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Results for “Multiple System Atrophy”

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PubMed2003

MRI-based volumetric differentiation of sporadic cerebellar ataxia.

The term idiopathic cerebellar ataxia (IDCA) designates a variety of cerebellar syndromes that may present with a purely cerebellar syndrome (IDCA-C) or with additional extracerebellar features (IDCA-P). Multiple system atrophy is also a sporadic neurodegenerative disorder of unknown origin that may cause prominent cerebellar symptoms (MSA-C). The final neuropathological answer to the question whether IDCA-P and MSA-C represent different varieties of one disease or two distinct entities is still lacking. Three-dimensional MRI-based volumetry allows morphological investigations intra vitam. Volumetric analysis of cerebellum, brainstem and basal ganglia was therefore performed in 46 patients with sporadic cerebellar ataxia and 16 age-matched healthy controls. Patients with dementia were excluded from the study since cognitive impairment is an exclusion criterion for the diagnosis of MSA. Cerebellar patients were clinically divided into two groups: 33 patients with multiple system atrophy with prominent cerebellar symptoms (MSA-C) and 13 patients with extracerebellar features not corresponding to MSA-C (IDCA-P). There was evidence for substantial cerebellar atrophy in both cerebellar groups while additional brainstem atrophy was significantly more pronounced in MSA-C patients. Absolute caudate and putamen atrophy was found to be restricted to single MSA-C individuals while group comparisons of mean volumes did not yield significant differences from controls. Based on the volumetric data, diagnosis could be correctly predicted in 94% of control, 82% of MSA-C and 100% of IDCA-P individuals. The finding of specific imaging characteristics strengthens (i) the value of MRI volumetry in separating MSA-C from other types of sporadic cerebellar ataxia, and (ii) the hypothesis of two independent neurodegenerative disorders in MSA-C and IDCA-P.

Adult↗

PubMed2004

Autonomic dysfunction in dementia with Lewy bodies.

OBJECTIVE: To assess autonomic function in patients with dementia with Lewy bodies (DLB). METHODS: The authors compared data from 20 DLB patients evaluated from 1995 to 2000 to 20 age-matched multiple system atrophy (MSA) and Parkinson disease (PD) patients evaluated from 1999 to 2002. Analysis of variance, Fisher exact test, and Student t-test were applied to compare disease characteristics, autonomic symptoms, and function tests on the Composite Autonomic Scoring Scale (CASS) and Thermoregulatory Sweat Test (TST). RESULTS: In DLB, mean age at onset of autonomic symptoms was 70.3 +/- 8.9 years. Orthostatic symptoms were common and orthostatic hypotension occurred in 10/20 DLB, 17/20 MSA, and 1/20 PD patients (p = 0.023, 0.003). CASS-sudomotor for DLB, MSA, and PD were 1.6 +/- 1.2, 2.5 +/- 0.7, and 0.9 +/- 0.8 (p < 0.00001). CASS-cardiovagal were 1.4 +/- 0.9, 2.1 +/- 0.8, and 0.7 +/- 0.6 (p < 0.00001). CASS-adrenergic function were 2.4 +/- 1.2, 3.5 +/- 0.9, and 0.5 +/- 0.6 (p < 0.00001). Total CASS were 5.2 +/- 2.0, 8.1 +/- 1.3, and 2.2 +/- 1.2 (p < 0.00001). The most common pattern of TST in DLB was distal anhidrosis. Mean duration of follow-up was 3.0 +/- 1.8 years. Six patients needed medication to maintain blood pressure and five had good response. CONCLUSIONS: Autonomic dysfunction is frequent in dementia with Lewy bodies and the severity is intermediate between that of multiple system atrophy and Parkinson disease.

Acetylcholine↗

PubMed2000

Visual contrast response functions in Parkinson's disease: evidence from electroretinograms, visually evoked potentials and psychophysics.

OBJECTIVES: Visual contrast detection thresholds and suprathreshold contrast discrimination thresholds were compared to luminance and flash/pattern electroretinograms (ERG) and visually evoked potentials (VEP) in patients with Parkinson's disease (n = 31), patients with multiple system atrophy (n = 6), patients with progressive supranuclear palsy (n = 6) and control patients without central nervous disease (n = 33). METHODS: The stimuli were luminance modulated full-field (flash) or horizontally oriented sinewave gratings (pattern), the latter having either a low (0.5 cycles/deg) or medium (4.0 cycles/deg) spatial frequency. Stimulus contrast ranged from 10 to 80% so that contrast response functions could be derived. RESULTS: Contrast thresholds were higher in the patients with Parkinson's disease than in the control patients. Contrast discrimination thresholds were also somewhat elevated in patients with Parkinson's disease. Pattern ERG amplitudes were significantly reduced in patients with Parkinson's disease for the medium spatial frequency stimulus, but less for the low spatial frequency and flash stimuli. CONCLUSIONS: Our results suggest that Parkinson's disease impairs contrast processing in the retina. VEP amplitudes did not significantly differ between the groups for the conditions tested. Patients with progressive supranuclear palsy also showed impaired contrast perception and reduced ERG amplitudes, whereas patients with multiple system atrophy were less impaired.

Aged↗

PubMed1994

Magnetic resonance imaging in progressive supranuclear palsy and other parkinsonian disorders.

High field intensity MRI may demonstrate signal abnormalities consistent with deposits of iron or other paramagnetic substances in several extrapyramidal disorders. Hallervorden-Spatz disease was the only disorder widely known to have iron deposits in the pallidum, that are now easily demonstrated in vivo by MRI. However, lower field intensity MRI may also demonstrate characteristic findings. In progressive supranuclear palsy, definite atrophy of the midbrain and of the region around the third ventricle is seen in slightly more than half of the cases. Minimal signal abnormalities are sometimes seen in the periaqueductal region, but MRI studies remain of little help in establishing the diagnosis of the disease. Asymmetric atrophy in the parietal regions is seen in corticobasal degeneration, as expected from pathological studies. Minimal alterations may be seen in the substantia nigra in Parkinson's disease. The most interesting MRI findings are observed in multiple system atrophies. Variable abnormal signal intensities, depending on the field intensity, are visible in the putamen in striatonigral degeneration and in Shy-Drager syndrome; in this latter condition the abnormalities are due to its striatonigral degeneration component. Atrophy of the pons, middle cerebellar peduncles, and cerebellum, and signal abnormalities in a characteristic distribution are visible in olivopontocerebellar atrophy. A combination of these posterior fossa abnormalities and putaminal alterations may confirm the involvement of the cerebellar and extrapyramidal systems in multiple system atrophies.

Atrophy↗

PubMed2003

Dysautonomia in Parkinson's disease: neurocardiological abnormalities.

Symptoms of abnormal autonomic-nervous-system function occur commonly in Parkinson's disease (PD). Orthostatic hypotension in patients with parkinsonism has been thought to be a side-effect of treatment with levodopa, a late stage in the disease progression, or, if prominent and early with respect to disordered movement, an indication of a different disease, such as multiple system atrophy. Instead, patients with PD and orthostatic hypotension have clear evidence for baroreflex failure and loss of sympathetic innervation, most noticeably in the heart. By contrast, patients with multiple system atrophy, which is difficult to distinguish clinically from PD, have intact cardiac sympathetic innervation. Post-mortem studies confirm this distinction. Because PD involves postganglionic sympathetic noradrenergic lesions, the disease seems to be not only a movement disorder with dopamine loss in the nigrostriatal system of the brain, but also a dysautonomia, with norepinephrine loss in the sympathetic nervous system of the heart.

Animals↗

PubMed2005

Characteristics of idiopathic REM sleep behavior disorder and that associated with MSA and PD.

OBJECTIVE: To compare the clinical and video-polysomnographic (VPSG) characteristics of idiopathic REM sleep behavior disorder (RBD) vs the RBD seen in multiple system atrophy (MSA) and Parkinson disease (PD). METHODS: Clinical features and VPSG measures were evaluated in 110 consecutive nondemented subjects (26 MSA, 45 PD, and 39 idiopathic RBD) free of psychoactive medications referred for suspected RBD to our sleep unit over a 5-year period, with extended follow-up (mean 26.9 +/- 21.3 months). RESULTS: Across the three groups studied, logistic regression analysis demonstrated that there were no differences in the quality of RBD symptoms (e.g., nature of unpleasant dream recall or behaviors witnessed by bed partners), most PSG variables, abnormal behaviors captured by VPSG, and clinical response to clonazepam. When compared to subjects with PD, however, patients with MSA had a significantly shorter duration of disease, a higher REM sleep without atonia percentage, a greater periodic leg movement index, and less total sleep time. Subjects with idiopathic RBD, as compared to those with either MSA or PD, were more often male, had greater self-reported clinical RBD severity, and were more often aware of their abnormal sleep behaviors. CONCLUSIONS: REM sleep behavior disorder (RBD)-related symptoms and neurophysiologic features are qualitatively similar in RBD subjects with the idiopathic form, multiple system atrophy (MSA), and Parkinson disease (PD). Polysomnographic abnormalities associated with RBD in the setting of MSA are greater than in PD, suggesting a more severe dysfunction in the structures that modulate REM sleep.

Age of Onset↗

PubMed2006

Feasibility of 2-deoxy-2-[18F]fluoro-D-glucose- A85380-PET for imaging of human cardiac nicotinic acetylcholine receptors in vivo.

Nicotinic acetylcholine receptors mediate the parasympathetic autonomic control of cardiac function. Aim of this study was the assessment of cardiac nicotinic acetylcholine receptor distribution with a novel (alpha4beta2) nicotinic acetylcholine receptor PET ligand (2-deoxy-2- [18F]fluoro-D-glucose-A85380) in humans. Five healthy volunteers without cardiac disease and six patients with either Parkinson's disease or multiple system atrophy without additional overt cardiac disease were evaluated with 2-deoxy-2-[18F]fluoro-D-glucose-A85380 PET-imaging to assess the cardiac parasympathetic innervation and the putative impact of both disorders. 2-deoxy-2- [18F]fluoro-D-glucose-A85380 whole body PET-scans were performed on a Siemens PET/CT biograph(TM) 75.4 min +/- 6.7 after i.v. injection of 371.2 +/- 58.1 MBq. Average count rate density of left ventricle ROI's and a standard ROI in the right lung were measured within three consecutive slices of 10.0 mm thickness. Heart-to-lung ratios were calculated in each volunteer and patient. Tracer uptake in the left ventricle could be measured in all of the five volunteers and the six patients. Heart-to-lung ratios in the volunteer group were not different from patients suffering from Parkinson's disease or MSA (3.2 +/- 0.5 vs 3.2 +/- 0.8 and 2.96+/-0.7, mean +/- SD), respectively. Human cardiac nicotinic acetylcholine receptors can be visualized and measured by 2-deoxy-2- [18F]fluoro-D-glucose-A85380 PET scans both in cardiac-healthy subjects and patients suffering from Parkinson's disease or multiple system atrophy. The heart- as well as the lung-tracer uptake was almost constant throughout all subjects leading to a good target-to-background ratio. These first results suggest no impact of either PD or MSA on cardiac nicotinic acetylcholine receptors.

Aged↗

PubMed1991

Autonomic involvement in extrapyramidal and cerebellar disorders.

We reviewed the clinical and autonomic features of all patients with extrapyramidal and cerebellar disorders studied in the Mayo Autonomic Reflex Laboratory from 1983 to 1989. Patients were grouped into the following categories (number in parentheses): Parkinson's disease (35); parkinsonism-plus (54); multiple system atrophy (75); hereditary multisystem degenerations (eleven); progressive supranuclear palsy (32); non-familial cerebellar degeneration (eleven); nonspecific sporadic multisystem degeneration (73). Severe autonomic failure occurred in 97% of patients with multiple system atrophy and 53% of the nonspecific sporadic multisystem degeneration patients respectively. Autonomic involvement was mild or absent in Parkinson's disease while parkinsonism-plus and non-familial cerebellar degeneration patients had moderate autonomic failure. Orthostatic blood pressure reduction, percentage of anhidrosis on thermoregulatory sweat test, quantitative sudomotor axon reflex test, forearm response and heart rate response to deep breathing strongly regressed with severity. A response to levodopa treatment in patients with parkinsonism was more likely if cerebellar signs and cognitive deficits were absent. The presence of levodopa induced dyskinesia was also a marker for a clinically favourable levodopa response. We conclude that there is a spectrum of autonomic involvement in these degenerative disorders and that autonomic studies are useful in separating them and monitoring their course.

Adult↗

PubMed2004

Apolipoprotein E epsilon 4 is a determinant for Alzheimer-type pathologic features in tauopathies, synucleinopathies, and frontotemporal degeneration.

OBJECTIVES: To determine if apolipoprotein E epsilon 4 influences the frequency of Alzheimer-type pathologic features in tauopathies, synucleinopathies, and frontotemporal degeneration and to determine if the frequency of Alzheimer-type pathologic features in synucleinopathies is similar to the frequency of such features in tauopathies and frontotemporal degeneration. METHODS: A total of 285 patients with pathologically proven neurodegenerative disorders, including diffuse and transitional Lewy body disease, frontotemporal degeneration, progressive supranuclear palsy, corticobasal degeneration, and multiple system atrophy, with a mean age of 75.1 +/- 9.3 years, were suitable for genetic and pathological analysis. Disorders were grouped as tauopathies (progressive supranuclear palsy and corticobasal degeneration), synucleinopathies (Lewy body disease and multiple system atrophy), and frontotemporal degeneration. Braak neurofibrillary tangle staging and quantitative scores of senile plaques were used to determine the degree of concomitant Alzheimer-type pathologic features in each case, and apolipoprotein E genotype was determined from DNA isolated from frozen brain tissue. The relationship of apolipoprotein E epsilon 4 to Alzheimer-type pathologic features was determined. RESULTS: Across all neurodegenerative disorders, apolipoprotein E epsilon 4 and older age independently predicted the co-occurrence of Alzheimer-type pathologic features (P<.001), whereas female sex had a lesser effect (P = .03). When divided into the 3 subgroups (tauopathies, synucleinopathies, and frontotemporal degeneration), apolipoprotein E epsilon 4 had a similar effect, whereas older age and female sex were less predictive. There was a significant difference between the frequency of Alzheimer-type pathologic features in synucleinopathies and the frequency of such features in tauopathies and frontotemporal degeneration (P<.001 for both). The frequency of apolipoprotein E epsilon 4 allele was not significantly different among the 3 groups. CONCLUSIONS: Apolipoprotein E epsilon 4, independent of older age and sex, contributes to the co-occurrence of Alzheimer-type pathologic features in tauopathies, synucleinopathies, and frontotemporal degeneration, but this does not explain why Alzheimer-type pathologic features are significantly more likely to coexist with synucleinopathies than with either tauopathies or frontotemporal degeneration.

Aged↗

PubMed2004

The spectrum of antineuronal autoantibodies in a series of neurological patients.

The aim of the present study is to identify the range of neurological disorders expressing antineuronal antibodies, evaluate the number of different patterns of reactivity that can be detected, and analyse the contribution of these studies to the identification of subgroups of patients. The records of 882 patients were reviewed and their sera and cerebrospinal fluids tested for antineuronal antibodies. Patients were initially divided into four groups according to suspected clinical diagnosis. Autoantibodies were detected by immunohistochemistry, Western blot of gradient-separated neuronal and recombinant proteins and by RIA. Cerebellar degeneration and sensory neuropathies were the most common neurological disorders in which paraneoplastic-related anti-neuronal antibodies were detected. However, in addition to PCA1/anti-Yo and ANNA1/anti-Hu antibodies, we found other reactivities in six patients with cerebellar degeneration: anti-GAD in three females and atypical in the other cases. The widest range of different anti-neuronal antibodies was detected in patients with peripheral sensory neuropathy. Few patients with Stiff-Person syndrome, temporal lobe epilepsy and myoclonus harboured anti-GAD antibodies. Atypical antibodies were detected in single cases with motor neuron disorder and multiple system atrophy. No anti-neuronal antibodies were detected in patients with neurological complications of connective tissue disorders other than Sjögren's syndrome, or in neurological diseases other than motor neuron disease and multiple system atrophy. Our study shows that the spectrum of neurological disorders in which anti-neuronal antibodies can be detected is wider than previously thought. In addition, we found patterns of neuronal staining and Western blot reactivity that differed from those so far reported. This may permit identification of subgroups of patients in whom strategies directed at removing and/or suppressing antibody production could be of some benefit.

Antibodies, Neoplasm↗

PubMed1991

The effects of the somatostatin analogue, octreotide, on postural hypotension, before and after food ingestion, in primary autonomic failure.

The effects of the somatostatin analogue, octreotide on postural hypotension have been compared with placebo, before and after food ingestion in two groups with primary autonomic failure; patients with pure autonomic failure, and patients with additional neurological involvement as part of multiple system atrophy. After placebo, supine blood pressure was unchanged, but after octreotide, it rose in both groups. Octreotide reduced pre-prandial postural and supine post-prandial hypotension in both pure autonomic failure and multiple system atrophy patients. Postural hypotension post-prandially was considerably worse after placebo; this was reduced after octreotide. Plasma noradrenaline and adrenaline levels remained unchanged. Plasma glucose levels rose higher and faster after placebo. Insulin levels were similar in both groups at rest, but rose higher in patients with pure autonomic failure after placebo. After octreotide, the insulin response in both groups was suppressed. We conclude that octreotide prevents post-prandial hypotension in both groups with primary autonomic failure and additionally reduces postural hypotension both before and after food ingestion. The greater rise in insulin levels in patients with pure autonomic failure suggests that insulin may be a contributing factor to the more severe post-prandial hypotension observed in this group of patients.

Adult↗

PubMed1999

Epitope mapping of LB509, a monoclonal antibody directed against human alpha-synuclein.

Alpha-synuclein is a 140 amino acid protein that forms the major component of the abnormal filaments that make up the Lewy bodies and Lewy neurites of Parkinson's disease and dementia with Lewy bodies. It is also the major component of the filamentous glial cytoplasmic inclusions of multiple system atrophy. Here we have used recombinant alpha-synucleins and peptide competition to show that the monoclonal anti-alpha-synuclein antibody LB509 recognizes amino acids 115-122 of human alpha-synuclein. The antibody strongly labelled filaments extracted from multiple system atrophy brain, showing the presence of residues 115-122 of alpha-synuclein. LB509 failed to react with mouse, rat and zebra finch alpha-synuclein, because of amino acid differences with human alpha-synuclein. Since LB509 recognizes human but not rodent alpha-synuclein, it will be a useful reagent for the characterization of mouse lines transgenic for human alpha-synuclein.

Animals↗

PubMed1999

Skin pathology findings in a cohort of 1500 adult and elderly subjects.

BACKGROUND: No extensive studies are available in the literature on the eventual skin pathology induced by neurologic or systemic diseases in elderly individuals. Other factors, such as health and hygiene, socioeconomic status, and climate can also play an important role. METHODS: Fifteen-hundred subjects (886 women and 614 men; mean age, 67.8 years; range, 39-90 years) were admitted to the Department of Geriatrics at the Oasi Institute between 1992 and 1997; all these subjects were carefully evaluated from a dermatologic point of view. Each subject underwent specialist examinations, routine blood analyses, thoracic X-rays, cerebral computerized tomography (CT) scan, and magnetic resonance imaging (MRI) when appropriate. A group of subjects without significant neurologic or systemic disease, comprising 116 women and 60 men (mean age, 64.5 years; range, range, 40-90 years), was selected and used as a normal control group. Subsequently, our attention was focused on the eventual presence of the following neurologic diseases: Alzheimer-type dementia, vascular dementia, mixed-type dementia, subcortical dementia, Parkinson's disease, vascular brain disease, hemiplegia, etc. Thus, different subgroups were formed on the basis of such diagnostic categories and the frequency of skin pathology in each subgroup was evaluated. RESULTS: Of the 1500 subjects, 1439 stated that they had never been affected by dermatologic disease. No statistically significant difference in frequency of skin pathology was found between normal controls and the different patient subgroups. Unsuspected and singular dermatoses were found, however, such as paraneoplastic syndromes, idiopathic tripe palms, white fibrous papulosis of the neck as an expression of photoaging, conditions induced by former popular traditions of Sicilian culture (anetoderma secondary to the application of Hirudo medicinalis and erythema ab igne), pigmented dermatoses never described before in Italy (prurigo pigmentosa and friction amyloidosis), and nail abnormalities (atypical half-and-half nail, and dyschromic nail changes in multiple system atrophy and in hemiplegia). CONCLUSIONS: The dermatologic screening performed in 1500 patients revealed several unexpected diagnoses and some original observations. Some rare dermatoses were described and certain hypotheses were suggested to explain the peculiar dyschromic changes of the fingernails in multiple system atrophy, the atypical cases of half-and-half nail, and the so-called idiopathic tripe palms associated with psoriasis.

Adult↗

PubMed1976

Dysautonomia in Parkinsonism: a clinicopathological study.

Necropsy studies were done on six patients with idiopathic paralysis agitans, one with multiple system atrophy including features of Parkinsonism, and one control. Autonomic functions had been evaluated during life to a varying degree. Intra-arterial blood pressure studies were carried out on two patients with paralysis agitans (cases 4 and 6) and the one with multiple system atrophy (case 7). Lewy bodies with or without cell loss were seen in the sympathetic ganglia of five cases of paralysis agitans. Three of these had orthostatic hypotension and the severity of the lesion approximately correlated with the degree of hypotension. It is concluded that the lesions of the sympathetic ganglia may play a major role in the production of orthostatic hypotension in idiopathic paralysis agitans.

Aged↗

PubMed1996

Decreased cerebrospinal fluid levels of substance P in Machado-Joseph disease.

To clarify the mechanism of brain impairment in Machado-Joseph disease (MJD), we measured the cerebrospinal fluid (CSF) levels of substance P in 7 patients (mean age 45.7 +/- 12.09 years) with this disease. Four patients had type I and three had type II disease. Findings were compared with those obtained in 14 age-matched controls, 8 patients with Parkinson's disease, 7 patients with multiple system atrophy, and 6 patients with myopathy. The CSF level of substance P was significantly (p = 0.0000) lower in the patients with MJD, being 44.5% of the control value. However, the mean CSF levels of substance P in the patients with Parkinson's disease, multiple system atrophy, or myopathy did not differ significantly from that in the control subjects. The alteration in the CSF level of substance P may be related to the neurological impairment observed in MJD.

Adult↗

PubMed1992

[Dysautonomia and multi-systemic atrophy of the nervous system (Shy-Drager's syndrome)].

The clinical expressions of primary autonomic nervous system failure are more or less numerous, orthostatic hypotension being only one of them. Clinical analysis reveals 3 categories of manifestations: pure progressive dysautonomia, dysautonomia associated with Parkinson's disease, and dysautonomia associated with multiple system atrophy of the nervous system also known as Shy-Drager syndrome. Neuropathological studies show that lesions of the efferent autonomic nervous system (tractus intermediolateralis, sympathetic ganglia) are frequently associated with lesions of the central nervous system the role of which in dysautonomia is still imperfectly known. Lesions of the central nervous system may present as genuine Parkinson's disease with Lew bodies or as multiple systemic atrophy with its two best individualized aspects: striatonigral atrophy and olivopontocerebellar atrophy. These various neurological aspects have their counterpart in biochemical abnormalities, prognosis and response to treatment.

Aged↗

PubMed2006

Measuring brain stem and cerebellar damage in parkinsonian syndromes using diffusion tensor MRI.

OBJECTIVE: To use diffusion tensor MRI to quantify and compare degeneration of the pons and cerebellar peduncles in multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and Parkinson disease (PD) and to relate changes in diffusion measures to clinical features and localized atrophy. METHODS: We used a region-of-interest approach to measure changes in fractional anisotropy and mean diffusivity in the middle cerebellar peduncles, decussation of the superior cerebellar peduncles, and pons in 17 patients with MSA, 17 with PSP, 12 with PD, and 12 healthy volunteers. We also evaluated atrophy of the cerebellar peduncles and pons on T2-weighted magnetic resonance images in patients with MSA and PSP. RESULTS: In MSA, fractional anisotropy was markedly reduced in the middle cerebellar peduncles, and mean diffusivity increased both here and in the pons compared with other groups, whereas in PSP, mean diffusivity was strikingly increased in the decussation of superior cerebellar peduncles. Cerebellar ataxia was related to mean diffusivity in the middle cerebellar peduncles (r = 0.71, p = 0.001) and pons (r = 0.60, p = 0.01) in MSA. Diffusion measures were related to localized atrophy in both MSA and PSP. CONCLUSIONS: Diffusion tensor MRI can be used to quantify neurodegenerative processes in different brain stem and cerebellar structures in multiple system atrophy and progressive supranuclear palsy during life, and may have diagnostic value. Larger studies of early, undifferentiated parkinsonian syndromes are indicated to provide estimates of the relative diagnostic value of diffusion measures, atrophy measures, and visual assessment of scans.

Aged↗

PubMed1997

Presymptomatic hypertension is a major feature in the diagnosis of progressive supranuclear palsy.

OBJECTIVE: To examine the history of hypertension (HT) in patients with parkinsonism (PS) of various causes. PATIENTS: Nine hundred twenty-three patients with PS listed in a citywide movement disorders registry. Hypertension was defined as blood pressure above 150/90 mm Hg on at least 2 occasions or a history of antihypertensive medication use. RESULTS: Overall, 184 patients (20.6%) had a history of HT. Fifty-three (16.6%) of 320 patients with levodopa-responsive parkinsonism (PD) had a history of HT; similar prevalence of presymptomatic HT was seen for PS and dementia (22/165 [13.3%]), including probable diffuse Lewy body disease and multiple system atrophy of the nigrostriatal degenerative type (33/171 [19.3%]). Olivopontocerebellar atrophy (7/40 [18%]), corticobasal ganglionic degeneration (5/15 [33.3%]), drug-induced PS (9/38 [23.1%]), and the predominantly tremulous forms of Parkinson disease (17/43 [39.5%]) showed a higher prevalence of HT. Patients with familial PS (n = 36), early-onset PD (n = 14), multiple system atrophy of the Shy-Drager type (n = 11), and postencephalitic PS (n = 6) had no history of HT. In 100 patients with PS of various rare etiologies, a history of HT was seen in less than 5%. The highest prevalence of HT was seen in patients with clinically diagnosed progressive supranuclear palsy (n = 42), of whom 34 (81.0%) had a history of HT. CONCLUSIONS: A presymptomatic history of HT is a major feature in the clinical history of progressive supranuclear palsy and may be a diagnostic criterion. Its significance is unknown, but adrenergic nuclei of the brainstem are severely affected, and HT may be the first symptom arising from involvement of these nuclei. This could also explain the features of small vessel disease seen on computed tomography or magnetic resonance imaging in 50% of our patients, as in previous reports.

Adult↗