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PubMed1991

Pure akinesia: an atypical manifestation of progressive supranuclear palsy.

Two patients with "pure akinesia" who showed the characteristic changes of progressive supranuclear palsy (PSP) at necropsy are described. They had akinesia but no rigidity or tremor, and ophthalmoplegia was not observed during the course of illness. The symptoms of "pure akinesia" was not improved by levodopa therapy but was considerably improved by L-threo-3,4-dihydroxy-phenylserine. At necropsy, pathological findings were not different from those reported for PSP. It is suggested that "pure akinesia" is an atypical manifestation of PSP, and that norepinephrinergic neurons may be involved in some types of PSP.

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PubMed1997

Cerebral oxygen metabolism in patients with progressive supranuclear palsy: a positron emission tomography study.

To investigate cerebral oxygen metabolism in progressive supranuclear palsy (PSP), 5 patients with a clinical diagnosis of PSP and a variable degree of cognitive deficit were selected for positron emission tomography (PET) of the brain. In 4 of them, a significant decrease in oxygen metabolism was found in all cortical regions, without frontal accentuation. In the group as a whole, this decrease was even slightly more marked in parietal and temporal regions. These findings are not consistent with earlier PET studies that demonstrated frontal targeting of hypometabolism in PSP patients. Part of this discrepancy can be explained by differences in methodology, although the use of different clinical criteria and overlap of PSP with other neurodegenerative diseases must be taken into account. It is concluded that the absence of frontal hypometabolism on PET examination does not exclude the diagnosis of PSP.

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PubMed2003

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.

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PubMed2000

Neuronal loss in Onuf's nucleus in three patients with progressive supranuclear palsy.

Disorders of micturition have been reported only sporadically in patients with progressive supranuclear palsy (PSP). We report the results of a clinicopathological study of 3 patients with a definite diagnosis of PSP at various stages of their illness with sphincter abnormalities. Electromyography of the sphincter muscles was performed in all 3 patients and was abnormal in 2. Morphological and morphometric evaluation of Onuf's nucleus in the sacral spinal cord, which is involved in sphincter control, showed severe cell loss, presence of neurofibrillary tangles, neuropil threads, and glial inclusions. We conclude that bladder dysfunction and abnormal sphincter electromyographic results are due to pathological changes in Onuf's nucleus, and we propose that sphincter abnormalities should be included in the list of possible symptoms of PSP.

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PubMed2004

Voxel based morphometry reveals a distinct pattern of frontal atrophy in progressive supranuclear palsy.

BACKGROUND: Frontal lobe atrophy is a well known neuropathological feature of progressive supranuclear palsy (PSP), accompanied by characteristic neuropsychological deficits. OBJECTIVE: To determine subregional frontal lobe atrophy patterns in patients with PSP using voxel based morphometry (VBM). METHODS: VBM is an observer unbiased volumetry which allows the investigation of the entire brain. An optimised protocol for normalisation, segmentation, and correction for volume changes in preprocessing was used. Grey matter, white matter, and cerebrospinal fluid (CSF) partitions in 12 patients with probable PSP were compared with 12 healthy controls matched for age and sex. RESULTS: In PSP patients, the following cortical areas were decreased in volume (p(corr)<0.05): the prefrontal cortex, predominantly the medial frontal gyri and a cluster in the left lateral middle frontal gyrus; the insular region including the frontal opercula; both supplementary motor areas; and the left medio-temporal area (V5). White matter comparisons revealed a volume reduction in both frontotemporal regions and the mesencephalon. Analysis of the CSF compartment showed no significant regional changes between the groups. CONCLUSIONS: Frontal atrophy in PSP predominantly involves mesio-frontal targets of striatal projections. This atrophy pattern probably accounts for cardinal PSP associated behavioural deficits.

Atrophy↗
PubMed2003

Performance on the dementia rating scale in Parkinson's disease with dementia and dementia with Lewy bodies: comparison with progressive supranuclear palsy and Alzheimer's disease.

BACKGROUND: The relation between dementia with Lewy bodies (DLB) and Parkinson's disease with dementia (PDD) is unknown. OBJECTIVES: To compare the cognitive profiles of patients with DLB and PDD, and compare those with the performance of patients with a subcortical dementia (progressive supranuclear palsy) and a cortical dementia (Alzheimer's disease). DESIGN: Survey of cognitive features. SETTING: General community in Rogaland county, Norway, and a university dementia and movement disorder research centre in the USA. PATIENTS: 60 patients with DLB, 35 with PDD, 49 with progressive supranuclear palsy, and 29 with Alzheimer's disease, diagnosed by either standardised clinical procedures and criteria (all PDD and Alzheimer cases and 76% of cases of progressive supranuclear palsy), or necropsy (all DLB cases and 24% of cases of progressive supranuclear palsy). Level of dementia severity was matched using the total score on the dementia rating scale adjusted for age and education. MAIN OUTCOME MEASURES: Dementia rating scale subscores corrected for age. RESULTS: No significant differences between the dementia rating scale subscores in the PDD and DLB groups were found in the severely demented patients; in patients with mild to moderate dementia the conceptualisation subscore was higher in PDD than in DLB (p = 0.03). Compared with Alzheimer's disease, PDD and DLB had higher memory subscores (p < 0.001) but lower initiation and perseveration (p = 0.008 and p=0.021) and construction subscores (p = 0.009 and p = 0.001). DLB patients had a lower conceptualisation subscore (p = 0.004). Compared with progressive supranuclear palsy, PDD and DLB patients had lower memory subscores (p < 0.001). CONCLUSIONS: The cognitive profiles of patients with DLB and PDD were similar, but they differed from those of patients with Alzheimer's disease and progressive supranuclear palsy. The cognitive pattern in DLB and PDD probably reflects the superimposition of subcortical deficits upon deficits typically associated with Alzheimer's disease.

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PubMed1989

Autologous adrenal medullary transplant in progressive supranuclear palsy.

We transplanted autologous adrenal medullary cells to the caudate nucleus in 3 patients with progressive supranuclear palsy, using the method Madrazo has employed for neural transplantation in Parkinson's disease. Major and minor complications occurred post-operatively from which the patients recovered. One patient had a marked improvement in his postural stability and a decreased incidence of falling. This change was evident at 1 month after surgery and has remained for the 6 months of follow-up. Postural reflexes were not altered in the other 2 patients. There was no change in extraocular movements, speech, or the rigid-bradykinetic features of parkinsonism in any patient. Adrenal medullary transplantation has only limited efficacy in progressive supranuclear palsy.

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PubMed2003

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

OBJECTIVE: To describe clinical features and identify prognostic predictors in progressive supranuclear palsy (PSP). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

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PubMed1992

Dopamine D1 and D2 receptors in progressive supranuclear palsy: an autoradiographic study.

Dopamine D1 and D2 receptors were studied in brain tissue sections from a typical patient with progressive supranuclear palsy and in 7 age-matched brains. The density of D1 receptors in the caudate-putamen and frontal cortex of the patient was within control limits. By contrast, the density of nigral D1 receptors and striatal D2 receptors was dramatically reduced in the patient as compared to the control brains. This work shows again that the loss of striatal D2 receptors is the most plausible explanation for the poor response to dopaminergic drugs in patients with progressive supranuclear palsy. While the loss of nigral D1 receptors can be explained by the loss of nigral neurons, it seems that neurons bearing striatal D1 receptors are spared in progressive supranuclear palsy. The clinical effects of selective D1 agonists are worth testing in this devastating disorder.

Atrophy↗
PubMed2005

Quantitative MRI measurement of superior cerebellar peduncle in progressive supranuclear palsy.

BACKGROUND: Postmortem studies have shown atrophy of the superior cerebellar peduncle (SCP) to distinguish progressive supranuclear palsy (PSP) from other neurodegenerative diseases. It is not clear whether MRI-based measurements can differentiate this relative atrophy of the SCP during life. METHODS: Volumetric MRI was acquired prospectively in 53 subjects: 19 with PSP, 10 with multiple system atrophy (MSA), 12 with Parkinson disease (PD), and 12 healthy controls. SCP volume was assessed by detailed quantitative volumetric measurement and independently by blinded visual rating of SCP atrophy. RESULTS: The mean SCP volume, corrected for total intracranial volume, was lower in patients with PSP than controls (p < 0.001), patients with MSA (p = 0.001), and patients with PD (p = 0.003). There was an overlap between individual SCP volume measurements in the PSP subjects and the other groups. Neuroradiologic rating correctly identified PSP cases based on the presence of SCP atrophy with a sensitivity of 74% and a specificity of 94%. CONCLUSIONS: The authors propose that together with other radiologic features of progressive supranuclear palsy (PSP) such as midbrain atrophy, a visual assessment of the superior cerebellar peduncle may help increase the clinical diagnostic accuracy in PSP.

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PubMed2003

Progressive supranuclear palsy phenotype secondary to CADASIL.

BACKGROUND AND PURPOSE: To report a unique case of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy manifesting as a progressive supranuclear palsy phenotype, thereby expanding its recognized presentations. METHODS: Review of the pertinent literature from MEDLINE, cross-referencing cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, progressive supranuclear palsy, and parkinsonism. Description of a 60-year-old woman who presented with a several year history of step-wise, progressive parkinsonism secondary to cerebral autosomal dominant arteriopathy. RESULTS: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy may present with a progressive supranuclear phenotype. CONCLUSION: Parkinsonism, including a progressive supranuclear palsy phenotype, is one of a growing number of recognized ways that cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy may present.

Dementia, Multi-Infarct↗
PubMed2003

Progressive supranuclear palsy: analysis of six cases.

We analyzed six cases of progressive supranuclear palsy (PSP) from 1 January 1998 to 31 June 2002. All patients underwent a complete range of laboratory tests, head computed tomographic (CT) scan or magnetic resonance imaging (MRI); three were evaluated by formal neuropsychological examination. All were taking L-dopa or dopamine agonists. At the onset of PSP the initial diagnosis was almost always Parkinson's disease. The head CT scan or MRI findings were non-specific, while neuropsychological testing disclosed cognitive impairment, with predominant subcortical-frontal involvement. We emphasize the still obvious current difficulty in diagnosing PSP at an early stage in clinical practice. Therefore it is essential to formulate better clinical diagnostic criteria, to permit correct management of the disease.

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PubMed1994

The syndrome of 'pure akinesia' and its relationship to progressive supranuclear palsy.

Five patients developed a stereotyped syndrome of progressive akinesia of gait, speech, and hand-writing without rigidity, tremor, or dementia. The symptoms did not improve with levodopa. These clinical findings conform to a syndrome described repeatedly in Japan since 1974 as "pure akinesia." Evidence has indicated that pure akinesia often represents a pre-ocular motor, and in some cases an ocular motor-sparing, form of progressive supranuclear palsy (PSP). Although we found disorders of eyelid movements, none of the patients demonstrated a gaze palsy on clinical examination. Four patients underwent eye movement recordings. The two patients with the longest disease duration had slow or small vertical saccades. These findings support the notion that patients with pure akinesia may develop a vertical gaze palsy, similar to that in PSP, late in their course. Our patients show that pure akinesia occurs in North America as well as in Japan. Recognition of the syndrome of pure akinesia may suggest the diagnosis of PSP before the development of abnormalities of ocular movement.

Dementia↗
PubMed2002

Expression and activity of antioxidants in the brain in progressive supranuclear palsy.

Recent evidence implicates oxidative stress in the pathophysiology of progressive supranuclear palsy (PSP). Thus, we undertook a study of the activity and localization of two essential antioxidant systems (superoxide dismutase [SOD] enzymes and total glutathione) in the human post-mortem PSP and control brain. Marked increases in SOD1 (Cu/ZnSOD) activity and glutathione levels were measured within most PSP brain regions examined, whereas, only the subthalamic nucleus exhibited a significant increase (+68%) in SOD2 (MnSOD) activity. Two additional cases with mild pathological abnormalities were studied. The first (case A) may represent an example of an asymptomatic PSP case, while the second (case B) had mild pathological abnormalities consistent with typical PSP. In case A, only the STN had elevated levels of SOD activity, in the absence of an increase in tissue glutathione content. In case B, SOD activities and tissue glutathione content were elevated in several regions. Immunolocalization of the SOD1 and SOD2 proteins in paraffin-embedded tissue sections revealed a marked increase in the density of SOD immunopositive profiles (particularly glia) in the typical PSP brain, particularly within the white matter. Together, our data argues strongly in favor of the involvement of oxidative stress in the etiology and progression of PSP, and suggests that deficit in SOD or glutathione metabolism are not causative.

Antioxidants↗
PubMed1986

Progressive supranuclear palsy with Lewy bodies.

An autopsy case is reported which revealed not only clinical and neuropathological features of progressive supranuclear palsy, but also the presence of large numbers of Lewy bodies in the brain stem nuclei and cerebral cortex. This case seems to be progressive supranuclear palsy with Lewy bodies distributed as in Parkinson's disease. Such case has not been previously reported.

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PubMed2001

Mitochondrial impairment in the cerebellum of the patients with progressive supranuclear palsy.

Abnormalities in energy metabolism and oxidative stress accompany many neurodegenerative diseases, including progressive supranuclear palsy (PSP). Previously, we showed decreased activities of a mitochondrial enzyme complex, alpha-ketoglutarate dehydrogenase complex (KGDHC), and marked increases in tissue malondialdehyde levels in post-mortem superior frontal cortex from the patients with PSP. The current study demonstrates that KGDHC is also significantly diminished (-58%) in the cerebellum from patients with PSP (n = 14), compared to age-matched control brains (n = 13). In contrast to cortex, markers of oxidative stress, such as malondialdehyde, tyrosine nitration or general protein carbonyl modification, did not increase in cerebellum. Furthermore, the protein levels of the individual components of KGDHC did not decline. The activities of two other mitochondrial enzymes were measured to determine whether the changes in KGDHC were selective. The activity of aconitase, a mitochondrial enzyme with an iron/sulfur cluster, is also significantly diminished (-50%), whereas glutamate dehydrogenase activity is unchanged. The present results suggest that the interaction of metabolic impairment and oxidative stress is region-specific in PSP brain. In cerebellum, reductions in KGDHC occur in the absence of increases in common measures of oxidative stress, and may underlie the metabolic deficits and contribute to pathological and clinical manifestation related to the cerebellum in patients with PSP.

Aconitate Hydratase↗
PubMed2003

Health-related quality of life in patients with progressive supranuclear palsy.

We assessed health-related quality of life (QoL) of patients with progressive supranuclear palsy (PSP), identified the most important QoL issues in patients with this disorder, and assessed the usefulness of existing QoL measures in patients with PSP. Twenty-seven patients in all stages of PSP and their carers underwent a semistructured in-depth interview on the impact of PSP and a neurological examination. They were also asked to complete existing measures of QoL and depression. An item-pool of issues relevant to QoL of patients with PSP was created from the patient and carer interviews. Carers and patients largely agreed on issues relevant for patients' QoL but more carers than patients considered symptoms of frontal lobe dysfunction as problematic for the patients. There was no association of QoL with age and gender, as assessed in interviews and on two QoL instruments. QoL deteriorated with increasing disease duration and severity and greater cognitive impairment and was associated with worse depression scores. While the generic SF-36 was not found to be useful to assess QoL in PSP, feasibility and validity for the PDQ-39 and the EQ-5D were acceptable in this study. However, additional issues relevant to patients with PSP that were not addressed in these instruments included visual disturbances, dysarthria, dysphagia, muddled thinking, confusion, and apathy. The generic EQ-5D and the Parkinson's disease-specific PDQ-39 are useful instruments to assess QoL in patients with PSP. However, they lack questions on important aspects of QoL in PSP that were reported by patients and carers in semistructured interviews. The item pool created in these interviews provides the basis for the development of disease-specific QoL instruments for patients with PSP.

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