Parkinson-plus Research Articles

A study of the correlation of non-motor symptoms of Parkinson’s disease and Parkinson plus syndrome with duration of the disease

Introduction: Depression is common in PD, occurring in up to one-half of the patients. Anxiety disorders may be as common as depression and the two are frequently co-existent. Parkinson plus syndrome (PPS) is a group of sporadic, neurodegenerative diseases of the central nervous system, less common and usually more severe than Parkinson’s disease. They are characterised by relatively rapid disease progression and the presence of features that are atypical for PD, such as early postural instability and dementia, severe autonomic failure, or pyramidal and cerebellar signs. Material and Methods: The study was conducted to assess the effect of duration in non-motor dysfunction in 60 patients with Parkinsonism including idiopathic Parkinson’s disease and Parkinson plus syndrome who attended either the outdoor services, or were admitted in the Neurology ward of our tertiary care centre, PGIMER & Dr. RML Hospital, New Delhi, India. Results: PD patients had no correlation between cognition and duration of disease (p=0.079 with MMSE and p=0.145 with SCOPA COG) but had significant correlation between depression, sleep problems, pain and autonomic dysfunction with duration of disease (p=0.027, 0.008, 0.010 and 0.042 respectively). Conclusion: Early recognition and diagnosis of PD and PPS is important as treatment may improve quality of life in patients. Co-existence of non-motor dysfunctions in the disease significantly adds to the burden of disease and affects the quality of life.

Can loss of the swallow tail sign help distinguish between Parkinson Disease and the Parkinson-Plus syndromes?

PurposeTo determine if loss of the swallow tail sign (STS) can distinguish Parkinson Disease (PD) from the Parkinson-Plus syndromes. MethodsTwenty-five patients with PD, 21 with Parkinson-Plus syndromes, and 14 control patients were included. Presence of the STS was assessed. ResultsThe STS was present in 79% of controls, statistically greater than the PD/Parkinson-Plus patients. There was no difference in the presence of the STS between the PD/Parkinson-Plus subgroups or when scanning at 1.5 T or 3 T. ConclusionsLoss of the STS could not distinguish between PD and Parkinson-Plus patients. The STS can be identified at both 1.5 T and 3 T.

Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis

ObjectiveTo undertake a systematic review and meta-analysis of studies that investigated prognostic factors and survival in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA).MethodsPublications of at least...

Neuropsychiatric symptoms and PET imaging characteristics in patients with Parkinson-plus syndromes

Objective To explore the neuropsychiatric symptoms and 18 F-fluoro-2-deoxy-D-glucose ( 18 F-FDG) PET imaging features of Parkinson-plus syndromes. Methods There were 8 patients with probable Parkinson-plus syndromes, including one case of multiple system atrophy-cerebellar predominant (MSA-C), 4 cases of progressive supranuclear palsy (PSP), one case of corticobasal ganglionic degeneration (CBD) and 2 cases of dementia with Lewy bodies (DLB). Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) were used to evaluate cognitive function, Neuropsychiatric Inventory (NPI) was used to evaluate neuropsychiatric behaviors, and Hamilton Depression Rating Scale-21 Items (HAMD-21) was used to evaluate the emotional state of patients. Results One MSA-C patient showed only anxiety. Four PSP patients showed different degrees of cognitive disorders, of whom 3 cases also presented obvious depression, anxiety, irritability and sleep disorders. One case of CBD showed dysfunction in executive function, visual spatial ability, verbal function, attention and orientation, as well as depression, anxiety, irritability and sleep disorders. Two cases of DLB were found unable to copy pentagon in MMSE chart or draw a circle in Clock Drawing Test (CDT), and they also presented hallucination, depression and indifference. As for the result of 18 F-FDG PET, one MSA-C patient showed cerebellarglucose hypometabolism; 4 PSP patients showed hypometabolism in bilateral symmetrical frontal lobes, anterior cingulate gyrus and parietal lobe, especially in thalamus, basal ganglia region and brain stem; one case of CBD showed hypometabolism in right lateral fronto-temporo-parieto-occipital lobes, left lateral parietal lobe, bilateral cingulate gyri and precuneus; 2 cases of DLB showed hypometabolism in bilateral temporo-occipital lobes. Conclusions Patients with early Parkinson-plus syndromes are easily misdiagnosed as mental illness and delayed treatment, in addition, their movement disorders and extrapyramidal symptoms would be aggravated due to misusage of sedative hypnotic drugs. Neuropsychological changes are found closely related to cognitive dysfunction. PET imaging has important clinical values to early diagnosis of Parkinson-plus syndromes. DOI: 10.3969/j.issn.1672-6731.2017.01.008

'Humming bird sign', 'Mickey Mouse sign', and 'morning glory sign' in progressive supranuclear palsy

Progressive supranuclear palsy (PSP), also known as the Steele–Richardson–Olszewski syndrome, is a form of Parkinson-plus syndrome. Patients with PSP show interesting radiological signs such as the 'Humming bird sign' and the 'Mickey mouse sign'. Here, we report a patient with PSP, in whom the MRI brain demonstrated the 'Humming bird sign', the 'Mickey mouse sign', and the 'Morning glory sign'.

Clinical applications of neurotoxin injections in Parkinson's disease and parkinson-plus syndromes: Experience of a neurology department

Clinical applications of neurotoxin injections in Parkinson's disease and parkinson-plus syndromes: Experience of a neurology department

DCTN1 p.K56R in progressive supranuclear palsy

IntroductionMutations in dynactin DCTN1 (p150glued) have previously been linked to familial motor neuron disease or Perry syndrome (PS) consisting of depression, parkinsonism and hypoventilation. MethodsWe sequenced DCTN1 in 636 Caucasian patients with parkinsonism (Parkinson's disease and Parkinson-plus syndromes) and 508 healthy controls. Variants (MAF < 0.01) were subsequently genotyped in Caucasian (1360 cases and 1009 controls) and Asian cohorts (1046 cases and 830 controls), and the functional implications of pathogenic variants were assessed. ResultsWe identified 17 rare variants leading to non-synonymous amino-acid substitutions. Four of the variants were only observed in control subjects, four in both cases and controls and the remaining nine in cases only. One of the variants, DCTN1 p.K56R, was present in two patients with progressive supranuclear palsy (PSP) with a shared minimal 2.2 Mb haplotype. Both subjects have parkinsonism as the most prominent symptom with abnormal ocular movements, moderate cognitive impairment and little to no l-dopa response. Neither subject presents with depression, central hypoventilation or weight loss. For one of the subjects MRI shows symmetrical atrophy of temporal and frontoparietal lobes. In HEK293 cells mutant p150glued (p.K56R) shows less affinity for microtubules than wild-type, with a more diffuse cytoplasmic distribution. ConclusionsWe have identified DCTN1 p.K56R in patients with PSP. This variant is immediately adjacent to the N-terminal p150glued ‘CAP-Gly’ domain, affects a highly conserved amino acid and alters the protein's affinity to microtubules and its cytoplasmic distribution.

Role of Iron Depsoition Patterns as Evaluated by Susceptibility-Weighted Imaging of the Brain in Differentiating Parkinson Disease and the Parkinson-Plus Syndromes (P1.012)

Role of Iron Depsoition Patterns as Evaluated by Susceptibility-Weighted Imaging of the Brain in Differentiating Parkinson Disease and the Parkinson-Plus Syndromes (P1.012)

Chapter 13 - Single-photon emission tomography

Single-photon emission computed tomography (SPECT) is a functional nuclear imaging technique that allows visualization and quantification of different in vivo physiologic and pathologic features of brain neurobiology. It has been used for many years in diagnosis of several neurologic and psychiatric disorders. In this chapter, we discuss the current state-of-the-art of SPECT imaging of brain perfusion and dopamine transporter (DAT) imaging. Brain perfusion SPECT imaging plays an important role in the localization of the seizure onset zone in patients with refractory epilepsy. In cerebrovascular disease, it can be useful in determining the cerebrovascular reserve. After traumatic brain injury, SPECT has shown perfusion abnormalities despite normal morphology. In the context of organ donation, the diagnosis of brain death can be made with high accuracy. In neurodegeneration, while amyloid or (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) are the nuclear diagnostic tools of preference for early and differential diagnosis of dementia, perfusion SPECT imaging can be useful, albeit with slightly lower accuracy. SPECT imaging of the dopamine transporter system is widely available in Europe and Asia, but since recently also in the USA, and has been accepted as an important diagnostic tool in the early and differential diagnosis of parkinsonism in patients with unclear clinical features. The combination of perfusion SPECT (or FDG-PET) and DAT imaging provides differential diagnosis between idiopathic Parkinson's disease, Parkinson-plus syndromes, dementia with Lewy bodies, and essential tremor.

Parkinson-plus syndromes: epidemiological findings from a Tunisian cohort of 210 patients

Parkinson-plus syndromes: epidemiological findings from a Tunisian cohort of 210 patients

Characteristics of Dysarthria and Cognitive Functions in Patients with Parkinson’s Disease and Parkinson-plus Syndrome

배경 및 목적: 본 연구는 공통적 특징으로 감별진단이 어려운 특발성 파킨슨병(idiopathic Parkinson's disease, IPD)과 파킨슨플러스 증후군 환자의 말/인지기능 특징을 살펴보고, 감별진단을 위한 유용한 요소인지 알아보고자 하였다. 방법: IPD 환자 11명과 파킨슨플러스 증후군 중 산발적 올리브교소뇌위축(sporadic...

Davunetide in patients with progressive supranuclear palsy: a randomised, double-blind, placebo-controlled phase 2/3 trial

In preclinical studies, davunetide promoted microtubule stability and reduced tau phosphorylation. Because progressive supranuclear palsy (PSP) is linked to tau pathology, davunetide could be a treatment for PSP. We assessed the safety and efficacy of davunetide in patients with PSP. In a double-blind, parallel group, phase 2/3 trial, participants were randomly assigned with permuted blocks in a 1:1 ratio to davunetide (30 mg twice daily, intranasally) or placebo for 52 weeks at 48 centres in Australia, Canada, France, Germany, the UK, and the USA. Participants met the modified Neuroprotection and Natural History in Parkinson Plus Syndrome study criteria for PSP. Primary endpoints were the change from baseline in PSP Rating Scale (PSPRS) and Schwab and England Activities of Daily Living (SEADL) scale at up to 52 weeks. All participants and study personnel were masked to treatment assignment. Analysis was by intention to treat. The trial is registered with Clinicaltrials.gov, number NCT01110720. 313 participants were randomly assigned to davunetide (n=157) or to placebo (n=156), and 241 (77%) completed the study (118 and 156 in the davunetide and placebo groups, respectively). There were no differences in the davunetide and placebo groups in the baseline PSPRS and SEADL. The davunetide and placebo groups did not differ in the change from baseline in PSPRS (median 11·8 [95% CI 10·5 to 13·0] vs 11·8 [10·5 to 13·0], respectively, p=0·41) or SEADL (-0·20 [-0·20 to -0·17] vs -0·20 [-0·22 to -0·17], respectively, p=0·92). 54 serious adverse events were reported in each of the treatment groups, including 11 deaths in the davunetide group and ten in the placebo group. The frequency of nasal adverse events was greater in the davunetide group than in the placebo group (epistaxis 18 [12%] of 156 vs 13 [8%] of 156, rhinorrhoea 15 [10%] vs eight [5%], and nasal discomfort 15 [10%] vs one [<1%]). Davunetide is not an effective treatment for PSP. Clinical trials of disease-modifying treatment are feasible in patients with PSP and should be pursued with other promising tau-directed treatments. Allon Therapeutics.

Cognition in corticobasal syndrome and progressive supranuclear palsy: A review

AbstractCorticobasal syndrome (CBS) and progressive supranuclear palsy (PSP) represent challenging neurodegenerative disorders for clinicians and nonclinical scientists alike. Although initially lumped together as “Parkinson's‐Plus” syndromes, CBS and PSP are clinically and pathologically distinct from Parkinson's disease. It is now clear that behavioral and cognitive changes are common in both syndromes and affect impact quality of life and carer burden considerably. We briefly review the clinical, pathological, and neuroradiological features of each syndrome, followed by more detailed descriptions of the behavioral and cognitive deficits encountered in CBS and PSP. Clinically and pathologically heterogeneous, CBS is characterized by a wide range of cognitive and behavioral disturbances. impairments in executive function and memory are common, but nonspecific. In contrast, deficits in language and visuospatial abilities appear to be more distinctive features of CBS; the relevance of specific patterns of impairment to the underlying histopathology, or prognosis, remains to be fully elucidated. As in CBS, behavioral and cognitive changes are almost universal in PSP, with a wide range of reported deficits. Apathy is very common, often paradoxically accompanied by impulsivity. Executive dysfunction is prominent, but memory and visuospatial deficits also occur. An emerging field is the study of social cognition, which appears impaired in both syndromes. As therapeutic strategies for neurodegenerative pathologies emerge, more specific diagnostic tools in CBS and PSP will be required. Careful clinicopathological correlation, and the development of biomarkers for specific histopathologies, will be important milestones on the road to effective treatments.

MP80-19 DIFFERENCES IN URODYNAMIC PARAMETERS BETWEEN PARKINSON′S DISEASE AND PARKINSON-PLUS SYNDROME

You have accessJournal of UrologyUrodynamics/Incontinence/Female Urology: Neurogenic Voiding Dysfunction1 Apr 2014MP80-19 DIFFERENCES IN URODYNAMIC PARAMETERS BETWEEN PARKINSON′S DISEASE AND PARKINSON-PLUS SYNDROME Sung Yong Cho, Seong Jin Jeong, Myong Kim, Hahn-Ey Lee, Jae Hyun Jung, Hyeon Jeong, Seung Bae Lee, Hwancheol Son, and Seung-June Oh Sung Yong ChoSung Yong Cho More articles by this author , Seong Jin JeongSeong Jin Jeong More articles by this author , Myong KimMyong Kim More articles by this author , Hahn-Ey LeeHahn-Ey Lee More articles by this author , Jae Hyun JungJae Hyun Jung More articles by this author , Hyeon JeongHyeon Jeong More articles by this author , Seung Bae LeeSeung Bae Lee More articles by this author , Hwancheol SonHwancheol Son More articles by this author , and Seung-June OhSeung-June Oh More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2014.02.2542AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Parkinson-plus syndrome (PPS) is a group of neurodegenerative diseases featuring the classical features of Parkinson¡¯s disease (PD) as well as additional features which distinguish them from simple idiopathic PD. We investigated differences in urodynamic parameters among three groups, which were; PD, multiple system atrophy (MSA), and other rare conditions of PPS. METHODS A retrospective study was performed in patients with PD presenting to three referral centers between Jan. 2005 and Dec. 2012. Patients who referred to the urology department for lower urinary tract symptoms underwent urodynamic studies. Patients with ambiguous subtypes of PPS, urinary tract infection, urinary stones, or other neurogenic conditions except for PD were excluded from the analysis. RESULTS A total of 607 patients (380 males and 227 females) were included in the analysis. The mean patient age was 67.0 (±8.6, SD) years. A consecutive 390 patients with PD (64.3%), 191 patients with MSA (31.5%) and 26 patients with other types of PPS (4.3%) were analyzed. MSA showed statistically significantly decreased maximal flow rate (Qmax) and increased post-voided residual (PVR) compared with other conditions (p <0.001). In addition, voiding failure occurred more frequently in MSA than other conditions (OR=1.9, 95% CI: 1.26-2.73). MSA showed significantly decreased bladder compliance (p=0.004), decreased bladder contractility (p<0.001) and an increased rate of voiding failure (p=0.005). Impaired contractility was identified more frequently in MSA than in other conditions (p <0.001). CONCLUSIONS MSA showed more significant impairment of lower urinary tract function than other conditions. Therefore, patients with MSA should be more cautiously managed for bladder dysfunction. Urodynamic results according to the subtype of parkinsonism PD MSA Other conditions of PPS P Value No. of patients 390 191 26 - Maximal flow rate (ml/sec) 7.6 ± 7.6‡ 4.9 ± 6.0 5.1 ± 6.2 < 0.001* Postvoid residual urine (ml) 78.0 ± 111.6‡ 144.8 ± 130.0 103.1 ± 93.7 < 0.001* Failure to void 83 (21.3%) 64 (33.5%)† 8 (30.8%) 0.005* First desire to void (ml) 173.7 ± 112.4 194.6 ± 126.2 186.6 ± 110.8 0.125 Strong desire to void (ml) 295.4 ± 135.3 314.9 ± 135.4 341.5 ± 146.8 0.160 Compliance < 20 ml/cmH2O 63 (16.2%) 55 (28.8%)† 3 (11.5%) 0.004* Detrusor overactivity 260 (66.7%) 124 (64.9%) 18 (69.2%) 0.868 PdetQmax (cmH2O) 35.9 ± 26.3 22.5 ± 23.4‡ 46.8 ± 7.7 < 0.001* Opening pressure (cmH2O) 36.2 ± 31.0 21.0 ± 22.9‡ 36.0 ± 25.4 < 0.001* Bladder contractility index 83.1 ± 46.9 53.1 ± 44.7‡ 80.8 ± 46.2 < 0.001* Presence of decreased contractility 247 (63.3%) 163 (85.3%)† 14 (53.8%) < 0.001* †,Significantly higher and ‡,Significantly lower than other parameters according to the post-hoc analysis; * P Value < .05 © 2014FiguresReferencesRelatedDetails Volume 191Issue 4SApril 2014Page: e945 Advertisement Copyright & Permissions© 2014MetricsAuthor Information Sung Yong Cho More articles by this author Seong Jin Jeong More articles by this author Myong Kim More articles by this author Hahn-Ey Lee More articles by this author Jae Hyun Jung More articles by this author Hyeon Jeong More articles by this author Seung Bae Lee More articles by this author Hwancheol Son More articles by this author Seung-June Oh More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

The value of novel MRI techniques in Parkinson-plus syndromes: Diffusion tensor imaging and anatomical connectivity studies

Conventional MRI is a well-described, highly useful tool for the differential diagnosis of degenerative parkinsonian syndromes. Nevertheless, the observed abnormalities may only appear in late-stage disease. Diffusion tensor imaging (DTI) can identify microstructural changes in brain tissue integrity and connectivity. The technique has proven value in the differential diagnosis of multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and Parkinson's disease (PD). Here, we performed a systematic review of the literature on the main corticosubcortical DTI abnormalities identified to date in the context of the diagnosis of MSA and PSP with diffusion-weighted imaging, diffusion tensor imaging and anatomical connectivity studies. In good agreement with the histological data, increased diffusivity in the putamen (in MSA and PSP), in the middle cerebellar peduncles (in MSA) and in the upper cerebellar peduncles (in PSP) has been reported. Motor pathway involvement is characterized by low fraction anisotropy (FA) in the primary motor cortex in MSA-P and PSP, a high apparent diffusion coefficient (ADC) and low FA in the supplementary motor area in PSP. We then outline the value of these techniques in differential diagnosis (especially with respect to PD). Anatomical connectivity studies have revealed a lower number of fibers in the corticospinal tract in MSA and PSP (relative to PD and controls) and fewer tracked cortical projection fibers in patients with PSP or late-stage MSA (relative to patients with early MSA or PD and controls). Lastly, we report the main literature data concerning the value of DTI parameters in monitoring disease progression. The observed correlations between DTI parameters on one hand and clinical scores and/or disease duration on the other constitute strong evidence of the value of DTI in monitoring disease progression. In MSA, the ataxia score was correlated with ADC values in the pons and the upper cerebellar peduncles, whereas both the motor score and the disease duration were correlated with putaminal ADC values. In conclusion, DTI and connectivity studies constitute promising tools for differentiating between “Parkinson-plus” syndromes.

Surgical Management of Airway Dysfunction in Parkinson's Disease Compared with Parkinson-Plus Syndromes

We sought to compare the laryngeal symptoms of Parkinson's disease (PD) with those of multiple system atrophy (MSA), a Parkinson-plus syndrome; to review the differences in surgical management of upper airway dysfunction between patients with PD and those with MSA; and to present a treatment algorithm for management of upper airway disorders in patients with PD and MSA. We analyzed the airway manifestations of each disease, including clinical and physiological test results and management outcomes, in a case series of 30 patients (24 with PD and 6 with MSA). Vocal fold atrophy causing bowing with a midfold glottic gap was common in patients with PD. One third of patients with PD underwent vocal fold augmentation with noticeable improvement in vocal volume and phonation time. Tracheostomy was required for life-threatening sleep apnea in 50% of the patients with MSA. Systemic medications and speech therapy were integral components of the management regimen. Surgical management of laryngeal disorders in patients with PD should focus on restoring bulk to atrophic vocal folds to minimize glottic gaps, thus improving vocalization efficiency even in the presence of impaired respiratory effort. Conversely, the autonomic dysfunction that characterizes MSA results in upper airway obstruction, and thus surgical management focuses primarily on maintaining an adequate airway, which frequently necessitates tracheostomy.

Levodopa Responsiveness in Adult-onset Lower Limb Dystonia is Associated with the Development of Parkinson’s Disease

<strong>Background:</strong> Adult-onset primary lower limb dystonia (AOPLLD) has been reported as an early sign of Parkinson’s disease (PD) or Parkinson-plus syndrome in case series. No prior systematic analysis has assessed clinical clues predicting later development of PD or Parkinson-plus syndrome. <strong>Methods:</strong> We identified patients with AOPLLD from medical records. We excluded patients who had not been diagnosed by a neurologist, and who had a preexisting diagnosis of PD, psychogenic, or secondary dystonia. Records were subdivided into those who later developed PD or Parkinson-plus disorders and those who did not. The following clinical characteristics were compared between the two groups: dystonia onset age, type of dystonia, levodopa response, anticholinergic response, and family history of Parkinsonism or tremor. <strong>Results:</strong> Twenty-two AOPLLD patients were identified: 77% female; the median dystonia onset age was 53 years. Eight (37%) developed Parkinson’s disease; 2 (9%) developed corticobasal syndrome. Twelve patients (54%) did not develop Parkinsonism after a median follow-up period of 1.5 years. There was a significant difference in leg dystonia levodopa response between the two groups (p=0.02). <strong>Conclusion:</strong> In patients with AOPLLD, leg dystonia with levodopa response is associated with the future development of PD.

Levodopa Responsiveness in Adult-onset Lower Limb Dystonia is Associated with the Development of Parkinson's Disease.

Adult-onset primary lower limb dystonia (AOPLLD) has been reported as an early sign of Parkinson's disease (PD) or Parkinson-plus syndrome in case series. No prior systematic analysis has assessed clinical clues predicting later development of PD or Parkinson-plus syndrome. We identified patients with AOPLLD from medical records. We excluded patients who had not been diagnosed by a neurologist, and who had a pre-existing diagnosis of PD, psychogenic, or secondary dystonia. Records were subdivided into those who later developed PD or Parkinson-plus disorders and those who did not. The following clinical characteristics were compared between the two groups: dystonia onset age, type of dystonia, levodopa response, anticholinergic response, and family history of Parkinsonism or tremor. Twenty-two AOPLLD patients were identified: 77% female; the median dystonia onset age was 53 years. Eight (37%) developed Parkinson's disease; 2 (9%) developed corticobasal syndrome. Twelve patients (54%) did not develop Parkinsonism after a median follow-up period of 1.5 years. There was a significant difference in leg dystonia levodopa response between the two groups (p = 0.02). In patients with AOPLLD, leg dystonia with levodopa response is associated with the future development of PD.

Accuracy of the national institute for neurological disorders and stroke/society for progressive supranuclear palsy and neuroprotection and natural history in Parkinson plus syndromes criteria for the diagnosis of progressive supranuclear palsy

Autopsy is the diagnostic gold standard for progressive supranuclear palsy (PSP). The National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy (NINDS-SPSP) criteria for the clinical diagnosis of "probable" PSP are thought to possess high specificity and low sensitivity. The NINDS-SPSP criteria for "possible" PSP are considered to increase sensitivity at the expense of specificity. The Neuroprotection and Natural History in Parkinson Plus Syndromes (NNIPPS) criteria are intended to improve sensitivity while maintaining high specificity. The aim of this study was to conduct a clinicopathological evaluation of the NINDS-SPSP and NNIPPS criteria in tertiary neurological centers. Defined clinical features and their year of onset were recorded by chart review in neuropathologically diagnosed patients with PSP, Parkinsons's disease (PD), MSA parkinsonism and corticobasal degeneration from four European brain banks. Fulfillment of the clinical diagnostic criteria was verified for each year after disease onset and for the final antemortem record. We analyzed 98 PSP patients and 46 disease controls. The NINDS-SPSP "probable" criteria yielded shorter time to diagnosis, slightly higher specificity and positive predictive value (PPV), and similar sensitivity, compared with the NNIPPS criteria. Unexpectedly, the NINDS-SPSP "possible" criteria yielded the lowest sensitivity, specificity, and PPV. A combination of NINDS-SPSP possible and probable criteria yielded the highest sensitivity. We suggest that the NINDS-SPSP probable criteria might be preferred for recruitment of patients for clinical trials, where an early and specific diagnosis is important. For routine clinical care, where high sensitivity is crucial, a combination of NINDS possible and probable criteria might be preferred.

Translational Neurodegeneration, a platform to share knowledge and experience in translational study of neurodegenerative diseases

Translational Neurodegeneration, a platform to share knowledge and experience in translational study of neurodegenerative diseases