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PubMed1999

Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration.

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are usually sporadic multi-system degenerations associated with filamentous tau inclusions in neurons and glia. As such they can be considered sporadic tauopathies in contrast to familial tauopathies linked to mutations in the tau gene. Mutations have not been found in the tau gene in either PSP or CBD. The clinical syndromes and neuroimaging of typical cases of PSP and CBD are distinct; however, atypical cases are described that have overlapping clinical and pathologic features. Both PSP and CBD have similar biochemical alterations in the tau protein, with the abnormal tau protein containing predominantly four-repeat tau. While there is overlap in the pathology in PSP and CBD, there are sufficient differences to continue the present day trend to consider these separate disorders. Several important pathologic features differentiate PSP from CBD. Ballooned neurons are frequent and nearly a sine qua non for CBD, but they are found in PSP at a frequency similar to that of other neurodegenerative diseases, such as Alzheimer's disease. Astrocytic lesions are different, with tufted astrocytes found in motor cortex and striatum in PSP and astrocytic plaques in focal atrophic cortices in CBD. The most characteristic neuronal tau pathology in CBD is wispy, fine filamentous inclusions within neuronal cell bodies, while affected neurons in PSP have compact, dense filamentous aggregates characteristic of globose neurofibrillary tangles. Thread-like processes in gray and white matter are much more numerous and widespread in CBD than in PSP. The brunt of the pathology in CBD is in the cerebrum, while the basal ganglia, diencephalon and brainstem are the targets of PSP. Further clinicopathologic studies will refine our understanding of these disorders and open the possibility that common etiologic factors may be identified for these unusual sporadic tauopathies.

Basal Ganglia↗

PubMed2002

Cholinergic neuronal loss in the basal forebrain and mesopontine tegmentum of progressive supranuclear palsy and corticobasal degeneration.

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are clinically characterized by atypical parkinsonism and cognitive disorders and classified in the same histopathological category showing neuronal and glial neurofibrillary tangles (NFTs). To characterize the vulnerability of the forebrain and midbrain cholinergic systems in PSP and CBD, we performed a comparative study of cholinergic neuronal changes in the nucleus basalis of Meynert (NBM) and the laterodorsal tegmental and pedunculopontine tegmental nuclei (LdtgN and PptgN) of brains obtained at autopsy (three cases of PSP, one case of CBD and six cases without neurological diseases) by immunohistochemistry for choline acetyltransferase (ChAT) and Gallyas-Braak staining. In the NBM, the number of neurons and the ChAT-positivity rate of remaining neurons were decreased more in CBD than PSP. On the other hand, in the PptgN and LdtgN neurons were reduced much more, and more NFTs were observed in PSP than CBD. PSP showed a severe decrease of neurons and the ChAT-immunopositive neurons in the LdtgN but less in the PptgN. In CBD, there was a mild deletion of the ChAT-immunostained neurons in the PptgN, but not in the LdtgN. In PSP, cholinergic neurons in the LdtgN are likely to be more vulnerable than PptgN and NBM.

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PubMed1994

Muscle mitochondrial ATP production in progressive supranuclear palsy.

Six patients with progressive supranuclear palsy (PSP) and 12 age-matched disease-free subjects participated in this study designed to compare rates of ATP production by intact mitochondria from biopsied skeletal muscle. When pyruvate and malate were used as metabolic substrates, rates of ATP production were 0.184 +/- 0.025 mumol/min/U of citrate synthase (CS) activity (a mitochondrial marker) in control subjects and 0.131 +/- 0.051 mumol/min/U of CS in PSP patients. In the presence of succinate, rates of ATP formation were 0.137 +/- 0.02 mumol/min/U of CS in controls and 0.109 +/- 0.04 mumol/min/U of CS in patients. With N,N,N',N'-tetramethyl-p-phenylenediamine (TMPD) and ascorbate, rates were 0.034 +/- 0.008 mumol/min/U of CS in controls and 0.022 +/- 0.01 mumol/min/U of CS in PSP subjects. Differences between the control and PSP populations reached statistical significance with pyruvate/malate and TMPD/ascorbate. No differences in either muscle histopathology or histochemistry were found between patient and control subjects. Results of this study suggest that oxidative phosphorylation defects occur in muscle mitochondria from patients with PSP.

Adenosine Triphosphate↗

PubMed2004

Pathological heterogeneity in progressive supranuclear palsy and corticobasal degeneration.

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are sporadic neurodegenerative disorders of late adult life. Focal asymmetric cortical atrophy with ballooned neurons, nigral degeneration, and tau-positive neuronal and glial lesions in both the gray and white matter, especially astrocytic plaques in the affected cerebral cortex, are characteristic features of CBD. While cortical involvement may occur in PSP, ballooned neurons are sparse and limited to the limbic system, and tufted astrocytes are abundant in the precentral gyrus and striatum. The present findings suggest that PSP and CBD are distinct pathological entities. However, there exist 'atypical' cases of PSP and CBD. Severe cortical involvement or asymmetric cortical atrophy can be seen in PSP. Ballooned neurons are sparse or difficult to detect in some cases of CBD, in spite of typical cortical tau pathology. Cortical symptoms are absent or only mild in 'minimal change' CBD. Moreover, several neurodegenerative disorders can underlie CBD. This pathological heterogeneity leads to difficulty in the clinical and pathological diagnosis of both disorders.

Animals↗

PubMed1996

Autopsy-proven progressive supranuclear palsy in two siblings.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that is generally considered to be nonfamilial. We report a brother and sister with clinical and pathologic findings characteristic of PSP. Both developed parkinsonism in the eighth decade of life and within 5 years exhibited severe postural instability, bradykinesia, rigidity, dystonia, dysarthria, dysphagia, urinary incontinence, pseudobulbar palsy, and supranuclear oculomotor dysfunction but no tremor. Neither responded to levodopa and/or carbidopa. Their mother and, possibly, maternal grandfather reportedly suffered from a parkinsonian syndrome. Essential tremor occurred in the siblings' father and in two of the brother's three children. Autopsy in the brother at age 81 years and sister at age 79 years revealed changes typical of PSP with atrophy and neurofibrillary tangles in the globus pallidus, subthalamic nucleus, and rostral tegmental brainstem. No Lewy bodies were present. These cases are the first pair of relatives reported with autopsy confirmation of PSP in both and raise the question of genetic predisposition to PSP.

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PubMed1996

Follow-up study of risk factors in progressive supranuclear palsy.

The cause of progressive supranuclear palsy (PSP) is not known and has been little studied. The one previous controlled epidemiologic survey, performed at our center in 1986, found small-town experience and greater educational attainment as PSP risks, but, in retrospect, these results may have been produced by ascertainment bias. Since that time, several anecdotal reports have implicated heredity and various environmental exposures in the cause of some cases of PSP. To clarify the results of the previous study and to evaluate the more recently implicated candidate factors in a controlled fashion, we mailed a validated 69-item questionnaire to 91 personally examined patients with PSP and 104 unmatched controls with other neurologic conditions for which they had been referred to our tertiary neurologic center. We were able to match 75 subjects from each group by year of birth, sex, and race and subjected them to a separate matched-pair analysis. We allowed surrogates to supply any or all of the responses. Questions concerned hydrocarbon, pesticide, and herbicide exposure; urban/rural living; auto repair and other occupations; head trauma; educational attainment; maternal age; and family history of PSP, parkinsonism, dementia, and other neurologic conditions. A statistically significant finding was that patients with PSP were less likely to have completed at least 12 years of school (matched odds ratio = 0.35, 95% CI = 0.12-0.95, p = 0.022; unmatched odds ratio = 0.44, 95% CI = 0.21-0.89, p = 0.020). We hypothesize that this result may be a proxy for poor early-life nutrition or for occupational or residential exposure to an as-yet unsuspected toxin. Future studies should examine these potential risk factors in PSP.

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PubMed1987

Elementary motor perseveration in early diagnosis of progressive supranuclear palsy.

Three patients with progressive supranuclear palsy (PSP) with different symptoms at onset presented intense elementary motor perseveration before the appearance of the distinctive features of the illness. In elementary motor perseveration once an element of a movement has begun it is no longer inhibited at the right time and continues unchecked. Perseverations were observed in these patients during tests for dysdiadochokinesia, for reproduction of rhythmic structures and during the drawing of simple geometric shapes. Though this sign has not yet been described in PSP it is possible that an adequate search for its occurrence may show it to be an important sign for early diagnosis and an element for the clinical characterization of PSP.

Female↗

PubMed2002

[Neuroimaging features in progressive supranuclear palsy and corticobasal degeneration].

Progressive supranuclear palsy (PSP) and cortocobasal degeneration (CBD) are often clinically confused with each other. In this paper, based our previous and on-going morphological and functional neuroimaging studies, the features characteristic of the two diseases are discussed. In PSP patients, the atrophic and metabolic changes are dominant in the frontal lobes, basal ganglia, and midbrain, while in CBD patients, the changes are dominant in the parietal lobe. There is little overlap of topographical distribution of atrophy and functional changes between PSP and CBD, despite the considerable similarity of symptoms of the two disorders. The clear distinction between the two diseases may be in part caused by the criteria-based subject selection process; based on stringent clinical diagnostic criteria for each disorder, which have a high specificity and a low sensitivity, only patients that are typical of each disease are compared. Nevertheless, these neuroimaging features appear to reflect different clinical and pathologic phenotypes between the two diseases. These findings suggest that neuroimagings facilitate the differential diagnosis between patients with PSP and those with CBD.

Humans↗

PubMed1990

[Obsessive-compulsive behavior and progressive supranuclear palsy].

A case of progressive supranuclear palsy characterized by a loss of self-activation and a compulsive behaviour of the obsessive type is reported. The pathological examination was remarkable for the intensity of pallidal lesions and their diffusion to both the external and internal segments. While the loss of self-activation seemed to result from a damaged cortico-subcortical circuit forming a limbic loop, the compulsive behaviour of the obsessive type may have resulted from the interruption of a frontal-caudal-pallidal-luysian circuit.

Globus Pallidus↗

PubMed1989

[Palilalia associated with progressive supranuclear palsy].

Two cases of progressive supranuclear palsy (PSP) with palilalia were presented. Case 1: A 64-year-old woman was in good health until age 62 when she noted clumsiness in walking. Subsequently, forgetfulness, abrupt falls and difficulty in swallowing developed. At the age of 63, she tended to repeat same words in conversation. On admission, neurological examination revealed mental deterioration (Hasegawa's scale 19), unsteady emotional state, supranuclear vertical gaze palsy, pseudobulbar palsy, nuchal dystonia, pyramidal tract signs and extrapyramidal signs. Although spontaneous speech production was reduced, she could answer to questions with compulsive repetition of a phrase or word. She always repeated twice or three times without stuttering, logoclonus or "palilalie aphone". Case 2: A 68-year-old, right-handed woman noted double vision since about one year ago. Subsequently, she developed slowness of voluntary movement, forgetfulness and difficulty in walking. Neurological examination showed similar signs as Case 1. She was not palilaic on examination, however we found the description of her palilalia on nurse's working records. Palilalia was not present so constantly as Case 1, but continued for about a year. We suppose that palilalia is not a rare phenomenon in PSP. Although the pathogenesis of palilalia is unknown at present, we speculate the combination of extrapyramidal sign, pseudobulbar palsy and dementia may most contribute the pathogenesis of palilalia on PSP.

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PubMed1989

[Progressive supranuclear palsy].

Nine cases of progressive supranuclear palsy are reported in this paper. There are 6 males and 3 females in this series. The average age at admission was 62 years. The clinical features of those cases are: (1) onset at the presenile with gradual progression, (2) supranuclear vertical ophthalmoplegia, especially downgaze paresis, (3) disarthria, (4) gait disturbances, (5) dystonia and rigidity of the limbs, (6) clumsiness and ataxia, (7) masked face, (8) bilateral pyramidal signs and (9) mental disturbances. The CT scan in this series showed dilatation of the ventricular system, enlargement of the Sylvius fissure and cortical sulci. There was also enlargement of quadrigeminal and cisterns in the CT scan. The CT diagnosis were cerebral atrophy in 9 cases and brainstem atrophy in 8 cases in this series.

Adult↗

PubMed1999

Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration.

Progressive supranuclear palsy (PSP) and cortocobasal degeneration (CBD) are often clinically confused with each other because they share a rapid disease progression, parkinsonism that responds poorly or transiently to levodopa therapy, and associated signs (e.g., ocular abnormalities, pyramidal signs and cognitive involvement). To improve the accuracy in diagnosing these disorders, this study examined the clinical features of 51 patients pathologically diagnosed with PSP and CBD. Logistic regression analysis identified two sets of predictors (models) for CBD patients, one consisting of asymmetric parkinsonism, cognitive disturbances at onset and instability and falls at first clinic visit, and the other one of asymmetric parkinsonism, cognitive disturbances at symptom onset and speech disturbances. While PSP patients often had severe postural instability at onset, symmetric parkinsonism, vertical supranuclear gaze palsy, speech and frontal lobe-type features, CBD patients presented with lateralized motor (e.g., parkinsonism, dystonia or myoclonus) and cognitive signs (e.g., ideomotor apraxia, aphasia or alien limb). On the other hand, CBD patients presenting with an alternate phenotype characterized by early severe frontal dementia and bilateral parkinsonism were generally misdiagnosed. PSP patients without vertical supranuclear gaze palsy were misdiagnosed. Recognizing the features which differentiate these disorders and the less obvious disease presentations as well as developing an increased index of suspicion will improve the diagnostic accuracy of these disorders.

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PubMed1990

Effects of physostigmine on spatial attention in patients with progressive supranuclear palsy.

We tested patients with progressive supranuclear palsy and control subjects on a task of visuopatial attention. Targets preceded by cues on the same side were termed validly cued; and those on the opposite side, invalidly cued. For all subjects, validly cued targets were responded to faster than those that were invalidly cued. The difference between reaction times for invalidly and validly cued targets, which is hypothesized to measure attentional movement, was significantly increased for the patients. The performance of the controls on certain neuropsychological tests was correlated with their attentional ability. These correlations were altered by progressive supranuclear palsy. Physostigmine treatment of the patients induced a speeding of responses to invalidly cued targets as a function of the duration of the disease. These studies show defects in cognition and attention in patients with progressive supranuclear palsy and demonstrate that physostigmine reduces some of the abnormal visual attentional performance.

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PubMed1988

Progressive supranuclear palsy: nursing care implications.

Progressive supranuclear palsy (PSP) is an uncommon, but debilitating, neurological disease. Characterized by paralysis of eye movements, difficulty talking and swallowing, body trunk rigidity, gait ataxia, and dementia, the disease progresses rapidly over a 6-8 year time span. Death usually occurs from aspiration pneumonia. To provide meaningful care for the PSP patient and his/her family, the neuroscience nurse must first understand the disease pathophysiology, then the appropriate care interventions.

Bromocriptine↗

PubMed2005

The relationship between histopathological features of progressive supranuclear palsy and disease duration.

Progressive supranuclear palsy (PSP) is a distinct clinicopathological entity characterized by neurofibrillary tangles, tufted astrocytes, threads and oligodendroglial coiled bodies. These four lesions are distributed in varying densities throughout subcortical and brainstem structures. There is no data regarding the relationship of disease duration to lesion burden severity in PSP. We report the results of analysis of semiquantitative data collected as part of the diagnostic evaluation on 97 cases of 'pure' PSP, submitted to the Society of PSP Brain Bank. Surprisingly, the data suggests that in PSP as duration of illness increases there is a decrease in oligodendroglial tau burden.

Aged↗

PubMed2003

Natural history and clinical features of progressive supranuclear palsy: a clinical study.

Progressive supranuclear palsy (PSP or Steele-Richardson-Olszewski syndrome) is one of the most-common types of atypical parkinsonism. To characterize the natural history and the clinical features of PSP, we reviewed the records of 25 patients followed in our clinic since 1991, with a clinical diagnosis of PSP according to NINDS and Golbe criteria. Progressive onset of early bilateral bradykinesia and postural instability with falls during the 5th decade strongly support the diagnosis of PSP in our patients. Pseudobulbar symptoms are very common at onset and during the course of the illness.

Age of Onset↗

PubMed2006

Characteristics and symptom management of progressive supranuclear palsy: a multidisciplinary approach.

Progressive supranuclear palsy (PSP), a neurodegenerative disorder that may be mistaken for Parkinson's disease early in its course, is the most common type of atypical parkinsonism. PSP is fatal; it transforms a normal person into an invalid within 5-7 years of the onset of symptoms. PSP requires a multidisciplinary approach to symptom management and treatment. The neuroscience nurse who is knowledgeable about PSP and the complex way in which it overlaps with numerous other devastating neurological diagnoses can help in achieving the correct diagnosis and treatment. The nurse must understand the disease pathophysiology and the plan of care required and is instrumental in recognizing the needs of the patient and the family.

Activities of Daily Living↗

PubMed2003

Progressive supranuclear palsy presenting with primary progressive aphasia--clinicopathological report of an autopsy case.

We report a Japanese autopsy case of progressive supranuclear palsy (PSP). The male patient was 74 years old at the time of death. At age 64, he developed non-fluent aphasia that progressed slowly over 8 years, eventually associated with behavioral abnormality, postural instability, and dysphagia at 2 years prior to his death. Magnetic resonance imaging of the brain at age 73 demonstrated marked atrophy of the frontal lobes, particularly on the left side. Neuropathological examination revealed the typical pathology of PSP: loss of neurons, gliosis, occurrence of neurofibrillary tangles, oligodendroglial coiled bodies, and tuft-shaped astrocytes in the frontal cortex, associated with argyrophilic threads in the underlying white matter, in the basal ganglia, including the thalamus, globus pallidus, and subthalamic nucleus, and in the brainstem nuclei, including the substantia nigra, pontine nucleus, and inferior olivary nucleus. No astrocytic plaques or ballooned neurons were observed. Protein analysis revealed accumulation of hyperphosphorylated tau of 68 and 64 kDa consisting of the four repeat tau isoforms. We conclude that the present case represented PSP with an 8-year history of primary progressive aphasia (PPA). Although focal cortical symptoms in PSP are rare or absent, we should keep in mind the possibility of atypical PSP in which cortical pathology is predominant, particularly in the frontal lobe, and could result in PPA.

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