical recanalization and intra-arterial thrombolysis. Recanalization results were assessed by angiography immediately after the procedure. The neurologic status was evaluated before and after the treatment with a follow-up as long as 90 days using the National Institutes of Health Stroke Scale and the modified Rankin Scale. Results Successful revascularization of extracranial ICA with acute stent implantation was achieved in 21 patients (95%). There was no acute stent thrombosis. After successful recanalization of the origin of the ICA, the intracranial recanalization with Thrombolysis In Myocardial Infarction flow grade 2/3 was achieved in 11 of the 18 patients (61%). The overall recanalization rate (extracranial and intracranial) was 14 of 22 patients (63%). Nine patients (41%) had a modified Rankin Scale score of ≤2 at 90 days. The mortality rate was 13.6% at 90 days. Conclusions Carotid artery stenting in acute atherosclerotic extracranial ICA occlusion with severe stroke symptoms is feasible, safe, and useful within the first 6 h after symptom onset.

Background and Purpose— The purpose of this study was to demonstrate a new approach to the use of a self-expanding stent in the treatment of acute ischemic stroke. Methods— Twenty-two consecutive patients with acute intracerebral artery occlusions were treated with a self-expandable intracranial stent, which was withdrawn in its unfolded state. For this technique, we used the Solitaire AB/FR, which is the only intracranial stent that is fully recoverable. Eight patients had an occlusion of the basilar artery, 12 had a middle cerebral artery occlusion, and 2 had terminal carotid artery occlusions; 6 of these had to be treated first for an acute occlusion originating in the internal carotid artery. Recanalization results were assessed by follow-up angiography immediately after the procedure. Neurologic status was evaluated before and after treatment (90-day follow-up) according to the National Institutes of Health Stroke Scale and modified Rankin scale. Results— Successful revascularization was achieved in 20 of 22 (90.9%) patients (thrombolysis in cerebral infarction [TICI] 2a/b and 3), a TICI 3 state was accomplished in 12 patients, and partial recanalization or slow distal branch filling with filling of more than two-thirds of the vessel territory was achieved in 8 patients (TICI 2b). There was immediate flow restoration in 21 of 22 (95.4%) cases after deployment of the device. The stent was removed in its unfolded state in all patients. The mean time from stroke symptom onset to recanalization was 277 minutes, with a standard deviation of 118 minutes. Mean National Institutes of Health Stroke Scale score on admission was 19.4, with a standard deviation of 5.7. Almost two-thirds of the patients (63.6%) improved by >10 points on the National Institutes of Health Stroke Scale at discharge, and 50% showed a modified Rankin scale score of ≤2 at 90 days (59% with a modified Rankin scale ≤3). Mortality was 18.1%. In 1 case, an asymptomatic intracranial hemorrhage was detected on control computed tomography, and 2 patients had a symptomatic intracranial hemorrhage. Conclusion— Withdrawal of an unfolded, fully recoverable, intracranial stent yielded very promising angiographic and clinical results. It combines the advantages of prompt flow restoration and mechanical thrombectomy.

Substantia nigra hyperechogenicity (SN+) is a common finding in transcranial ultrasound studies of parkinsonian patients. However, this echofeature is also found in 10% of healthy controls and is here associated with PD risk factors such as male gender and positive family history for PD, as well as with putative premotor symptoms such as hyposmia, depression, sleep disturbances, and subtle neuropsychological and motor impairment. SN+ is hypothesized to indicate an increased risk to develop PD during lifetime. PET studies substantiate this hypothesis, given that SN+ controls showed reduced striatal [(18)Fluoro] Dopa uptake in PET studies. First results of longitudinal follow-up studies show a higher relative risk for future PD in SN+ asymptomatic individuals.

Objective Transcranial sonography (TCS) shows characteristic hyperechogenicity of the substantia nigra (SN) in patients with Parkinson9s disease (PD). Although this feature is well established, sufficient observer reliability and diagnostic accuracy are prerequisites for advancements of this method. Methods The authors investigated both aspects in a cross-sectional study with four blinded TCS raters in 22 PD patients and 10 healthy controls. Results As expected, the authors found significant bilateral SN hyperechogenicity in PD patients. Quantitative computerised SN planimetry had a substantial intra- (intraclass correlation coefficient (ICC) 0.97 and 0.93 respectively for both hemispheres) and inter-rater reliability (ICC 0.84 and 0.89), while visual semiquantitative echogenicity grading of the SN revealed a moderate intrarater (weighted kappa 0.80 ipsilateral and 0.74 contralateral) and slight (0.33) to fair (0.51) inter-rater reliability only. Diagnostic accuracy measured as the area under the curve of receiver-operating characteristics plots was highest in TCS of the SN opposite the clinically most affected body side (planimetry 0.821, echogenicity grading 0.792) with a hyperechogenic area of 0.24 cm 2 as the optimum cut-off value for the differentiation between PD and controls (sensitivity 79%, specificity 81%). Conclusions The data demonstrate that the observer variability of SN planimetry is low in the hands of experienced investigators. This approach also offers adequate diagnostic accuracy. The authors conclude that reliable SN TCS data on PD can be achieved in clinical routine and multicentre trials when standardised analysis protocols and certain quality criteria of brain parenchyma sonography are met.