Assessment of conventional magnetic resonance morphological measurements in diagnosis and differential diagnosis of progressive supranuclear paralysis / 中华老年医学杂志

Objective:To study the features as well as the diagnosis and differential diagnosis values by conventional MRI morphometrics in different clinical subtypes of progressive supranuclear palsy(PSP).Methods:Forty five patients with PSP were included, comprising three PSP subtypes: 15 cases of Richardson's syndrome(PSP-RS), 15 cases of Parkinson's syndrome(PSP-P)and 15 cases of progressive frozen gait(PSP-PFG). In addition, three control groups were established: 15 cases of multiple system atrophy-Parkinson's syndrome(MSA-P), 30 cases of primary Parkinson's disease(PD)and 40 healthy controls(HC). Midbrain area-to-Pons area ratio(M/P), Magnetic Resonance Parkinsonism Index(MRPI, MRPI2.0), width ratio of middle cerebellar peduncle to superior cerebellar peduncle(MCP/SCP), Midbrain-to-Pons ratio(MTPR), Angle of cerebral peduncle(A cp), third ventricle width/frontal horns width ratio(V 3rd/FH), and Humming bird sign rating scale(HBS-RS)scores were calculated.Diagnostic sensitivity and specificity were performed by ROC curve to assess the accuracy of these imaging indicators in the diagnosis and differential diagnosis of PSP and its subtypes. Results:The MRPI, MRPI2.0, MCP/SCP and HBS-RS scores were significantly higher in PSP group than in other control groups( H=69.351, 66.776, 33.926 and 84.694, all P<0.05), while M/P and MTPR were significantly lower in PSP group than in other control groups(H=60.101 and 77.276, all P<0.05). PSP group also had higher V 3rd/FH compared with PD or HC group( F=17.168, P<0.05), but not with MSA-P group( Z=-1.602, P>0.05). The above differences also existed between each PSP subgroup and control groups.Among PSP subgroups, PSP-PFG subgroup had a larger A cp than did PSP-RS( Z=-2.510, P<0.05), and had higher HBS-RS score than did PSP-P group( Z=-2.380, P<0.05). No significant differences in other MRI morphometric indexes were identified among PSP subtypes.The M/P, MRPI, MTPR, MRPI2.0, HBS-RS score showed good accuracy in diagnosing PSP and its each subgroup, with HBS-RS score being the most accurate indicator, when the cutoff value was 2, the AUC values were all higher than 0.99, and the sensitivity and specificity were all above 90%.PSP and its subtypes were best distinguished from MSA-P by MRPI, when the cutoff value was 9.94, the AUC values were all higher than 0.90, with the sensitivity of 100% and specificity of 86.67%.PSP and its subtypes were best distinguished from PD by MTPR, AUC values were all above 0.95, with slightly different cutoff values.Almost all the morphological measurement parameters failed to show significant sensitivity and specificity in discriminating subtypes of PSP.The sensitivity and specificity of almost all MRI morphometry indicators in differentiating different subtypes of PSP are not high. Conclusions:MRI morphometrics have a high value both in the diagnosis of PSP and its subtypes, and also in specific application fields.MRI morphometrics have a limited value in discriminating PSP subtypes.

A Cortical Substrate for Square-Wave Jerks in Progressive Supranuclear Palsy

Increased Signal in the Superior Cerebellar Peduncle of Patients with Progressive Supranuclear Palsy

OBJECTIVE: The provisional diagnosis of progressive supranuclear palsy (PSP) depends on a combination of typical clinical features and specific MRI findings, such as atrophy of the tegmentum in the midbrain. Atrophy of the superior cerebellar peduncle (SCP) distinguishes PSP from other types of parkinsonism. Histological factors affect the conventional fluid-attenuated inversion recovery (FLAIR) signals, such as the extent of neuronal loss and gliosis. METHODS: We investigated patients with PSP to verify the percentage of patients with various PSP phenotypes presenting a high signal intensity in the SCP. Three interviewers, who were not informed about the clinical data, visually inspected the presence or absence of a high signal intensity in the SCP on the FLAIR images. We measured the pixel value in the SCP of each patient. Clinical characteristics were evaluated using the Mann-Whitney test, followed by the χ² test. RESULTS: Ten of the 51 patients with PSP showed a high signal intensity in the SCP on FLAIR MRI. Higher pixel values were observed within the SCP of patients with a high signal intensity in the SCP than in patients without a high signal intensity (p < 0.001). The sensitivity and specificity of the high signal intensity in the SCP of patients with PSP was 19.6% and 100%, respectively. This finding was more frequently observed in patients with PSP with Richardson's syndrome (PSP-RS) (25.7%) than other phenotypes (6.2%). CONCLUSION: The high signal intensity in the SCP on FLAIR MRI might be an effective diagnostic tool for PSP-RS.

Clinical Milestones Preceding the Diagnosis of Multiple System Atrophy and Progressive Supranuclear Palsy: A Retrospective Cohort Study

OBJECTIVE: Multiple System Atrophy (MSA) and progressive supranuclear palsy (PSP) are rapidly progressive forms of degenerative Parkinsonism. The difficulties of diagnosing MSA and PSP in their early stages may lead to delayed referral to appropriate specialists and distress to patients, as well as delaying symptomatic treatment and participation in clinical trials. This work aimed to describe the symptoms that patients with MSA and PSP developed and plot their emergence relative to final diagnosis using a median onset in months. METHODS: Forty-seven patients from the United Kingdom with MSA or PSP diagnosed by a movement disorder specialist were interviewed with carers or relatives to establish milestone onset. This was corroborated using clinical notes and letters. RESULTS: In the MSA cohort (n = 23), autonomic symptoms (median 5.5 months before diagnosis) and falls (median 1 month before diagnosis) were the two clinical milestones which occurred before diagnosis. In the PSP cohort (n = 24), falling was the only milestone which occurred before diagnosis (median of 18.5 months). CONCLUSION: This study shows that PSP patients experience falling more than a year and a half an average before receiving a diagnosis and although MSA patients also tended to fall, this was much closer to the time of diagnosis. Further work with larger cohorts may illustrate whether these preliminary findings can be generalised to guide diagnosis and management.

No Evidence of a Contribution of the Vestibular System to Frequent Falls in Progressive Supranuclear Palsy

BACKGROUND AND PURPOSE: Conflicting results about vestibular function in progressive supranuclear palsy (PSP) prompted a systematic examination of the semicircular canal function, otolith function, and postural stability. METHODS: Sixteen patients with probable PSP [9 females, age=72±6 years (mean±SD), mean disease duration=3.6 years, and mean PSP Rating Scale score=31] and 17 age-matched controls were examined using the video head impulse test, caloric testing, ocular and cervical vestibular evoked myogenic potentials (o- and cVEMPs), video-oculography, and posturography. RESULTS: There was no evidence of impaired function of the angular vestibulo-ocular reflex (gain=1.0±0.1), and caloric testing also produced normal findings. In terms of otolith function, there was no significant difference between PSP patients and controls in the absolute peakto-peak amplitude of the oVEMP (13.5±7.2 µV and 12.5±5.6 µV, respectively; p=0.8) or the corrected peak-to-peak amplitude of the cVEMP (0.6±0.3 µV and 0.5±0.2 µV, p=0.3). The total root-mean-square body sway was significantly increased in patients with PSP compared to controls (eyes open/head straight/hard platform: 9.3±3.7 m/min and 6.9±2.1 m/min, respectively; p=0.032). As expected, the saccade velocities were significantly lower in PSP patients than in controls: horizontal, 234±92°/sec and 442±66°/sec, respectively; downward, 109±105°/sec and 344±72°/sec; and upward, 121±110°/sec and 348±78°/sec (all p<0.01). CONCLUSIONS: We found no evidence of impairment of either high- or low-frequency semicircular function or otolith organ function in the examined PSP patients. It therefore appears that other causes such as degeneration of supratentorial pathways lead to postural imbalance and falls in patients with PSP.

Prospective Characterization of Cognitive Function in Typical and ‘Brainstem Predominant'Progressive Supranuclear Palsy Phenotypes

OBJECTIVE: Clinicopathological studies over the last decade have broadened the clinical spectrum of progressive supranuclear palsy (PSP) to include several distinct clinical syndromes. We examined the cognitive profiles of patients with PSP-Richardson's syndrome (PSP-RS) and two atypical ‘brainstem predominant' PSP phenotypes (PSP-parkinsonism, PSP-P; and PSP-pure akinesia with gait freezing, PSP-PAGF) using a comprehensive neuropsychological battery. METHODS: Fourteen patients diagnosed as PSP-RS, three patients with PSP-P and four patients with PSP-PAGF were assessed using a comprehensive battery of neuropsychological tests.

Progress of the application of optical coherence tomography in atypical parkinsonian syndromes / 中华神经科杂志

Retinal damage has been found in atypical parkinsonian syndromes (APS) which include multiple system atrophy, progressive supranuclear palsy, and corticobasal syndrome. Optical coherence tomography (OCT) is a noninvasive, noncontact, repeatable bioimaging technique that can be used to evaluate the structural changes in the retina. We reviewed the recent progress of OCT technology in APS for reference in clinic.

End-stage Treatment of a Patient with Progressive Supranuclear Palsy and Severe Retroflexion of the Neck -Improvement in Retroflexion of the Neck by Botulinum Therapy: A Case Report- / 日本プライマリ・ケア連合学会誌

Progressive supranuclear palsy (PSP) comprises psychiatric, neurological and physical complications. In the late stage, most patients with PSP are bed-bound because of motor disability. PSP manifests as predominantly axial abnormality in posture and abnormal muscle tone, resulting in cervical retroflexion and somatic pains. Severe retroflexion of the neck is frequently a cause of repeated aspiration pneumonia that affects survival expectancy. We injected botulinum toxin (BTX) for severe retroflexion using a CT-guided procedure to confirm the anatomical locations of paravertebral muscles and to localize the paravertebral target muscles for a patient with PSP who had repeated pneumonia caused by retroflexion-related dysphagia. BTX injection treatment markedly ameliorated the cervical dystonia and concomitant dysphagia. The patient has recovered and may be able to be discharged to home. There are few reports on the BTX injection technique, but BTX has analgesic effects and induces relaxation of abnormal muscle tension. Therefore, it may be applicable to other movement disorders at the late stage.

Síndrome da Paralisia Supranuclear Progressiva: As dificuldades de diagnóstico e a atuação da Equipe multidisciplinar quanto aos cuidados à pessoa afetada por esta doença / Progressive Supranuclear Palsy Syndrome: The difficulties of diagnosis and the performance of the Multidisciplinary Team regarding the care of the person affected by this disease / Síndrome de parálisis supranuclear progresiva: Las dificultades de diagnóstico y el trabajo del equipo multidisciplinario sobre el cuidado de la persona afectada por esta enfermedad

A Síndrome da Paralisia Supranuclear Progressiva (PSP) é doença neurodegenerativa do Sistema Nervoso Central (SNC), rara, e de difícil diagnóstico, afetando principalmente o tronco cerebral e os núcleos da base. O quadro clínico se caracteriza por oftalmoparesia supranuclear, instabilidade postural e demência. O objetivo do estudo foi investigar a fisiopatologia, diagnóstico, tratamento e assistência da equipe multidisciplinar às pessoas com PSP. Revisão integrativa de 15 artigos publicados na base de dados da Biblioteca Virtual da Saúde, BVS, envolvendo estudos de casos e pesquisa de campo. O estudo revelou pouca publicação acerca da doença e, por ser rara, não existe fármaco eficiente e eficaz; o diagnóstico é limitado nas primeiras manifestações, e somente possível por meio de exames mecanicistas. Em razão de existir parco material sobre a assistência a estes casos, sugere-se que os Conselhos, Associações de Neurologia e demais especialidades envolvidas no tratamento desenvolvam, divulguem mais detalhes sobre a doença, a fim de se criar um protocolo de atendimento integral aos afetados pela síndrome, bem como o necessário apoio aos familiares e cuidadores, que auxilie nas práticas da assistência ambulatorial e familiar.

Progressive Supranuclear Palsy Syndrome (PSP) is a rare and difficult diagnosis of the central nervous system (CNS) neurodegenerative disease that mainly affects the brainstem and nuclei of the base. The clinical picture is characterized by supranuclear ophthalmoparesis, postural instability and dementia. Objective: to investigate the pathophysiology, diagnosis, treatment and assistance of the multidisciplinary team to PSP users. Integrative review of 15 articles published in the database of the Virtual Health Library, VHL, involving case studies and field research. Results: the study revealed little publication about the disease and, because it is rare, there is no efficient and effective drug; The diagnosis is limited in the first manifestations, and it is only possible by means of mechanistic examinations. Because there is little material on the assistance to these cases, it is suggested that the Neurology Councils and Associations and other specificities involved in the treatment develop and disseminate more details about the disease, in order to create a protocol for comprehensive care Affected by this syndrome, as well as the necessary support for family members and caregivers, to assist in outpatient and family care practices.

Parálisis Supranuclear Progresiva Syndrome (PSP) es una enfermedad neurodegenerativa del sistema nervioso central (SNC), una rara y difícil de diagnosticar, que afecta principalmente el tronco cerebral y los ganglios basales. El cuadro clínico se caracteriza por oftalmoparesia supranuclear, inestabilidad postural y demencia. Investigar la fisiopatología, diagnóstico, tratamiento y atención del equipo multidisciplinario para llevar a la PSP. Revisión integradora de 15 artículos publicados en la Biblioteca Virtual en Salud Base de datos, BVS, que incluye casos de estudio y la investigación de campo. El estudio mostro poca publicación de la enfermedad y, debido a que es raro, no hay ningún fármaco eficaz y eficiente; el diagnóstico es limitada en las primeras manifestaciones, y sólo es posible a través de pruebas mecanicistas. Dado que el material que hay escasa la ayuda a estos casos, se sugiere que las juntas y asociaciones de neurología y otras especilidades implicados en el tratamiento desarrollan y dan a conocer más detalles acerca de la enfermedad, de manera que se crea un protocolo de tratamiento integral para afectadas por este síndrome, así como el apoyo necesario a las familias y cuidadores para ayudar en las prácticas de atención ambulatoria y la familia.

Progressive Supranuclear Gaze Palsy with Predominant Cerebellar Ataxia: A Case Series with Videos

Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2.3 years after the onset of ataxia. Mild cerebellar volume loss and midbrain atrophy were detected on brain imaging, which are supportive of a diagnosis of PSP. Videos are presented illustrating the co-existence of cerebellar signs and supranuclear gaze palsy and the disappearance of cerebellar signs with disease progression. Better recognition and the development of validated diagnostic criteria would aid in the antemortem recognition of this rare condition.

‘Hummingbird’ Sign in a Patient with Guam Parkinsonism-Dementia Complex

We present a case of a 71-year-old male Chamorro patient from Guam who presented with progressive supranuclear palsy (PSP)-Richardson’s syndrome. Considering his strong family history of parkinsonism and a PSP phenotype, he was clinically diagnosed with Guam parkinsonism-dementia complex (PDC). Magnetic resonance imaging (MRI) of the brain revealed prominent midbrain atrophy with preserved pontine volume, forming the ‘hummingbird’ sign, which has not been described before in Guam PDC. Molecular analysis of the chromosome 9 open reading frame 72 gene (C9orf72) showed only 6 GGGGCC repeats. We discuss the clinico-pathological similarities and differences between PSP and Guam PDC, and highlight the topography of neuropathological changes seen in Guam PDC to explain the appearance of the ‘hummingbird’ sign on MRI.

Treatments and Rehabilitation for Progressive Supranuclear Palsy (review) / 中国康复理论与实践

Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease. The etiology and pathogenesis are still unclear. Nowadays there is a lack of an effective molecular marker which can effectively assist the early diagnosis and prognosis of this disease. This article reviewed the pathological anatomy, diagnostic criteria, subtypes and clinical manifestations, imaging studies, scales evaluation, treat-ment and rehabilitation, complications and risk factors of PSP. Patients with PSP should be managed by a multidisciplinary team. The man-agement of PSP should include the rehabilitation of disorder and palliative treatment, as well as support and education, to improve the quali-ty of life for patients and their caregivers.

Preliminary study of DWI differential diagnosis in multiple system atrophy,progressive supranuclear palsy and Parkinson's disease / 实用放射学杂志

Objective To explore the diffusion-weighted imaging(DWI) values in diagnosis and differential diagnosis in multiple system atrophy (MSA),progressive supranuclear palsy (PSP) and Parkinson's disease (PD).Methods Conventional MRI and DWI were performed in 24 clinically proved MSA patients,35 PD patients,12 PSP patients,and 32 age-matched normal controls (the control group).DWI was performed using a single shot-spin echo-echo planar imaging sequence,and ADC values were measured in the ROIs of different brain regions(including basal ganglia,thalamus,middle cerebellar peduncles and superior cerebellar peduncles).Then one way ANOVA test was used for statistical analysis.Results PSP group had the largest ADC values in putamen,globus pallidus,caudate,andsuperiorcerebellar peduncles[(0.75±0.43) ×10-3 mm2/s,(0.77±0.06)×10 3 mm2/s,(0.77±0.44)×10-3 mm2/s and (1.11±0.22)× 10-3 mm2/s].The ADC values of globus pallidus and caudate had statistically difference comparing with MSA [(0.73±0.04) × 10-3 mm2/s and (0.73±0.40) × 10-3 mm2/s],PD[(0.67±0.11) × 10-3 mm2/s and (0.73±0.04) × 10-3 mm2/s],and control group[(0.71±0.05) × 10-3 mm2/s and (0.72±0.04) × 10-3 mm2/s].MSA group had the largest ADC values in middle cerebellar peduncles[(0.95±0.16) × 10-3 mm2/s],and there was statistically significant difference,comparing with PSP,PD and control group[(0.74±0.49) × 10-3 mm2/s,(0.69±0.50) × 10-3 mm2/s and (0.68±0.31) × 10-3 mm2/s].The ADC values of PD group and control group had no statistically significant difference in putamen,globus pallidus,caudate,thalamus,middle cerebellar peduncles and superior cerebellar peduncles(P＞0.05).Conclusion ADC values in the basal ganglia,the middle cerebellar peduncles and superior cerebellar peduncles have very important significance in differential diagnosis in MSA,PSP and PD.

Progressive Supranuclear Palsy-Like Syndrome after Surgical Repair of Chronic Ascending Aorta Dissection

A 70-year-old woman underwent cardiopulmonary bypass surgery for aorta dissection. After 10 days she developed a vertical gaze palsy, and 2 months later she presented with dysarthria, bradykinesia, postural instability, blepharospasm, and truncal tilt to the left. Brain imaging indicated old lacunes in the bilateral thalamus. Her symptoms remained unchanged during a 4-year follow-up, which seems to be incompatible with progressive supranuclear palsy (PSP). However, the clinical features of this case were suggestive of PSP-like syndrome after cardiopulmonary bypass surgery.

Clinical Approach to Progressive Supranuclear Palsy

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.

Longitudinal Clinical Changes of Non-Fluent/Agrammatic Primary Progressive Aphasia as Tau Spectrum Disorder: A Case Report

BACKGROUND: Tauopathies are a group of diseases caused by the accumulation of hyperphosphorylated tau protein in the central nervous system. Previous studies have revealed that there is considerable overlap in clinical, pathological, and genetic features among different taupathies. CASE REPORT: We report a patient with non-fluent/agrammatic primary progressive aphasia at the initial assessment. Over time, other symptoms belonging to corticobasal degeneration and progressive supranuclear palsy appeared in this patient. CONCLUSIONS: Clinical overlapping features in these disorders may represent different phenotypes of a single disease process.

Utility of the Midbrain Tegmentum Diameter in the Differential Diagnosis of Progressive Supranuclear Palsy from Idiopathic Parkinson's Disease

BACKGROUND AND PURPOSE: Various magnetic resonance (MR) measurements have been proposed to aid in differentiating between progressive supranuclear palsy (PSP) and idiopathic Parkinson's disease (IPD); however, these methods have not been compared directly. The aim of this study was to determine which measurement method exhibits the highest power to differentiate between PSP and IPD. METHODS: Brain MR images from 82 IPD and 29 PSP patients were analyzed retrospectively. T1-weighted 3D volumetric axial images, or sagittal images reconstructed from those axial images were examined. MR measurements included the length from the interpeduncular fossa to the center of the cerebral aqueduct at the mid-mammillary-body level, adjusted according to the anterior commissure-posterior commissure length (MB(Tegm)), the ratio of the midbrain area to the pons area (M/P ratio) as measured by both Oba's method (Oba M/P) and Cosottini's method (Cosottini M/P), and a modified MR parkinsonism index (mMRPI). RESULTS: Receiver operating characteristic (ROC) analysis indicated that the areas under the ROC curves (AUCs) exceeded 0.70, with a high intrarater reliability for all MR measurement methods. ROC analyses of four MR measurements yielded AUCs of 0.69-0.76. At the cutoff value with the highest Youden index, mMRPI had the highest sensitivity, while Oba M/P offered the highest specificity. A comparison of the ROC analyses revealed that MB(Tegm) was superior to mMRPI in differentiating PSP from IPD (p=0.049). There was no difference in discriminating power among Oba M/P, Cosottini M/P, and MB(Tegm). CONCLUSIONS: Simple measurements of MB(Tegm) on axial MR images at the mid-mammillary-body level are comparable to measurements of the M/P ratio with regard to their ability to discriminate PSP from IPD.

Assessment of Cerebral Vascular Reserve Capacity Prior to Carotid ArteryStenting: Brain Single Photon Emission Computed Tomography images and Magnetic Resonance Arterial Spin Labeling images

No abstract available.

Probable Creutzfeldt-Jakob Disease Presenting as Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a clinical syndrome comprising vertical supranuclear palsy, parkinsonism, postural instability, and mild dementia. Other disorders can present with similar clinical pictures. In this report we describe a case of Creutzfeldt-Jakob disease (CJD) presenting with the PSP and discuss which features may help to prevent misdiagnosis. To the best of our knowledge, this is the first report of CJD presenting with PSP syndrome in Korea.

Postural Tachycardia Syndrome Presented With Acrocyanosis

No abstract available.