The Experts below are selected from a list of 7452 Experts worldwide ranked by ideXlab platform
Irene Litvan - One of the best experts on this subject based on the ideXlab platform.
-
behavioral abnormalities in progressive Supranuclear Palsy
Psychiatry Research-neuroimaging, 2013Co-Authors: Adam Gerstenecker, Kevin Duff, Benjamin T Mast, Irene LitvanAbstract:Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disorder in which, classically, patients present with postural instability and falls, parkinsonism, and slowing of vertical saccades. PSP patients typically have deficits in cognitive functioning, difficulties with most daily activities, and present with notable behavioral disturbances—particularly apathy, impulsivity, and irritability. Using data from 154 patients meeting criteria for clinically probable PSP, domain and total scores of the Neuropsychiatric Inventory were examined and compared to demographics, disease severity, cognition, and motor features. Behavioral abnormalities were common in this cohort of PSP patients, with more than half experiencing apathy, depression, and sleeping problems, and approximately one third displaying agitation, irritability, disinhibition, and eating problems. Few clinical correlates of neuropsychiatric symptoms were observed in this cohort. Given the prevalence of neuropsychiatric symptoms in PSP, these patients are expected to be frequently seen by psychiatrists and other mental health professionals for symptom management and increased quality of life. Clinical trials are clearly needed to address the neuropsychiatric morbidity in these patients.
-
executive dysfunction is the primary cognitive impairment in progressive Supranuclear Palsy
Archives of Clinical Neuropsychology, 2013Co-Authors: Adam Gerstenecker, Kevin Duff, Benjamin T Mast, Tanis J Ferman, Irene LitvanAbstract:Cognitive difficulties appear to be a more prevalent clinical feature in progressive Supranuclear Palsy (PSP) than previously thought, and significant cognitive impairment is prevalent in a majority of patients PSP patients not considered clinically demented. The neurocognitive performance of 200 patients with PSP across multiple sites was examined with a variety of commonly used neuropsychological tests. Results indicate primary executive dysfunction (e.g., 74% impaired on the Frontal Assessment Battery, 55% impaired on Initiation/Perseveration subscale of the Dementia Rating Scale), with milder difficulties in memory, construction, and naming. These results have important clinical implications for providers following patients with PSP.
-
performance on the dementia rating scale in parkinson s disease with dementia and dementia with lewy bodies comparison with progressive Supranuclear Palsy and alzheimer s disease
Journal of Neurology Neurosurgery and Psychiatry, 2003Co-Authors: Dag Aarsland, Irene Litvan, David P Salmon, Douglas Galasko, Tore Wentzellarsen, Jan Petter LarsenAbstract:Background: The relation between dementia with Lewy bodies (DLB) and Parkinson’s disease with dementia (PDD) is unknown. Objectives: To compare the cognitive profiles of patients with DLB and PDD, and compare those with the performance of patients with a subcortical dementia (progressive Supranuclear Palsy) and a cortical dementia (Alzheimer’s disease). Design: Survey of cognitive features. Setting: General community in Rogaland county, Norway, and a university dementia and movement disorder research centre in the USA. Patients: 60 patients with DLB, 35 with PDD, 49 with progressive Supranuclear Palsy, and 29 with Alzheimer’s disease, diagnosed by either standardised clinical procedures and criteria (all PDD and Alzheimer cases and 76% of cases of progressive Supranuclear Palsy), or necropsy (all DLB cases and 24% of cases of progressive Supranuclear Palsy). Level of dementia severity was matched using the total score on the dementia rating scale adjusted for age and education. Main outcome measures: Dementia rating scale subscores corrected for age. Results: No significant differences between the dementia rating scale subscores in the PDD and DLB groups were found in the severely demented patients; in patients with mild to moderate dementia the conceptualisation subscore was higher in PDD than in DLB (p = 0.03). Compared with Alzheimer’s disease, PDD and DLB had higher memory subscores (p < 0.001) but lower initiation and perseveration (p = 0.008 and p=0.021) and construction subscores (p = 0.009 and p = 0.001). DLB patients had a lower conceptualisation subscore (p = 0.004). Compared with progressive Supranuclear Palsy, PDD and DLB patients had lower memory subscores (p < 0.001). Conclusions: The cognitive profiles of patients with DLB and PDD were similar, but they differed from those of patients with Alzheimer’s disease and progressive Supranuclear Palsy. The cognitive pattern in DLB and PDD probably reflects the superimposition of subcortical deficits upon deficits typically associated with Alzheimer’s disease.
-
comparison of apraxia in corticobasal degeneration and progressive Supranuclear Palsy
Neurology, 2001Co-Authors: V Pharr, B Fantie, M Stark, Irene Litvan, Bob Uttl, Jordan GrafmanAbstract:Objective: To describe ideomotor apraxia in patients with corticobasal degeneration and those with progressive Supranuclear Palsy, two parkinsonian disorders that are often misdiagnosed due to the overlap in their clinical features, and to determine whether systematic apraxia testing is useful for differential diagnosis. Methods: Fourteen patients fulfilling National Institute of Neurological Disorders and Stroke–Society for Progressive Supranuclear Palsy clinical criteria for progressive Supranuclear Palsy, 13 patients fulfilling modified Lang criteria for corticobasal degeneration, and 12 normal healthy control subjects were given the Test of Oral and Limb Apraxia, which was scored according to the Florida Apraxia Battery for occurrence of various types of apraxic errors. Results: Both patients with progressive Supranuclear Palsy and corticobasal degeneration committed a greater number of apraxic errors than normal healthy control subjects on both transitive and intransitive tasks (p Conclusions: Patients with corticobasal degeneration show more severe ideomotor apraxia than patients with progressive Supranuclear Palsy, and systematic assessment of ideomotor apraxia facilitates the differential diagnosis between patients with progressive Supranuclear Palsy and those with corticobasal degeneration.
-
association of an extended haplotype in the tau gene with progressive Supranuclear Palsy
Human Molecular Genetics, 1999Co-Authors: Matt Baker, Dennis W Dickson, Irene Litvan, Henry Houlden, Jennifer Adamson, Jordi Pereztur, John Hardy, T Lynch, Eileen H Bigio, Mike HuttonAbstract:We describe two extended haplotypes that cover the human tau gene. In a total of similar to 200 unrelated caucasian individuals there is complete disequilibrium between polymorphisms which span the gene (which covers similar to 100 kb of DNA), This suggests that the establishment of the two haplotypes was an ancient event and either that recombination is suppressed in this region, or that recombinant genes are selected against. Furthermore, we shaw that the more common haplotype (H1) is significantly over-represented in patients with progressive Supranuclear Palsy (PSP), extending earlier reports of an association between an intronic dinucleotide polymorphism and PSP.
Anthony E Lang - One of the best experts on this subject based on the ideXlab platform.
-
positron emission tomography imaging of tau pathology in progressive Supranuclear Palsy
Journal of Cerebral Blood Flow and Metabolism, 2017Co-Authors: Sarah Coakeley, Yuko Koshimori, Pablo Rusjan, Madeleine Harris, Christine Ghadery, Anthony E LangAbstract:Progressive Supranuclear Palsy is a rare form of atypical Parkinsonism that differs neuropathologically from other parkinsonian disorders. While many parkinsonian disorders such as Parkinson’s disease, Lewy body dementia, and multiple system atrophy are classified as synucleinopathies, progressive Supranuclear Palsy is coined a tauopathy due to the aggregation of pathological tau in the brain. [18F]AV-1451 (also known as [18F]-T807) is a positron emission tomography radiotracer that binds to paired helical filaments of tau in Alzheimer’s disease. We investigated whether [18F]AV-1451 could be used as biomarker for the diagnosis and disease progression monitoring in progressive Supranuclear Palsy. Six progressive Supranuclear Palsy, six Parkinson’s disease, and 10 age-matched healthy controls were recruited. An anatomical MRI and a 90-min PET scan, using [18F]AV-1451, were acquired from all participants. The standardized uptake value ratio from 60 to 90 min post-injection was calculated in each region of in...
-
positron emission tomography imaging of tau pathology in progressive Supranuclear Palsy
Journal of Cerebral Blood Flow and Metabolism, 2017Co-Authors: Sarah Coakeley, Yuko Koshimori, Pablo Rusjan, Madeleine Harris, Christine Ghadery, Anthony E LangAbstract:Progressive Supranuclear Palsy is a rare form of atypical Parkinsonism that differs neuropathologically from other parkinsonian disorders. While many parkinsonian disorders such as Parkinson’s disease, Lewy body dementia, and multiple system atrophy are classified as synucleinopathies, progressive Supranuclear Palsy is coined a tauopathy due to the aggregation of pathological tau in the brain. [18F]AV-1451 (also known as [18F]-T807) is a positron emission tomography radiotracer that binds to paired helical filaments of tau in Alzheimer’s disease. We investigated whether [18F]AV-1451 could be used as biomarker for the diagnosis and disease progression monitoring in progressive Supranuclear Palsy. Six progressive Supranuclear Palsy, six Parkinson’s disease, and 10 age-matched healthy controls were recruited. An anatomical MRI and a 90-min PET scan, using [18F]AV-1451, were acquired from all participants. The standardized uptake value ratio from 60 to 90 min post-injection was calculated in each region of in...
-
corticobasal ganglionic degeneration and progressive Supranuclear Palsy presenting with cognitive decline
Brain Pathology, 2006Co-Authors: Catherine Bergeron, Andrea Davis, Anthony E LangAbstract:: Corticobasal ganglionic degeneration (CBGD) and progressive Supranuclear Palsy (PSP) were originally described in the sixties as predominantly motor syndromes. Over the years, the detailed study of additional cases of CBGD has shown that it is a distinctive histological entity which can often present as dementia or aphasia. Although some pathological features of CBGD overlap with those of other forms of non-Alzheimer non-Lewy body dementia, the distribution and relative number of these abnormalities and the distinctive pattern of tau immunodeposits allows the distinction of CBGD from Pick's disease and fronto-temporal dementia. In contrast, PSP only rarely presents with prominent dementia or behavioral changes. In these unusual PSP cases, care must be taken to exclude the diagnoses of CBGD and familial tangle-only dementia.
-
treatment of progressive Supranuclear Palsy and corticobasal degeneration
Movement Disorders, 2005Co-Authors: Anthony E LangAbstract:Success in treating patients with progressive Supranuclear Palsy and corticobasal degeneration remains exceedingly low. This finding probably relates to the widespread distribution of the pathological changes that account for the varied and complex spectrum of clinical manifestations. Dopaminergic drugs are regularly used for the parkinsonian features; however, these rarely result in more than modest benefit, and when better or sustained responses are obtained, as sometimes occurs in progressive Supranuclear Palsy, the clinical features are atypical and diagnosis is often delayed or not made in life. A variety of other treatments have been used in both disorders, sometimes directed at other specific features such as dystonia or myoclonus, and these treatments will be reviewed. Greater success in treating these disorders will require advances in our understanding of their cause(s) or the pathogenetic mechanisms underlying the neurodegenerative processes. The similarities in the molecular pathology of these four-repeat tauopathies suggests that important advances in the management of one will have a definite impact on the treatment of the other.
Sarah Coakeley - One of the best experts on this subject based on the ideXlab platform.
-
positron emission tomography imaging of tau pathology in progressive Supranuclear Palsy
Journal of Cerebral Blood Flow and Metabolism, 2017Co-Authors: Sarah Coakeley, Yuko Koshimori, Pablo Rusjan, Madeleine Harris, Christine Ghadery, Anthony E LangAbstract:Progressive Supranuclear Palsy is a rare form of atypical Parkinsonism that differs neuropathologically from other parkinsonian disorders. While many parkinsonian disorders such as Parkinson’s disease, Lewy body dementia, and multiple system atrophy are classified as synucleinopathies, progressive Supranuclear Palsy is coined a tauopathy due to the aggregation of pathological tau in the brain. [18F]AV-1451 (also known as [18F]-T807) is a positron emission tomography radiotracer that binds to paired helical filaments of tau in Alzheimer’s disease. We investigated whether [18F]AV-1451 could be used as biomarker for the diagnosis and disease progression monitoring in progressive Supranuclear Palsy. Six progressive Supranuclear Palsy, six Parkinson’s disease, and 10 age-matched healthy controls were recruited. An anatomical MRI and a 90-min PET scan, using [18F]AV-1451, were acquired from all participants. The standardized uptake value ratio from 60 to 90 min post-injection was calculated in each region of in...
-
positron emission tomography imaging of tau pathology in progressive Supranuclear Palsy
Journal of Cerebral Blood Flow and Metabolism, 2017Co-Authors: Sarah Coakeley, Yuko Koshimori, Pablo Rusjan, Madeleine Harris, Christine Ghadery, Anthony E LangAbstract:Progressive Supranuclear Palsy is a rare form of atypical Parkinsonism that differs neuropathologically from other parkinsonian disorders. While many parkinsonian disorders such as Parkinson’s disease, Lewy body dementia, and multiple system atrophy are classified as synucleinopathies, progressive Supranuclear Palsy is coined a tauopathy due to the aggregation of pathological tau in the brain. [18F]AV-1451 (also known as [18F]-T807) is a positron emission tomography radiotracer that binds to paired helical filaments of tau in Alzheimer’s disease. We investigated whether [18F]AV-1451 could be used as biomarker for the diagnosis and disease progression monitoring in progressive Supranuclear Palsy. Six progressive Supranuclear Palsy, six Parkinson’s disease, and 10 age-matched healthy controls were recruited. An anatomical MRI and a 90-min PET scan, using [18F]AV-1451, were acquired from all participants. The standardized uptake value ratio from 60 to 90 min post-injection was calculated in each region of in...
Helen Ling - One of the best experts on this subject based on the ideXlab platform.
-
clinical approach to progressive Supranuclear Palsy
Journal of Movement Disorders, 2016Co-Authors: Helen LingAbstract:Sixty years ago, Steele, Richardson and Olszewski designated progressive Supranuclear Palsy (PSP) as a new clinicopathological entity in their seminal paper. Since then, in addition to the classic Richardson's syndrome (RS), different clinical phenotypic presentations have been linked with this four-repeat tauopathy. The clinical heterogeneity is associated with variability of regional distribution and severity of abnormal tau accumulation and neuronal loss. In PSP subtypes, the presence of certain clinical pointers may be useful for antemortem prediction of the underlying PSP-tau pathology. Midbrain atrophy on conventional MRI correlates with the clinical phenotype of RS but is not predictive of PSP pathology. Cerebrospinal fluid biomarkers and tau ligand positron emission tomography are promising biomarkers of PSP. A multidisciplinary approach to meet the patients' complex needs is the current core treatment strategy for this devastating disorder.
-
concomitant progressive Supranuclear Palsy and chronic traumatic encephalopathy in a boxer
Acta neuropathologica communications, 2014Co-Authors: Helen Ling, Andrew J Lees, Eleanna Kara, Tamas Revesz, Gordon T Plant, Davide MartinoAbstract:We report the case of a 75-year-old ex-professional boxer who developed diplopia and eye movement abnormalities in his 60’s followed by memory impairment, low mood and recurrent falls. Examination shortly before death revealed hypomimia, dysarthria, vertical Supranuclear gaze Palsy and impaired postural reflexes. Pathological examination demonstrated 4-repeat tau neuronal and glial lesions, including tufted astrocytes, consistent with a diagnosis of progressive Supranuclear Palsy. In addition, neurofibrillary tangles composed of mixed 3-repeat and 4-repeat tau and astrocytic tangles in a distribution highly suggestive of chronic traumatic encephalopathy were observed together with limbic TDP-43 pathology. Possible mechanisms for the co-occurrence of these two tau pathologies are discussed.
-
characteristics of progressive Supranuclear Palsy presenting with corticobasal syndrome a cortical variant
Neuropathology and Applied Neurobiology, 2014Co-Authors: Helen Ling, Luke A Massey, Andrew J Lees, Janice L Holton, R De Silva, R Courtney, G Hondhamuni, Nin Bajaj, James Lowe, Tamas ReveszAbstract:Since the first description of the classical presentation of progressive Supranuclear Palsy (PSP) in 1963, now known as Richardson's syndrome (PSP-RS), several distinct clinical syndromes have been associated with PSP-tau pathology. Like other neurodegenerative disorders, the severity and distribution of phosphorylated tau pathology are closely associated with the clinical heterogeneity of PSP variants. PSP with corticobasal syndrome presentation (PSP-CBS) was reported to have more tau load in the mid-frontal and inferior-parietal cortices than in PSP-RS. However, it is uncertain if differences exist in the distribution of tau pathology in other brain regions or if the overall tau load is increased in the brains of PSP-CBS.
-
the midbrain to pons ratio a simple and specific mri sign of progressive Supranuclear Palsy
Neurology, 2013Co-Authors: Luke A Massey, Helen Ling, Tamas Revesz, David R Williams, Hans Rolf Jager, Dominic C Paviour, Sean S Osullivan, Constantinos Kallis, Janice L Holton, David J BurnAbstract:MRI-based measurements used to diagnose progressive Supranuclear Palsy (PSP) typically lack pathologic verification and are not easy to use routinely. We aimed to develop in histologically proven disease a simple measure of the midbrain and pons on sagittal MRI to identify PSP.
-
hypokinesia without decrement distinguishes progressive Supranuclear Palsy from parkinson s disease
Brain, 2012Co-Authors: Helen Ling, Luke A Massey, Andrew J Lees, Peter BrownAbstract:Repetitive finger tapping is commonly used to assess bradykinesia in Parkinson's disease. The Queen Square Brain Bank diagnostic criterion of Parkinson's disease defines bradykinesia as ‘slowness of initiation with progressive reduction in speed and amplitude of repetitive action’. Although progressive Supranuclear Palsy is considered an atypical parkinsonian syndrome, it is not known whether patients with progressive Supranuclear Palsy have criteria-defined bradykinesia. This study objectively assessed repetitive finger tap performance and handwriting in patients with Parkinson's disease ( n = 15), progressive Supranuclear Palsy ( n = 9) and healthy age- and gender-matched controls ( n = 16). The motion of the hand and digits was recorded in 3D during 15-s repetitive index finger-to-thumb tapping trials. The main finding was hypokinesia without decrement in patients with progressive Supranuclear Palsy, which differed from the finger tap pattern in Parkinson's disease. Average finger separation amplitude in progressive Supranuclear Palsy was less than half of that in controls and Parkinson's disease ( P < 0.001 in both cases). Change in tap amplitude over consecutive taps was computed by linear regression. The average amplitude slope in progressive Supranuclear Palsy was nearly zero (0.01°/cycle) indicating a lack of decrement, which differed from the negative slope in patients with Parkinson's disease OFF levodopa (−0.20°/cycle, P = 0.002). ‘Hypokinesia’, defined as <50% of control group's mean amplitude, combined with ‘absence of decrement’, defined as mean positive amplitude slope, were identified in 87% of finger tap trials in the progressive Supranuclear Palsy group and only 12% in the Parkinson's disease OFF levodopa group. In progressive Supranuclear Palsy, the mean amplitude was not correlated with disease duration or other clinimetric scores. In Parkinson's disease, finger tap pattern was compatible with criteria-defined bradykinesia, characterized by slowness with progressive reduction in amplitude and speed and increased variability in speed throughout the tap trial. In Parkinson's disease, smaller amplitude, slower speed and greater speed variability were all associated with a more severe Unified Parkinson's Disease Rating Scale motor score. Analyses of handwriting showed that micrographia, defined as smaller than 50% of the control group's mean script size, was present in 75% of patients with progressive Supranuclear Palsy and 15% of patients with Parkinson's disease ( P = 0.022). Most scripts performed by patients with progressive Supranuclear Palsy did not exhibit decrements in script size. In conclusion, patients with progressive Supranuclear Palsy have a specific finger tap pattern of ‘hypokinesia without decrement’ and they do not have criteria-defined limb bradykinesia. Similarly, ‘micrographia’ and ‘lack of decrement in script size’ are also more common in progressive Supranuclear Palsy than in Parkinson's disease. * Abbreviations : PD-OFF : Parkinson's disease OFF levodopa medication PD-ON : Parkinson's disease ON levodopa medication PSP : progressive Supranuclear Palsy UPDRS : Unified Parkinson's disease rating scale
Andrew J Lees - One of the best experts on this subject based on the ideXlab platform.
-
concomitant progressive Supranuclear Palsy and chronic traumatic encephalopathy in a boxer
Acta neuropathologica communications, 2014Co-Authors: Helen Ling, Andrew J Lees, Eleanna Kara, Tamas Revesz, Gordon T Plant, Davide MartinoAbstract:We report the case of a 75-year-old ex-professional boxer who developed diplopia and eye movement abnormalities in his 60’s followed by memory impairment, low mood and recurrent falls. Examination shortly before death revealed hypomimia, dysarthria, vertical Supranuclear gaze Palsy and impaired postural reflexes. Pathological examination demonstrated 4-repeat tau neuronal and glial lesions, including tufted astrocytes, consistent with a diagnosis of progressive Supranuclear Palsy. In addition, neurofibrillary tangles composed of mixed 3-repeat and 4-repeat tau and astrocytic tangles in a distribution highly suggestive of chronic traumatic encephalopathy were observed together with limbic TDP-43 pathology. Possible mechanisms for the co-occurrence of these two tau pathologies are discussed.
-
characteristics of progressive Supranuclear Palsy presenting with corticobasal syndrome a cortical variant
Neuropathology and Applied Neurobiology, 2014Co-Authors: Helen Ling, Luke A Massey, Andrew J Lees, Janice L Holton, R De Silva, R Courtney, G Hondhamuni, Nin Bajaj, James Lowe, Tamas ReveszAbstract:Since the first description of the classical presentation of progressive Supranuclear Palsy (PSP) in 1963, now known as Richardson's syndrome (PSP-RS), several distinct clinical syndromes have been associated with PSP-tau pathology. Like other neurodegenerative disorders, the severity and distribution of phosphorylated tau pathology are closely associated with the clinical heterogeneity of PSP variants. PSP with corticobasal syndrome presentation (PSP-CBS) was reported to have more tau load in the mid-frontal and inferior-parietal cortices than in PSP-RS. However, it is uncertain if differences exist in the distribution of tau pathology in other brain regions or if the overall tau load is increased in the brains of PSP-CBS.
-
hypokinesia without decrement distinguishes progressive Supranuclear Palsy from parkinson s disease
Brain, 2012Co-Authors: Helen Ling, Luke A Massey, Andrew J Lees, Peter BrownAbstract:Repetitive finger tapping is commonly used to assess bradykinesia in Parkinson's disease. The Queen Square Brain Bank diagnostic criterion of Parkinson's disease defines bradykinesia as ‘slowness of initiation with progressive reduction in speed and amplitude of repetitive action’. Although progressive Supranuclear Palsy is considered an atypical parkinsonian syndrome, it is not known whether patients with progressive Supranuclear Palsy have criteria-defined bradykinesia. This study objectively assessed repetitive finger tap performance and handwriting in patients with Parkinson's disease ( n = 15), progressive Supranuclear Palsy ( n = 9) and healthy age- and gender-matched controls ( n = 16). The motion of the hand and digits was recorded in 3D during 15-s repetitive index finger-to-thumb tapping trials. The main finding was hypokinesia without decrement in patients with progressive Supranuclear Palsy, which differed from the finger tap pattern in Parkinson's disease. Average finger separation amplitude in progressive Supranuclear Palsy was less than half of that in controls and Parkinson's disease ( P < 0.001 in both cases). Change in tap amplitude over consecutive taps was computed by linear regression. The average amplitude slope in progressive Supranuclear Palsy was nearly zero (0.01°/cycle) indicating a lack of decrement, which differed from the negative slope in patients with Parkinson's disease OFF levodopa (−0.20°/cycle, P = 0.002). ‘Hypokinesia’, defined as <50% of control group's mean amplitude, combined with ‘absence of decrement’, defined as mean positive amplitude slope, were identified in 87% of finger tap trials in the progressive Supranuclear Palsy group and only 12% in the Parkinson's disease OFF levodopa group. In progressive Supranuclear Palsy, the mean amplitude was not correlated with disease duration or other clinimetric scores. In Parkinson's disease, finger tap pattern was compatible with criteria-defined bradykinesia, characterized by slowness with progressive reduction in amplitude and speed and increased variability in speed throughout the tap trial. In Parkinson's disease, smaller amplitude, slower speed and greater speed variability were all associated with a more severe Unified Parkinson's Disease Rating Scale motor score. Analyses of handwriting showed that micrographia, defined as smaller than 50% of the control group's mean script size, was present in 75% of patients with progressive Supranuclear Palsy and 15% of patients with Parkinson's disease ( P = 0.022). Most scripts performed by patients with progressive Supranuclear Palsy did not exhibit decrements in script size. In conclusion, patients with progressive Supranuclear Palsy have a specific finger tap pattern of ‘hypokinesia without decrement’ and they do not have criteria-defined limb bradykinesia. Similarly, ‘micrographia’ and ‘lack of decrement in script size’ are also more common in progressive Supranuclear Palsy than in Parkinson's disease. * Abbreviations : PD-OFF : Parkinson's disease OFF levodopa medication PD-ON : Parkinson's disease ON levodopa medication PSP : progressive Supranuclear Palsy UPDRS : Unified Parkinson's disease rating scale
-
hypokinesia without decrement distinguishes progressive Supranuclear Palsy from parkinson s disease
Brain, 2012Co-Authors: Helen Ling, Luke A Massey, Andrew J Lees, Peter Brown, B L DayAbstract:Repetitive finger tapping is commonly used to assess bradykinesia in Parkinson's disease. The Queen Square Brain Bank diagnostic criterion of Parkinson's disease defines bradykinesia as 'slowness of initiation with progressive reduction in speed and amplitude of repetitive action'. Although progressive Supranuclear Palsy is considered an atypical parkinsonian syndrome, it is not known whether patients with progressive Supranuclear Palsy have criteria-defined bradykinesia. This study objectively assessed repetitive finger tap performance and handwriting in patients with Parkinson's disease (n = 15), progressive Supranuclear Palsy (n = 9) and healthy age- and gender-matched controls (n = 16). The motion of the hand and digits was recorded in 3D during 15-s repetitive index finger-to-thumb tapping trials. The main finding was hypokinesia without decrement in patients with progressive Supranuclear Palsy, which differed from the finger tap pattern in Parkinson's disease. Average finger separation amplitude in progressive Supranuclear Palsy was less than half of that in controls and Parkinson's disease (P < 0.001 in both cases). Change in tap amplitude over consecutive taps was computed by linear regression. The average amplitude slope in progressive Supranuclear Palsy was nearly zero (0.01°/cycle) indicating a lack of decrement, which differed from the negative slope in patients with Parkinson's disease OFF levodopa (-0.20°/cycle, P = 0.002). 'Hypokinesia', defined as <50% of control group's mean amplitude, combined with 'absence of decrement', defined as mean positive amplitude slope, were identified in 87% of finger tap trials in the progressive Supranuclear Palsy group and only 12% in the Parkinson's disease OFF levodopa group. In progressive Supranuclear Palsy, the mean amplitude was not correlated with disease duration or other clinimetric scores. In Parkinson's disease, finger tap pattern was compatible with criteria-defined bradykinesia, characterized by slowness with progressive reduction in amplitude and speed and increased variability in speed throughout the tap trial. In Parkinson's disease, smaller amplitude, slower speed and greater speed variability were all associated with a more severe Unified Parkinson's Disease Rating Scale motor score. Analyses of handwriting showed that micrographia, defined as smaller than 50% of the control group's mean script size, was present in 75% of patients with progressive Supranuclear Palsy and 15% of patients with Parkinson's disease (P = 0.022). Most scripts performed by patients with progressive Supranuclear Palsy did not exhibit decrements in script size. In conclusion, patients with progressive Supranuclear Palsy have a specific finger tap pattern of 'hypokinesia without decrement' and they do not have criteria-defined limb bradykinesia. Similarly, 'micrographia' and 'lack of decrement in script size' are also more common in progressive Supranuclear Palsy than in Parkinson's disease.
-
progressive Supranuclear Palsy clinicopathological concepts and diagnostic challenges
Lancet Neurology, 2009Co-Authors: David R Williams, Andrew J LeesAbstract:Progressive Supranuclear Palsy (PSP) is a clinical syndrome comprising Supranuclear Palsy, postural instability, and mild dementia. Neuropathologically, PSP is defined by the accumulation of neurofibrillary tangles. Since the first description of PSP in 1963, several distinct clinical syndromes have been described that are associated with PSP; this discovery challenges the traditional clinicopathological definition and complicates diagnosis in the absence of a reliable, disease-specific biomarker. We review the emerging nosology in this field and contrast the clinical and pathological characteristics of the different disease subgroups. These new insights emphasise that the pathological events and processes that lead to the accumulation of phosphorylated tau protein in the brain are best considered as dynamic processes that can develop at different rates, leading to different clinical phenomena. Moreover, for patients for whom the diagnosis is unclear, clinicians must continue to describe accurately the clinical picture of each individual, rather than label them with inaccurate diagnostic categories, such as atypical parkinsonism or PSP mimics. In this way, the development of the clinical features can be informative in assigning less common nosological categories that give clues to the underlying pathology and an understanding of the expected clinical course.
