Society For Progressive Supranuclear Palsy Research Articles

Understanding the relationship between freezing of gait and other progressive supranuclear palsy features

IntroductionFreezing of gait (FoG) leads to falls and reduces quality of life, but little is known about FoG in progressive supranuclear palsy (PSP). This study aim was to identify the clinical parameters associated with FoG in PSP patients. Methods349 patients meeting the National Institute for Neurological Disorders and Society for PSP (NINDS-SPSP) clinical diagnostic criteria were divided into two groups: PSP with FoG (n = 159) and PSP without FoG (n = 190). To determine if FoG in PSP associates with demographics, motor performance, visual difficulties, and executive function, we used the Frontal Assessment Battery (FAB), Mattis Dementia Rating Scale (DRS), Unified Parkinson's Disease Rating Scale (UPDRS), PSP Rating Scale (PSPRS), Modified Hoehn & Yahr staging, and Schwab and England Activities Daily Living (S&EADL) scale. UPDRS was used to identify FoG. Individual items of each clinical assessment with p-value < 0.05 in the univariate logistic regression analyses were included in the backward stepwise multivariate regression analysis. ResultsBoth groups were similar in demographics. 45.6% of patients had FoG, which was present at onset and increased with disease duration. There were no between-group significant associations between FoG and visual disturbances, executive function and overall cognition, but on univariate analyses, FoG was significantly associated with bradykinesia, rigidity, gait, and posture. In the multivariate model FoG was associated with disease duration and speech. ConclusionsOur findings indicate that disease duration and speech have the most significant association with FoG. These findings may suggest that FoG and speech difficulties in PSP share a similar pathophysiology.

Progressive Supranuclear Palsy with Predominant Cerebellar Ataxia.

Progressive supranuclear palsy (PSP) is characterized by supranuclear gaze palsy, dystonic rigidity of the neck and upper trunk, frequent falls and mild cognitive impairment. Cerebellar ataxia is one of the exclusion criteria given by the National Institute of Neurological Disorders and Stroke and the Society for Progressive Supranuclear Palsy. As a result, pathologically proven PSP patients exhibiting cerebellar ataxia have often been misdiagnosed with spinocerebellar degeneration, specifically multiple system atrophy with predominant cerebellar ataxia (MSA-C). However, more recently, it has been recognized that patients with PSP can present with truncal and limb ataxia as their initial symptom and/or main manifestation. These patients can be classified as having PSP with predominant cerebellar ataxia (PSP-C), a new subtype of PSP. Since the development of this classification, patients with PSP-C have been identified primarily in Asian countries, and it has been noted that this condition is very rare in Western communities. Furthermore, the clinical features of PSP-C have been identified, enabling it to be distinguished from other subtypes of PSP and MSA-C. In this review, we describe the clinical and neuropathological features of PSP-C. The hypothesized pathophysiology of cerebellar ataxia in PSP-C is also discussed.

Are the International Parkinson disease and Movement Disorder Society progressive supranuclear palsy (IPMDS-PSP) diagnostic criteria accurate enough to differentiate common PSP phenotypes?

The International Parkinson Disease and Movement Disorder Society PSP study group (IPMDS-PSP) recently published new clinical diagnostic criteria for progressive supranuclear palsy (PSP). Currently, there is no data regarding the accuracy of these sets of criteria for differentiating various PSP phenotypes. We discuss the accuracy of the IPMDS-PSP criteria for differentiation of patients with the PSP- Richardson phenotype (PSP-RS) from those with the PSP-Parkinsonism (PSP-P) using data from a sample of 274 clinically diagnosed PSP patients participating in the Environmental Genetic PSP (ENGENE-PSP) case control study. Using National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) criteria and the Williams criteria we categorized 259 of these patients as probable PSP-RS and 15 as PSP-P. The IPD-MDS PSP-RS and PSP-P criteria were unable to distinguish the PSP-RS from the PSP-P phenotypes in this sample. Nearly all (92.6%; 240 out of 259) the PSP-RS patients and over half (60%; 9 out of 15) of the PSP-P patients fulfilled both the IPMDS criteria for PSP-RS and PSP-P. Applying the newly proposed multiple allocation extinction rules decreased the number of overlapping diagnoses among the NINDS-SPSP PSP-RS patients, however problems remained in the PSP-P group. Diagnostic accuracy might be improved by modification of timelines for development of falls and other parkinsonian features.

Diagnostic validity of magnetic resonance parkinsonism index in differentiating patients with progressive supranuclear palsy from patients with Parkinson's disease

BackgroundProgressive supranuclear palsy is a neuropathologically defined disease, and many studies worked on detecting the diagnostic use of Magnetic resonance imaging. This article purposed to detect the diagnostic performance of Magnetic resonance parkinsonism index (MRPI). MethodsWe systematically searched electronic database PubMed for articles published since 1996 using the National Institute of Neurological Disorders and Stroke and Society for PSP (NINDS-SPSP) criteria as the diagnostic standard. Methodological quality was assessed by Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) and software Review Manager 5.3, software STATA 14.0 and meta-disc were applied in statistics analysis. ResultsTotally 14 articles were included in this article. MRPI is proved to have pooled sensitivity of 0.98, pooled specificity of 0.99 in differentiating patients with Progressive supranuclear palsy (PSP) from patients with Parkinson's disease (PD) and the area under the Receiver operating characteristic curve value was 1.00. ConclusionMRPI shows excellent performance in differentiating patients with PSP from patients with PD, the clinical usage of MRPI in auxiliary diagnosis of PSP is recommended.

Sensitivity and Specificity of Diagnostic Criteria for Progressive Supranuclear Palsy.

In 2017, the International Parkinson and Movement Disorder Society put forward new clinical criteria for the diagnosis of PSP, recognizing diverse PSP phenotypes. In this study, we compared the sensitivity and specificity of the new criteria with the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy criteria at different times. Patients with clinical parkinsonism, clinical and/or neuropathological diagnosis of PSP, were identified from the Society for Progressive Supranuclear Palsy brain bank. All patients had neuropathologic diagnoses and detailed clinical examination performed by a neurologist at 1 of the 3 Mayo Clinic sites, in Florida, Arizona, and Minnesota. Clinical symptoms and signs were abstracted retrospectively in a blinded fashion and used to determine whether patients met either diagnostic criterion. Patients were divided into early and late disease stage groups using a 3-year cutoff. A total of 129 patients were included, of whom 66 had PSP pathology (51%). The remainder had other neurodegenerative diseases. The overall sensitivity of the International Parkinson and Movement Disorder Society criteria was 87.9%, compared with 45.5% for the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy criteria, whereas the specificity of the International Parkinson and Movement Disorder Society probable PSP criteria was 85.7%, compared with 90.5% for the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy. Individual patients were noted to have features of multiple PSP phenotypes. The International Parkinson and Movement Disorder Society criteria recognize several phenotypes of progressive supranuclear palsy and hence have higher sensitivity than the previous criteria. © 2019 International Parkinson and Movement Disorder Society.

Atrophy in midbrain & cerebral/cerebellar pedunculi is characteristic for progressive supranuclear palsy - A double-validation whole-brain meta-analysis.

ObjectiveProgressive supranuclear palsy (PSP) is an atypical parkinsonian syndrome characterized by vertical gaze palsy and postural instability. Midbrain atrophy is suggested as a hallmark, but it has not been validated systematically in whole-brain imaging.MethodsWe conducted whole-brain meta-analyses identifying disease-related atrophy in structural MRI. Eighteen studies were identified (N = 315 PSP, 393 controls) and separated into gray or white matter analyses (15/12). All patients were diagnosed according to the National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP criteria, Litvan et al. (1996a)), which are now considered as PSP-Richardson syndrome (Höglinger et al., 2017). With overlay analyses, we double-validated two meta-analytical algorithms: anatomical likelihood estimation and seed-based D mapping. Additionally, we conducted region-of-interest effect size meta-analyses on radiological biomarkers and subtraction analyses differentiating PSP from Parkinson's disease.ResultsWhole brain meta-analyses revealed consistent gray matter atrophy in bilateral thalamus, anterior insulae, midbrain, and left caudate nucleus. White matter alterations were consistently detected in bilateral superior/middle cerebellar pedunculi, cerebral pedunculi, and midbrain atrophy. Region-of-interest meta-analyses demonstrated that midbrain metrics generally perform very well in distinguishing PSP from other parkinsonian syndromes with strong effect sizes. Subtraction analyses identified the midbrain as differentiating between PSP and Parkinson's disease.ConclusionsOur meta-analyses identify gray matter atrophy of the midbrain and white matter atrophy of the cerebral/cerebellar pedunculi and midbrain as characteristic for PSP. Results support the incorporation of structural MRI data, and particularly these structures, into the revised PSP diagnostic criteria.

Phenotypic Variants of Patients with Progressive Supranuclear Palsy.

Progressive supranuclear palsy (PSP) is a commonly observed disease among Parkinson plus syndromes, and its early and accurate diagnosis is usually not possible. PSP is currently reported to have eight different subtypes. This study aims to determine the phenotypic subtypes of PSP and reveal their accompanying characteristics. Eighteen patients who were diagnosed as having PSP according to the National Institute for Neurological Disorders and Society for PSP (NINDS-SPSP) criteria were included in the study. Initial symptoms of the patients, their initial diagnosis, course of the disease with time, cranial images, and predominant clinical features were assessed with regard to the subtypes of PSP. The mean age of the patients was 65.83 years. According to the NINDS-SPSP criteria, all patients were diagnosed as having probable PSP. An evaluation of the patients with respect to the subtypes showed that five different subtypes had predominant characteristics. Richardson's syndrome was found to be the most common subtype. Classifying PSP with regard to its phenotypes may be useful for informing clinicians about the diverse clinical presentations of PSP, thereby possibly improving the diagnostic sensitivity of the disease.

Diagnostic accuracy of Magnetic Resonance Parkinsonism Index in differentiating progressive supranuclear palsy from Parkinson's disease and controls in Indian patients.

An assessment of the sensitivity and specificity of magnetic resonance (MR) imaging measurements of midbrain, pons, middle cerebellar peduncles (MCPs), and superior cerebellar peduncles (SCPs) and MR Parkinsonism Index (MRPI) in differentiating progressive supranuclear palsy (PSP) from Parkinson's disease (PD) and controls was performed. The correlation of these MR imaging measurements with the duration and severity of disease in the Indian patients using the PSP rating scale (PSPRS) was also performed. Twenty-six consecutive patients were enrolled in this study, satisfying the diagnostic criteria by the National Institute for Neurological Disorders and Stroke, and the Society for PSP (NINDS-SPSP), along with 13 PD and 30 control patients. All PSP patients were assessed using the PSP rating scale and staging system. Radiologists were blinded to the clinical diagnoses. MRPI was calculated by multiplying the pons area/midbrain area ratio by MCP width/SCP width ratio. The midbrain/pons area (M/P) ratio was measured as the ratio of midbrain area to pons area. Mean MRPI in PSP patients (23.48 ± 9.61) was significantly higher than that in PD patients (9.07 ± 2.23) and controls (9.45 ± 1.87). In this study, MRPI was 100% sensitive, specific, and accurate in differentiating PSP from PD and was 96.3% sensitive, 100% specific, and 98.21% accurate in differentiating PSP from controls. No correlation was found between the duration of disease, PSP rating scale, PSP staging system, and MRPI in the present study. MRPI was only marginally superior to the M/P ratio in differentiating between PSP and PD patients on an individual basis. No overlapping values were observed in the PSP and PD patients. Magnetic Resonance Parkinsonism Index is more sensitive, specific, and accurate in differentiating PSP from PD in the early stages on an individual basis.

Progressive supra-nuclear palsy: frequency of cardinal extrapyramidal features at first presentation

ObjectivesCardinal extrapyramidal features of progressive supranuclear palsy (PSP) help in clinically differentiating this condition from Parkinson's disease and other Parkinsonian syndromes. However, not all extrapyramidal features may be initially present,...

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.

Clinical heterogeneity in progressive supranuclear palsy: Problems of clinical diagnostic criteria of NINDS-SPSP in a retrospective study of seven Japanese autopsy cases

Progressive supranuclear palsy (PSP) is known to display variable atypical clinical features. In the absence of clinical markers to diagnose PSP, neuropathological examination is the "gold standard" for diagnosis. We retrospectively investigated clinical features in seven autopsy-confirmed cases of PSP. Only three patients (42.9%) matched the clinical diagnostic criteria of PSP proposed by the National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) at the time of death. In addition, only one patient (14.3%) matched these criteria at the time of the initial symptoms. Such underdiagnosis of PSP was mainly caused by heterogeneity, variety of the timing, and presence of symptoms in exclusion criteria. The present study also demonstrated that the clinical features of PSP may change dramatically according to the disease stage. Target symptoms should be selected based on time and stage to optimize patient quality of life.

Progressive supranuclear palsy combined with Alzheimer's disease: A clinicopathological study of two autopsy cases

We present here the clinicopathological characteristics of two autopsy-confirmed cases comorbid of progressive supranuclear palsy (PSP) and Alzheimer's disease (AD). Histopathologically, the amount and distribution of neurofibrillary tangles (NFTs) in the basal ganglia and brainstem fulfilled the pathological criteria of PSP proposed by the National Institute of Neurological Disorders and Stroke--The Society for PSP (NINDS-SPSP). The Braak stages of senile plaques and NFTs were stage C and stage V in Case 1, and stage C and stage IV in Case 2. These neuropathological findings confirmed that the two patients had combined PSP with AD. Our patients presented clinically with executive dysfunction prior to memory disturbance as an early symptom. Not only neurological symptoms such as gait disturbance, supranuclear ophthalmoplegia and pseudobulbar palsy, but emotional and personality changes and delirium were prominent. Therefore, symptoms of subcortical dementia of PSP were more predominant than AD-related symptoms in the present two patients. Comorbid PSP and AD further complicates the clinical picture and makes clinical diagnosis even more difficult.

Measuring health-related quality of life in patients with progressive supranuclear palsy

The development of psychometrically sound health-related quality of life (Hr-QoL) instruments has made it possible to assess subjectively experienced Hr-QoL quantitatively, and to incorporate Hr-QoL as a measure in medical research and clinical trials. Hr-QoL in patients with progressive supranuclear palsy (PSP) has been measured using generic (not disease-specific) instruments, or Parkinson’s disease-specific scales. We give an overview of the development of a disease-specific Hr-QoL instrument for patients with PSP. Based on interviews with 27 patients with PSP a preliminary Hr-QoL instrument was developed and administered to over 300 people with PSP in the UK and US. Following psychometric analysis a rating scale with a physical and a mental subscale emerged. In this patient sample, both subscales satisfied criteria for scaling assumptions, acceptability, reliability and validity (correlations with other measures consistent with a priori hypotheses). The psychometric properties of this questionnaire are undergoing further evaluation. We are grateful to the Society for Progressive Supranuclear Palsy and The Progressive Supranuclear Palsy (PSP Europe) Association who supported this study, and to patients in both the UK and the US for taking the time and effort in participating in this research.

Atypical unclassifiable parkinsonism on Guadeloupe: An environmental toxic hypothesis

We characterize the clinical features of Parkinson's syndrome on Guadeloupe and describe possible environmental causes. Consecutive patients who were referred to the University Hospital at Pointe a Pitre with parkinsonism from September 1996 to May 2002 were included. All cases were examined in a standardized manner by a neurologist with a special interest in movement disorders and independently by 3 external movement disorders specialists, using standard operational clinical diagnostic criteria. The subjects were 265 patients with Parkinson's syndrome living on Guadeloupe, four fifths of whom had been referred by primary care physicians and one fifth by neurologists. The levodopa response was assessed after a minimum period of 1 month of continuous treatment. All patients had brain computed tomography or brain magnetic resonance imaging scans and detailed neuropsychological examinations. Of 265 patients, only 66 were classified as Parkinson's disease, whereas 58 fulfilled the National Institute of Neurological Disorders and Stroke (NINDS) and Society for Progressive Supranuclear Palsy (SPSP) criteria for progressive supranuclear palsy, 100 had unclassifiable parkinsonism, characterized by dopa-unresponsiveness, marked axial rigidity, relative symmetry of parkinsonian features, early dysarthria, and frontolimbic cognitive impairment. Within this group, early postural instability, dysarthria, a frontal behavior disorder, cortical or subcortical atrophy, pyramidal signs, axial rigidity, and family history of neurodegenerative disorders were associated with poorer prognosis. A very large number of unclassifiable cases of atypical parkinsonism that do not fulfill operational criteria for Parkinson's disease or other defined motor neurodegenerations has been observed on Guadeloupe. Most patients closely resemble descriptions of bodig from Guam. In both geographic isolates, an environmental cause has been discussed. Annonaceae fruits and herbal teas are consumed on both islands. These plants contain several neurotoxins, particularly acetogenins, which induce dopaminergic neuron loss in animals. Neuronal death involves cholinergic and dopaminergic cells of the substantia nigra and GABAergic neurons of the striatum, associated with microglial proliferation. The development of atypical parkinsonism in Guadeloupe and probably elsewhere, could result from synergistic toxicity, but acetogenins are probably the most potent neurotoxin, acting as mitochondrial complex I inhibitor.

Progressive supranuclear palsy in a sample of Brazilian population: clinical features of 16 patients.

Progressive supranuclear palsy (PSP) is an uncommon disorder characterized by marked postural instability, vertical gaze abnormalities and axial rigidity. The purpose of this study is to report the clinical features of 16 consecutive subjects seen over a 10-year period at a Movement Disorders Clinic. These subjects fulfilled criteria for probable PSP namely those of the National Institute of Neurologic Disorders and Stroke (NINDS) and the Society for PSP (SPSP). This patient-group represented 2.1% of all degenerative parkinsonians observed and the mean age of onset of the disease was 64.7 years (sd = +/- 7.2). Postural instability with falls was the most frequent initial feature presented in PSP patients (62.5%). The hallmark of the disease, the supranuclear vertical gaze palsy, appeared after 2.3 years of disease onset, and only 12.5% had such manifestation at the first evaluation. Transient tremor was observed with a relatively high frequency in this group (44%), but only 19% had rest tremor. Chronic dacryocystitis, probably related to a paucity of blinking, was observed in two patients as an inaugural manifestation. In the first evaluation, only 19% of the 16 patients were diagnosed as probable PSP. The mean interval prior to the final diagnosis was 2.4 years.

The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK.

We performed a study to estimate the point prevalence of progressive supranuclear palsy (PSP) in the UK at national, regional and community levels. A 'Russian doll' design was used in which the population denominator for each of the three studies was successively smaller, whilst the method of case ascertainment became increasingly more rigorous. The NINDS-SPSP (National Institute of Neurological Disorders and the Society for Progressive Supranuclear Palsy) diagnostic criteria for PSP were applied throughout the study for case definition. The national study identified cases using passive referral mechanisms [e.g. the British Neurological Surveillance Unit (BNSU), PSP (Europe) Association patient register]. We identified 577 cases of PSP, giving a national prevalence estimate of 1.0 per 100 000 [95% confidence interval (CI) 0.9-1.1]. The North of England regional study used active 'multiple source' case ascertainment from a collaborative network of neurologists and non-neurologists. We identified 80 cases of PSP in this study, giving a crude and age-adjusted prevalence of 3.1 (95% CI 2.4-3.8) and 2.4 (1.9-3.0) per 100 000, respectively. Of these 80 cases, 51 patients (65%) were referred initially to non-neurologists and 10 patients (13%) had not seen a neurologist at any stage of their illness. The proportion of female cases was significantly greater in the regional than in the national study (61% versus 44%; P < 0.02). Cases referred to non-neurologists were significantly older than those referred to neurologists in the regional study (median age 73 versus 69.5 years; P < 0.01). Patients in the community study were identified via diagnostic and therapeutic registers from a representative sample of general practices in Newcastle upon Tyne. We identified 17 cases of PSP, yielding crude and age-adjusted prevalences of 6.5 (95% CI 3.4-9.7) and 5.0 (95% CI 2.5-7.5) per 100 000, respectively. Seven of the 17 cases (41%) had not previously been diagnosed as PSP. This study suggests that PSP is more common than previously considered, is commonly misdiagnosed and that the majority of cases are not initially referred to neurologists. The study also confirms the importance of active and detailed case ascertainment in ensuring reliable prevalence estimates.

A comparison of tau protein in cerebrospinal fluid between corticobasal degeneration and progressive supranuclear palsy

Many clinical and pathological discussions have been focused on the difficulty of differential diagnosis between corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) in recent years. This study was conducted to evaluate the usefulness of tau proteins in cerebrospinal fluid (CSF) for the differentiation of these two diseases. Subjects consisted of 10 patients with CBD (four males and six females with a mean age of 67.9±5.8 years), 12 patients with PSP (eight males and four females with a mean age of 62.6±5.8 years) and 36 control subjects (CTL) (16 males and 20 females with a mean age of 65.8±9.9 years). The CBD group included patients with probable CBD, while all the patients in the PSP group satisfied the diagnostic criteria developed by the National Institute of Neurological Disorders and Stroke and Society for PSP (NINDS-SPSP). CSF tau proteins were measured with the sandwich ELISA method (Innogenetics, Belgium). The CSF tau protein level was 320.1±86.5 pg/ml in the CBD group, 151.5±52.7 pg/ml in the PSP group and 128.7±91.7 pg/ml in the CTL group. Significant differences were noted in tau protein levels between the CBD group and both the PSP group (P<0.001) and the CTL group (P<0.005). We suggested that the measurement of CSF tau proteins may be useful for the differentiation between CBD and PSP.

Caregiving in progressive supranuclear palsy.

Basic issues regarding factors influencing progressive supranuclear palsy (PSP) patient caregiver burden remain unresolved, including whether and how disease severity and duration influence caregiver burden. To examine the relation between PSP patient caregiver burden and disease severity, describe the time course of caregiver burden in relation to disease progression, and identify the contribution of other factors (e.g., patient memory and behavioral problems, caregiver gender) to caregiver burden. Mail survey of 180 caregivers of PSP patients (living at home and cared for by nonprofessionals) who were members of the Society for Progressive Supranuclear Palsy. The survey included the Burden Interview, an instrument measuring caregiver burden by inquiring how they feel about different aspects and demands of caregiving. Caregiver burden was related to both PSP disease severity (r = 0.40) and disability/need for assistance (r = 0.43). The relation between burden and disease duration was nonlinear, following the same time course as disease severity; the burden increased during the first 18 months postdiagnosis and leveled off thereafter. Women reported more burden than men (caregiver sex delta r2 = 0.05), even after controlling for disease severity and duration (delta r2 = 0.22) and patient gender (delta r2 < 0.01; NS), patient depression (delta r2 = 0.09), and aggressiveness (delta r2 = 0.02). Burden in PSP is related to disease severity, disease duration, and caregiver gender (even after controlling for patient's memory, patient depression, and aggression).

Characterizing swallowing abnormalities in progressive supranuclear palsy.

The dysphagia that occurs as an early sign of progressive supranuclear palsy (PSP), and which may predispose patients to aspiration pneumonia, has never been fully characterized. We evaluated 27 patients (mean +/- SEM: age, 64.9 +/- 1 years; symptom duration, 52 +/- 5 months) who met the clinical National Institute of Neurological Disorders and Stroke and Society for PSP (NINDS-SPSP) criteria for possible or probable PSP, with a swallowing questionnaire, an oral motor and speech examination, and either a modified barium swallow or ultrasound studies. Twenty-eight age- and sex-matched healthy controls (age, 65.6 +/- 1.5 years) were also evaluated with the questionnaire, oral examination, and the ultrasound study. We used ANOVA statistics to evaluate differences between groups; nonparametric correlations to assess associations between swallowing and motor and cognitive abnormalities; and logistic regression analysis to determine if the items of the questionnaire or oral examination predicted ultrasound or modified barium swallow abnormalities. While PSP patients had at least one complaint on the swallowing questionnaire (mean, 6.6), healthy controls had fewer and less relevant complaints (0.3). Patients with moderate-to-severe cognitive disabilities had significantly more complaints of dysphagia than those with mild or no impairment. PSP patients' oral motor skills and speech were mildly impaired but significantly different from those of controls. In the ultrasound studies, PSP patients had significantly fewer continuous swallows and required a longer duration to complete their swallows than did healthy controls. They also had mild-to-moderate abnormalities in the modified barium swallow study. The swallowing questionnaire, oral motor examination, and speech production examination accurately predicted the abnormalities detected with the swallowing studies. While 75% of patients had abnormal speech, all but one had abnormal swallowing studies. Thus, although dysphagia is associated with dysarthria, the two conditions are not always paired in the same patient. Our results suggest that the swallowing questionnaire and oral motor examination are an easy and cost-effective method to predict the swallowing disturbances in PSP.

Society for progressive supranuclear palsy: Third annual medical student award

Society for progressive supranuclear palsy: Third annual medical student award