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PubMed2006

Current and future treatments in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is an atypical parkinsonian disorder that, in spite of its growing recognition, is still underdiagnosed. For management, prognosis, and research, an accurate and early diagnosis is essential. PSP is a relentlessly progressive neurodegenerative disorder, clinically characterized by parkinsonism with prominent axial involvement and postural instability, bulbar symptoms, supranuclear ophthalmoplegia, and executive dysfunction. Abnormal neuronal and glial four-repeat tau aggregations affecting the basal ganglia and selective brainstem structures result in dysfunction of the five frontosubcortical circuits and brainstem functions. Primary therapeutic approaches are based on neurotransmitter replacement and palliative strategies. This article reviews the experience and challenges with neurotransmitter replacement and palliative strategies through an extensive literature search of studies published between 1965 and 2005. The role of and limited experience with alternative therapies, such as deep brain stimulation and pallidotomy, are also discussed. Advances in the development of biological therapies for PSP and a better understanding of its etiopathogenesis will likely result from epidemiologic studies and developed four-repeat tau-transgenic animal models. The management of patients with this disorder poses a considerable challenge and includes symptomatic and palliative strategies, as well as education and support, to improve the quality of life for patients and their caregivers.

Journal Article↗

PubMed1997

[Progressive supranuclear palsy].

Progressive supranuclear paly (PSP) was firstly reported by Steel in 1964. This condition was separated from Parkinsonism by both clinical symptoms and neuropathological findings. Recently, in an attempt to improve diagnostic accuracy to give appropriate informed concepts and to select correct cases for drug studies or other research purpose, diagnostic criteria for PSP have been developed. PSP begins in the presenile period and duration of illness is 5.9 years (1.2-10.3 years; Maher and Lees, 1986). Cardinal clinical symptoms of PSP are supranuclear gaze palsy, neck dystonia, parkinsonism, pseudobulbar palsy, gait imbalance with frequent falls and subcortical dementia. Supranuclear gaze palsy and bradykinesia are essential for diagnosis. MR-imaging of PSP shows dilatation of the third ventricle. Other laboratory examinations show no specific findings. Neuropathologically, marked dilation of the third ventricle and volume loss of periaqueductal area of the midbrain are noted in macroscopic view. Microscopical examination reveals neuronal loss and gliosis in the tegmentum, the tectum, periaqueductal gray, the dentate-rubro-pallido-luysial area, and the inferior olivary nucleus. Neuropathological hallmarks of PSP are neuronal loss, presence of the globose typed neurofibrillary degeneration, and glial tangles (so called tuft shaped astrocyte and coiled body). Atypical cases of PSP are reported. Such cases are reported as pure akinesia, PSP without ophthalmoplegia, dementia predominant PSP, pathologically diagnosed pallido-nigro-luysial atrophy (PNLA), pathologically diagnosed corticobasal degeneration which showed no laterality, and so on. Reported cases as pure akinesia was diagnosed as PSP or PNLA by neuropathological findings. Improvement of diagnostic accuracy in PSP is expected to ithrapeutic trials, to investigate the etiology, and to separate the other clinical entity from PSP.

Female↗

PubMed1987

Limb dystonia in progressive supranuclear palsy.

Progressive limb dystonia contributed to disability in 8 of 30 patients with progressive supranuclear palsy (PSP). In five, it was present when the patients were on no medication. In four, it had been present before the distinctive ophthalmoplegia permitted a correct diagnosis. The severity of limb dystonia did not correlate with the severity of either ophthalmoplegia or neck dystonia. The importance of dystonia in the pathophysiology of PSP is emphasized, with regard to both the branchial dystonia that underlies several cardinal features of the disease, and to the frequent occurrence of limb dystonia as an early sign.

Aged↗

PubMed2000

A role for the substantia nigra pars reticulata in the gaze palsy of progressive supranuclear palsy.

We examined the topography and degree of cell loss within basal ganglia structures commonly involved in progressive supranuclear palsy in order to identify any relationship between degeneration in these nuclei and gaze palsy. Serial section analyses and unbiased quantitative techniques were applied to brain tissue from six cases with progressive supranuclear palsy (four with gaze palsy and two without) and six controls with no neurological or neuropathological abnormalities. The total number of nucleolated neurons within the substantia nigra pars compacta (SNc) and reticulata (SNr), the subthalamic nucleus, and the internal and external segments of the globus pallidus was determined for all subjects and the data expressed as percentages of control values to compare degeneration across these basal ganglia structures. The density of neurofibrillary tangles was also evaluated within these structures. Despite significant subcortical neurofibrillary tangle formation in all cases, there was considerable variability in the degree of neuronal cell loss in all basal ganglia regions, except the SNc which was consistently affected. There was no correlation between the ranked density of neurofibrillary tangles and the degree of neuronal cell loss in any basal ganglia region. Comparisons between cases with and without gaze palsy revealed a 40% greater decrease in the number of SNr neurons in cases with gaze palsy (75 +/- 8% loss) compared with those without (35 +/- 14% loss). This was the largest difference between these cases. As the SNr projects to the superior colliculus, degeneration of this basal ganglia structure may disrupt eye movements in progressive supranuclear palsy.

Aged↗

PubMedDate not supplied

[A clinical and epidemiological description of a series of patients diagnosed as suffering from progressive supranuclear palsy].

INTRODUCTION: Progressive supranuclear palsy is a disease that normally presents only sporadically in adults and courses in a progressive, chronic manner. It is characterised by the presence of supranuclear ophthalmoplegia, postural instability, a Parkinsonian syndrome, pseudobulbar affect, cervical dystonia and cognitive impairment. PATIENTS AND METHODS: We conducted a descriptive study of clinical and epidemiological features in a series of 18 patients who satisfied the mandatory NINDS-SPSP clinical eligibility criteria for the likely diagnosis of progressive supranuclear palsy, using the scale developed by Golbe et al. RESULTS AND CONCLUSIONS: The mean age of onset of the disease was 58.6 +/- 8.2 years, 55.5% of the patients were males, the average history of the disease at the time of diagnosis was 4.39 +/- 2.3 years, and there was a diagnostic subregister in the first 4 years of the disease. Gait disorders, falls and slowness were the most frequently observed presenting forms of the disease. During their first four years with the disease, 75% of the patients were totally independent when it came to carrying out activities of daily living, whereas after the fourth year there was a predominance of the need for aid and absolute dependence. Dysphagia was more frequent in the later stages of the disease. Ocular motility disorders and impaired cognitive functioning were obvious in the initial stages of the disease, and there was a strong correlation between the length of time the disease had been coursing and the severity of the ocular and cognitive disorders.

Cognition Disorders↗

PubMed2003

Diagnostic accuracy of progressive supranuclear palsy in the Society for Progressive Supranuclear Palsy brain bank.

Diagnostic accuracy has been addressed previously for Parkinson's disease in a brain bank collection, but accuracy of progressive supranuclear palsy (PSP) has not been addressed in a similar setting. Clinical and genetic features of pathologically confirmed cases of PSP were compared with misdiagnosed cases to determine ways to improve diagnostic accuracy. Medical records were reviewed for 180 cases sent to the Society of Progressive Supranuclear Palsy Brain Bank that had standardized neuropathologic evaluations as well as determination of apolipoprotein E and tau genotypes. Of the 180 cases studied, 137 had PSP and 43 had other pathologic diagnoses. Corticobasal degeneration (CBD), multiple system atrophy (MSA), and diffuse Lewy body disease (DLBD) accounted for 70% of the misdiagnosed cases. History of tremor, psychosis, dementia, and asymmetric findings were more frequent in misdiagnosed cases. The frequency of H1 tau haplotype (93 vs. 80%) and H1H1 genotype (86 vs. 66%) were significantly greater and APOE epsilon4 carrier state was significantly less (17 vs. 41 %) in PSP compared with misdiagnosed cases. Pathologic evaluation of clinically diagnosed PSP remains important for definitive diagnosis, and CBD, MSA, and DLBD are the disorders most likely to be misdiagnosed as PSP. Tremor, psychosis, early dementia, asymmetric findings, absence of H1 haplotype, and presence of APOE epsilon4 should raise questions about a diagnosis of PSP.

Aged↗

PubMed2005

A new mutation of the tau gene, G303V, in early-onset familial progressive supranuclear palsy.

BACKGROUND: Progressive supranuclear palsy (PSP) is a clinicopathological syndrome related to tau deposits and in linkage disequilibrium with tau polymorphisms. Some rare familial PSP cases have been related to tau gene mutations. OBJECTIVE: To present the clinical, pathological, and molecular data of one family with early-onset autosomal dominant PSP. DESIGN: We performed clinical examinations, quantitative neurological tests, positron emission tomographic scans with fluorodopa F 18 and raclopride C 11, analysis of tau mutations, neuropathological examinations, and protein analyses on brain specimens. RESULTS: Three family members had PSP confirmed by pathological features in the proband. A novel mutation of tau, G303V, was found in the proband and other family members. tau Isoforms with 4 microtubule-binding repeats were overexpressed in the proband brain. CONCLUSIONS: The G303V mutation of tau is associated with autosomal dominant PSP. Expression of 4 microtubule-binding repeat tau isoforms is increased in the proband.

Amino Acid Substitution↗

PubMed1994

Vascular progressive supranuclear palsy.

BACKGROUND: Progressive supranuclear palsy (PSP) is a neurologic syndrome of unknown cause. This idiopathic type of PSP is usually associated with characteristic clinical and pathological features. OBJECTIVE: To assess evidence of cerebrovascular disease in a population of patients with clinically defined PSP, and to compare clinical and neuroimaging features in vascular versus idiopathic PSP. DESIGN AND METHODS: Using predetermined criteria, the records of 128 patients diagnosed with PSP were reviewed for evidence of vascular disease. RESULTS: Thirty patients (23.3%) satisfied criteria for vascular PSP. The vascular group differed from the idiopathic group by asymmetric and predominantly lower body involvement (p < 0.05). Corticospinal signs, pseudobulbar signs, gait difficulties, dementia, and incontinence of bowels and bladder were also more common in the vascular group, but these differences did not reach statistical significance. CONCLUSION: PSP is a syndrome which can be caused by cerebro-vascular disease. In addition to an increased frequency of stroke risk factors and neuroimaging evidence of vascular disease, vascular PSP can be differentiated from idiopathic PSP by a higher degree of asymmetry, lower body involvement, and evidence of corticospinal and pseudobulbar signs.

Aged↗

PubMedDate not supplied

MR vs CT in progressive supranuclear palsy.

Nine progressive supranuclear palsy (PSP) patients were studied with computerized tomography (CT) and magnetic resonance (MR) in order to determine the efficacy of each in detecting atrophy of the brainstem. Three additional PSP patients were evaluated with MRI for quantitative (electronic) measurements of the colliculi, pons and midbrain tegmentum. Both CT and MRI were equally effective in demonstrating midbrain atrophy. The MR was able to utilize the sagittal view to visualize thinning of the collicular (quadrigeminal) plate, a useful sign in PSP. Atrophy of the thinned collicular plate is more pronounced in the superior colliculus, one of the most common sites of pathology in PSP. The MR is able to make quantitative measurements of the degree of atrophy of the colliculi, pons and midbrain tegmentum.

Adolescent↗

PubMed2004

Midbrain hypometabolism as early diagnostic sign for progressive supranuclear palsy.

OBJECTIVE: Progressive supranuclear palsy (PSP) is often misdiagnosed in early phase. The purpose of this study is to investigate the feature of [(18)F]fluoro-2-deoxy-d-glucose (FDG) positron emission tomography images for the early diagnosis of PSP. METHODS: We studied 15 patients with PSP and 16 normal subjects. Using SPM99 and analysis of covariance to eliminate the effect of aging, the differences between PSP and normals were displayed as a statistical map. In the PSP, we also investigated the correlation with duration and with the subscores of Unified Parkinson's Disease Rating Scale. RESULTS: The glucose metabolism of midbrain was significantly lower in PSP than in normals. However, correlation was not found between the metabolism of midbrain and clinical deterioration. CONCLUSIONS: The statistical map clearly demonstrated the hypometabolism of midbrain in PSP, which is independent of the clinical deterioration. The hypometabolism of midbrain is one of the most promising sign for early diagnosis of PSP.

Adult↗

PubMed2002

Increase of cdk5 is related to neurofibrillary pathology in progressive supranuclear palsy.

BACKGROUND: Progressive supranuclear palsy (PSP) is characterized by a pure neurofibrillary tau pathology involving mainly basal ganglia and brainstem nuclei. In addition to a haplotype of the tau gene potentially favoring tau aggregation, lipoperoxidation has been shown to be associated with PSP tau pathology. OBJECTIVE: To analyze cdk5/p35 complex, a kinase that regulates neurite outgrowth, as a potential cellular mechanism underlying tau phosphorylation in brain tissues from PSP and control cases and comparatively in cerebral cortex from subjects with AD. METHODS: Cdk5/p35 protein levels and distribution were evaluated by immunoblotting and immunocytochemistry in brain regions from seven PSP, six AD, and seven control cases, with similar postmortem intervals. RESULTS: Total cdk5 protein levels were significantly increased by more than threefold in PSP tissue and were augmented in PSP neurons, codistributed with tau immunoreactivity. P35, the regulatory subunit of cdk5, was degraded by postmortem proteolysis to the same extent in PSP, AD, and control tissues. CONCLUSIONS: The proteolysis in vivo of p35, the regulatory subunit of the kinase, is not ascertainable because it is masked by its postmortem degradation. The study, however, indicates that in PSP, the alteration of cdk5 is different from that described in AD and suggests that the absence of amyloid beta protein deposition may account for the different pathways responsible for the same kinase activation.

Aged↗

PubMedDate not supplied

[Descriptive study of a serie of patients affected by progressive supranuclear palsy].

INTRODUCTION: Progressive supranuclear palsy is a neurodegenerative disorder affecting diverse neurologic systems. The actual treatment response is poor in most patients. OBJECTIVE: review of a long series of patients affected by PSP in several aspects. PATIENTS AND METHODS: A series of patients was reviewed by means of the register questionnaire of PSP in Spain (from PSP Disabling Rating Scale and Staging System). This is carried out on the patients when the diagnostic is done. It was achieved a descriptive of the patients, in several aspects, and an evaluation of the treatment in relation to the dose and the duration. RESULTS: In general, the age of diagnostic is 66 years, there is not neurological illness in the family, falls and disorders of gait are the most representative parameters. The neuroimage shows fronto temporal atrophy. The treatment response is poor, despite the dose and the duration. CONCLUSIONS: Our series confirms the typical dates of the illness an the poor response to treatment with L Dopa.

Aged↗

PubMedDate not supplied

Central conduction time in progressive supranuclear palsy.

Progressive nuclear palsy (PSP) is a parkinson-like extrapyramidal disorder with pathological evidence of brain stem demyelination. We studied brain stem auditory (BAEP) and somatosensory (SSEP) evoked potentials in 8 patients with progressive supra-nuclear palsy to look for evidence of such central demyelination. We found minor alterations in BAEPs and frequently abnormal SSEPs, suggestive of brain stem white matter involvement in this disorder. Evoked potentials may assist in the differentiation of PSP from other parkinsonian conditions.

Aged↗

PubMedDate not supplied

"Learned helplessness" and the patient with progressive supranuclear palsy.

Progressive supra-nuclear palsy is a neurologically debilitating disease of unknown cause and with no effective treatment. Learned helplessness, a major cause of depression in the institutionalized elderly, is a feeling of worthlessness and powerlessness resulting in a decreased incentive to try. This article provides insight into these two problems and specific nursing interventions to enhance self care.

Aged↗

PubMed2005

Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia.

Progressive supranuclear palsy (PSP) is a clinicopathological entity typically presenting as an akinetic rigid syndrome with early falls, axial rigidity, vertical supranuclear gaze palsy and levodopa resistance. Pathological features consist of tau deposition in neuronal and glial cells located mainly in subcortical and brainstem structures. Rare cases with the pathological diagnosis of atypical PSP have been described in which neocortical tau deposition is more widespread than what is usually seen in typical PSP. Progressive nonfluent aphasia (PNFA) is a syndrome characterized by spontaneous nonfluent speech and early preserved comprehension of language. Apraxia of speech (AOS) is a motor speech disorder that may be a feature of PNFA. We report the clinical and pathological findings of four cases that presented with features most consistent with PNFA predominated by AOS. Pathological features in these four cases included the typical features of PSP subcortically and in brainstem structures, but combined with tau-positive neuronal and glial pathology in the neocortex. Comprehensive semiquantitative analyses of tau burden including neurofibrillary tangles and pretangles, coiled bodies, tufted astrocytes and threads were undertaken in the four cases of atypical PSP and compared to 10 cases of typical PSP. Semiquantitative analysis demonstrated that in atypical PSP, the pathology shifts from subcortical grey and brainstem regions, commonly affected in typical PSP, towards neocortical regions. This shift in pathology accounts for the presentation of PNFA and AOS observed in our patients, as well as the lack of classic features of PSP. These cases demonstrate that atypical PSP can present as AOS and PNFA without the classic features of PSP.

Adult↗

PubMed2002

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

BACKGROUND: 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. OBJECTIVE: To evaluate clinical and pathologic features of cases of vascular PSP submitted to a PSP brain bank. DESIGN: 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. RESULTS: 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. CONCLUSIONS: 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.

Accidental Falls↗

PubMed2004

Relationship between frontotemporal dementia and corticobasal degeneration/progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) were described as separate entities, but prior to that an extrapyramidal variety of Pick's disease was recognized. Subsequently a pathological overlap between these conditions and clinical overlap between frontotemporal dementia, primary progressive aphasia, corticobasal degeneration syndrome and more recently PSP was recognized. Initially only the movement disorder had been emphasized, but now the behavioral and language symptoms are considered common. The syndromes of frontotemporal dementia/Pick's disease can be produced by underlying CBD, PSP or Pick pathology as well as with neural inclusions of the motor neuron disease type. The concept of this overlap has been confirmed genetically finding a similar spectrum of pathology with different tau mutations and even with tau negative pathology, which could be a deficiency of normal tau. The overlap of CBD with PSP and both with PPA and FTD allows to consider these relatively rare conditions as part of a more commonly occurring degenerative disease than previously recognized.

Basal Ganglia Diseases↗

PubMed1994

Progressive supranuclear palsy. Historical notes.

Progressive supranuclear palsy (PSP) is the name Dr. J. Clifford Richardson chose to designate an unusual clinical syndrome he first identified in the 1950s. Neurofibrillary degeneration is the hallmark of this fatal brain disease, and during our study of Richardson's patients, Professor Jerzy Olszewski and I also observed granulovacuolar degeneration, and widespread nerve cell loss and gliosis in subcortical and brain stem nuclei. The histopathological features bear a striking resemblance to those seen in postencephalitic parkinsonism after von Economo's epidemic encephalitis, and in the parkinsonism-dementia complex of Guam (PDC). During the past 30 years, neurologists confirm that progressive supranuclear palsy is a universal, sporadic and not uncommon neurodegeneration of middle and late life. Many fine studies, as reported here, have advanced our understanding of PSP but its cause, and thereby its cure, is still to be revealed. These historical notes tell of our observations from 1955 to 1975. We are pleased that colleagues remember these early descriptions and honor us by calling this disease, the Steele-Richardson-Olszewski (SRO) syndrome.

Canada↗