Criteria For Progressive Supranuclear Palsy Research Articles

Differentiation of atypical Parkinson syndrome and delineation from idiopathic Parkinson syndrome with routine magnetic resonance tomography

Atrophy of frontal lobe, midbrain, pons, and cerebellum was studied in 16 patients with progressive supranuclear palsy (PSP), 14 with multiple system atrophy of striatonigral type (MSA-P), 20 with idiopathic Parkinson's disease (IPS), and 12 age-matched healthy controls using axial T2-weighted MR images (1.5 Teslar). With <16 mm, the PSP group showed significantly lower anteroposterior midbrain diameters than the IPS, MSA-P, and control groups. We conclude that measurement of the anteroposterior diameter of the midbrain with axial T2-weighted MRI is a useful feature and should be incorporated into the diagnostic criteria for PSP. In addition to the typical slit hyperintensity in margin of putamen and decreased signal intensity in dorsolateral putamen, we found cerebellar atrophy in 64% of patients with MSA-P. Before now, this was considered a typical sign of multiple system atrophy of cerebellar type (MSA-C). The use of this feature in the differential diagnosis of both types of multiple system atrophy is debatable.

神経変性疾患（パーキンソン症候群を中心に）

Among the diseases causing parkinsonism, drug-induced parkinsonism is important because physicians often use anti-dopaminergic drugs such as benzamides for gastrointestinal disorders. In 28 patients with drug-induced parkinsonism, 13 (47%) showed persistent parkinsonism even 6 months after the cessation of the causative drugs. This means anti-dopaminergic drugs may bring subclinical parkinsonism to the clinical level especially in the elderly. Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and multiple system atrophy including striatonigral degeneration were recognized in 2003 as specific diseases by the National Health Insurance of Japan. The diagnostic criteria of PSP (the modification of NINDS-SPSP criteria) made by the study group of the Ministry of Health, Welfare and Labour (MHWL) of Japan has demonstrated that their sensitivity was 83% and specificity 97% in "probable PSP". Patients with CBD were surveyed among the main neurologic institutions in Japan using the provisional diagnostic criteria There were 151 patients with "probable CBD" and 13 with "definite CBD". Based on these data, the number of patients with PSP and CBD in 2001 was estimated as 6,400 (5.03 per 100,000) and 2,500 (1.93 per 100,000), respectively, in Japan. Neuroprotective drugs hold hope for the treatment of neurodegenerative diseases. Although many agents were reported to be neuroprotective for Parkinson's disease from in vitro studies, pramipexole, a dopamine agonist, is the only one with good clinical evidence based on neuroimaging. Other dopamine agonists as well as various nerve trophic factors may also have the same effects, though further confirmatory studies are necessary.

Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study.

To describe clinical features and identify prognostic predictors in progressive supranuclear palsy (PSP). Record-based diagnosis according to National Institute of Neurological Disorders and Stroke-Society for Progressive Supranuclear Palsy criteria was performed in 187 cases of PSP. Clinical information was abstracted from patient records. Sixty-two patients (33%) were examined by the investigators. Forty-nine of 62 patients (79%) underwent standardized clinical assessment. Predictors of survival were examined after a mean of 6.4 years. The most common symptoms at disease onset related to mobility (69%). Of patients undergoing standardized clinical assessment, diplopia occurred in 61%, photophobia in 43%, and eyelid apraxia in 43%. Seventy-five cases (40%) died during follow-up. Older age at onset and classification as probable PSP were associated with poorer survival. Onset of falls (hazard ratio 3.28, 95% CI 1.17 to 9.13), speech problems (hazard ratio 4.74, 95% CI 1.10 to 20.4), or diplopia (hazard ratio 4.23, 95% CI 1.23 to 14.6) within 1 year and swallowing problems within 2 years (hazard ratio 3.91, 95% CI 1.39 to 11.0) were associated with a worse prognosis. Mobility problems are the commonest early feature in PSP and visual symptoms are often functionally disabling. Early falls, speech and swallowing problems, diplopia, and early insertion of a percutaneous gastrostomy predict reduced survival.

A clinicopathological study of vascular progressive supranuclear palsy: a multi-infarct disorder presenting as progressive supranuclear palsy.

Clinical features suggesting a diagnosis of progressive supranuclear palsy (PSP) include early falls, axial rigidity, vertical supranuclear ophthalmoplegia, and levodopa unresponsiveness. When these clinical features are present, the diagnosis is almost always PSP, yet vascular disease sometimes has a similar presentation, referred to as vascular PSP. To evaluate clinical and pathologic features of cases of vascular PSP submitted to a PSP brain bank. Review of gross and microscopic neuropathological features, determination of tau haplotype, and medical record review of 4 patients with an antemortem diagnosis of PSP who did not meet the pathologic criteria for PSP and instead had vascular pathologic abnormalities. All patients had vertical supranuclear ophthalmoplegia, a history of falls, and a gradually progressive disease course. Falls began 1 year after symptom onset, and all patients had asymmetric findings on a neurological examination. A magnetic resonance imaging scan revealed lacunar basal ganglia infarcts in one patient and an increased T2-weighted signal in the corona radiata and centrum semiovale in another. Gross and microscopic neuropathological studies demonstrated infarcts in the cerebral cortex (n = 4), thalamus (n = 4), basal ganglia (n = 3), and cerebellum (n = 4). The brainstem was affected in one patient, but no infarcts were detected in the subthalamic nucleus or substantia nigra. Of the 4 patients, 3 carried an H2 tau haplotype, a rare occurrence in the general population. Asymmetric signs, falls after 1 year of symptom onset, vascular lesions on a magnetic resonance imaging scan, and an H2 tau haplotype may help differentiate vascular PSP from PSP. Thalamic and basal ganglia infarcts are common in patients with vascular PSP and, when present, may contribute to misdiagnosis.

Free and Bound Volatile Composition of Red Wines ofVitis viniferaL. cv. Öküzgözü and Bogazkere Grown in Turkey

<h3>Background</h3> Recent diagnostic criteria for progressive supranuclear palsy (PSP) by the Movement Disorder Society (MDS) operationalise recently recognised subtypes, beyond the ‘classical’ Richardson phenotype (PSP-RS): predominant parkinsonism (PSP-P), predominant gait freezing (PSP-PGF), and frontal-cognitive- behavioural presentations (PSP-F). <h3>Aims</h3> To evaluate the proportion of patients fulfilling MDS compared with previous NINDS-SPSP criteria; to establish the number of patients fulfilling operationalised subtypes criteria; and to explore the evolution of PSP subtypes. <h3>Methods</h3> A retrospective case note review study of 201 patients clinically diagnosed with PSP in a combined behavioural neurology and movement disorders service over a 10-year period. <h3>Results</h3> Of 201 patients at first presentation, 187 met MDS-PSP criteria (157 probable, 8 possible, 22 sug- gestive) compared with 152 for NINDS-SPSP criteria. The number of probable PSP patients fulfilling opera- tionalised subtype criteria were: 125 PSP-RS, 111 PSP-P, 5 PSP-PGF, and 37 PSP-F. Notably, 100 patients met criteria for more than one subtype. <h3>Conclusion</h3> MDS criteria allowed earlier PSP diagnosis than NINDS-SPSP criteria and captured a range of phenotypic subtypes. Further analysis will address the issue of determining subtype predominance and will explore the natural history of PSP phenotypes, with particular reference to frontal-cognitive- behavioural presentations.

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.

Diagnosis and management of progressive supranuclear palsy.

Because of the relevance of an early and accurate diagnosis for prognosis, management, and participation of patients in research, the classical clinical features--features that should raise suspicion of progressive supranuclear palsy (PSP) and those that should make the diagnosis of PSP unlikely--are discussed in this article. The accuracy of currently used clinical diagnostic criteria and the role of laboratory investigations in the diagnosis of PSP are reviewed. New terminology for the clinical diagnostic criteria for PSP is proposed. The main neuropathologic and neurochemical features supporting current main symptomatic and hypothesized experimental biologic therapies are suggested.

Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study

The prevalence of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) in the general population is unknown. Clinicopathological studies of patients diagnosed as having Parkinson's disease suggest that the two disorders may be underdiagnosed. We investigated the population prevalence of these disorders. We screened computerised records of 15 general practices in London, UK, for patients with specific diagnostic labels suggestive of Parkinson's disease or parkinsonism and all patients who had ever received antiparkinsonian medication. We assessed eligible patients by review of records, interview, physical examination, and video recordings of neurological signs for independent diagnostic confirmation. We diagnosed parkinsonian disorders according to published criteria and further reviewed patients with atypical features after 1 year. The participation rate was 84%. The age-adjusted prevalence for PSP was 6.4 per 100,000 (five probable and one possible case [95% CI 2.3-10.6]) and for MSA 4.4 per 100,000 (two probable and two possible cases [1.2-7.6]). An additional four atypical patients were identified at 1 year but did not fulfil criteria for PSP or MSA. Our results suggest that the true prevalence of PSP and MSA has been underestimated, since many patients in the community remain undiagnosed or misdiagnosed.

Accuracy of four clinical diagnostic criteria for the diagnosis of neurodegenerative dementias.

To evaluate the inter-rater reliability and validity of clinical diagnostic criteria for neurodegenerative dementias. Inter-rater accuracy of the diagnosis of AD has been explored, but there are few accuracy studies for progressive supranuclear palsy (PSP) and frontotemporal lobe dementia (FTD). Furthermore, there have been no simultaneous accuracy studies in a mixed sample of patients with cortical and subcortical neurodegenerative processes. Four experienced clinicians reviewed first-visit clinical data abstracted from the records of 40 pathologically diagnosed demented subjects. They were asked to apply the NINCDS-ADRDA criteria for AD, the NINDS-SPSP clinical criteria for PSP, the Lund and Manchester criteria for FTD, and the Consensus Guidelines for the Clinical Diagnosis of Dementia with Lewy Bodies (DLB). The generalized K for AD was 0.73, for PSP 0.82, for FTD 0.75, and for DLB 0.37. The K pool test showed a statistically significant difference between DLB and the other disease processes, and no differences were observed among AD, FTD, and PSP. The mean sensitivity for AD was 95%, for PSP 75%, for FTD 97%, and for DLB 34%. The mean specificity for AD was 79%, for PSP 98.5%, for FTD 97%, and for DLB 94%. We found improved inter-rater reliability for the diagnosis of AD among clinicians compared with earlier studies. Similarly, there was a near-perfect and substantial inter-rater agreement for the diagnosis of PSP and FTD. The sensitivity for the diagnosis of AD was high, although clinicians overdiagnosed this condition. However, there was a reasonable accuracy for the diagnosis of PSP and FTD. Heterogeneity of the clinical presentation of DLB significantly affected inter-rater agreement and accuracy. The use of multiple diagnostic criteria for cortical and subcortical dementia increases the level of clinical diagnostic accuracy.

Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders.

We investigated the validity and reliability of diagnoses made by eight neuropathologists who used the preliminary NINDS neuropathologic diagnostic criteria for progressive supranuclear palsy (PSP) and related disorders. The specific disorders were typical, atypical, and combined PSP, postencephalitic parkinsonism, corticobasal ganglionic degeneration, and Pick's disease. These disorders were chosen because of the difficulties in their neuropathologic differentiation. We assessed validity by measuring sensitivity and positive predictive value. Reliability was evaluated by measuring pairwise and group agreement. From a total of 62 histologic cases, each neuropathologist independently classified 16 to 19 cases for the pairwise analysis and 5 to 6 cases for the group analysis. The neuropathologists were unaware of the study design, unfamiliar with the assigned cases, and initially had no clinical information about the cases. Our results showed that with routine sampling and staining methods, neuropathologic examination alone was not fully adequate for differentiating the disorders. The main difficulties were discriminating the subtypes of PSP and separating postencephalitic parkinsonism from PSP. Corticobasal ganglionic degeneration and Pick's disease were less difficult to distinguish from PSP. The addition of minimal clinical information contributed to the accuracy of the diagnosis. On the basis of results obtained, we propose clinicopathologic diagnostic criteria to improve on the NINDS criteria.

Progressive supranuclear palsy: neuropathologically based diagnostic clinical criteria.

All cases examined postmortem at the Mayo Clinic that met the classic neuropathological criteria for progressive supranuclear palsy (PSP) were identified for retrospective clinical analyses. The necropsy material was re-examined by a second neuropathologist to confirm the pathological diagnosis of PSP, yielding 12 cases. A range of clinical signs were documented in these patients, with numerous findings beyond those noted in the original descriptions of this disorder. Atypical clinical findings included absence of supranuclear gaze palsy (two cases), prominent asymmetry (two), arm dystonia (two), upper limb apraxia (two), myoclonus (two), chorea (one), eyelid opening apraxia (one), and respiratory disturbance (one). A definite clinical diagnosis of PSP had been made during life in only eight of the 12 patients. From the retrospective analysis of these 12 cases, a set of clinical criteria were developed for the premortem diagnosis of PSP emphasising differences from other akinetic-rigid disorders.

Preliminary NINDS neuropathologic criteria for Steele‐Richardson‐Olszewski syndrome (progressive supranuclear palsy)

We present the preliminary neuropathologic criteria for progressive supranuclear palsy (PSP) as proposed at a workshop held at the National Institutes of Health, Bethesda, MD, April 24 and 25, 1993. The criteria distinguish typical, atypical, and combined PSP. A semiquantitative distribution of neurofibrillary tangles is the basis for the diagnosis of PSP. A high density of neurofibrillary tangles and neuropil threads in the basal ganglia and brain-stem is crucial for the diagnosis of typical PSP. Tau-positive astrocytes or their processes in areas of involvement help to confirm the diagnosis. Atypical cases of PSP are variants in which the severity or distribution of abnormalities deviates from the typical pattern. Criteria excluding the diagnosis of typical and atypical PSP are large or numerous infarcts, marked diffuse or focal atrophy, Lewy bodies, changes diagnostic of Alzheimer's disease, oligodendroglial argyrophilic inclusions, Pick bodies, diffuse spongiosis, and prion protein-positive amyloid plaques. The diagnosis of combined PSP is proposed when other neurologic disorders exist concomitantly with PSP.