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Biomedical subjects

A Schrag

Publications and source records attributed to A Schrag.

At least 19 recordsLinked to original sources
PubMed2007

Progression of dysautonomia in multiple system atrophy: a prospective study of self-perceived impairment.

To assess severity and progression of self-perceived dysautonomia and their impact on health-related quality of life (Hr-QoL) in multiple system atrophy (MSA), twenty-seven patients were recruited by the European MSA Study Group (EMSA-SG). At baseline, all patients completed the Composite Autonomic Symptom Scale (COMPASS) and the 36 item Short Form Health Survey (SF-36), and they were assessed using the 3-point global disease severity scale (SS-3) and the Unified MSA Rating Scale (UMSARS). After 6 months follow-up, the self completed COMPASS Change Scale (CCS), the SF-36, SS-3, and UMSARS were obtained. MSA patients showed marked self-perceived dysautonomia at baseline visit and pronounced worsening of dysautonomia severity on the CCS at follow-up. Severity and progression of dysautonomia did not correlate with age, disease duration, motor impairment and overall disease severity at baseline. There were no significant differences between genders and motor subtypes. Baseline COMPASS scores were, however, inversely correlated with SF-36 scores. Progression of self-perceived dysautonomia did not correlate with global disease progression. Hr-QoL scores were stable during follow-up. This is the first study to investigate self-perceived dysautonomia severity in MSA and its evolution over time. Our data suggest that dysautonomia should be recognized as a key target for therapeutic intervention in MSA.

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PubMed2006

Testing the validity of the PDQ-39 in patients with MSA.

We assessed the validity of the PDQ-39, a disease-specific health-related quality of life instrument for patients with Parkinson's disease, in patients with multiple system atrophy (MSA). Two hundred and seventy-nine patients completed the PDQ-39, the EQ-5D, the Hospital Anxiety and Depression Scale, and scales of life satisfaction and disease severity. Ceiling and floor effects were noted in some dimensions, and Mobility was skewed towards the severe end of the spectrum. Apart from the dimension of Social Support, all dimensions had high internal consistency. The factor structure of the PDQ-39 in MSA was stable, and convergent and divergent validity with other measures of quality of life and mental health were good. However, many of the specific features of MSA are not reflected in the PDQ-39. Higher order factor analysis did not support the use of a single summary index. We conclude that the PDQ-39 has only limited validity in patients with MSA.

Activities of Daily Living↗
PubMed2006

Measuring quality of life in PSP: the PSP-QoL.

OBJECTIVE: To develop a new patient-reported outcome measure for progressive supranuclear palsy (PSP) and to test its psychometric properties. METHODS: First, the authors generated a pool of potential scale items from in-depth patient interviews. Second, the authors administered these items, in the form of a questionnaire, to a sample of people with PSP and traditional psychometric methods were used to develop a rating scale satisfying standard criteria for reliability and validity. Third, the authors examined the psychometric properties of the rating scale in a second sample. RESULTS: In stage 1, a pool of 87 items was generated from 27 patient interviews. In stage 2, a scale with two subscales (physical, 22 items; mental, 23 items), satisfying standard criteria for reliability and validity, was developed from the response data of 225 patients with PSP. In stage 3, the scale was examined in 188 people with PSP. Missing data were low, scores in both subscales were evenly distributed, floor and ceiling effects were small. Reliability was high (Cronbach's alpha 0.93, 0.95; test-retest 0.95, 0.92). Validity was supported by the interscale intercorrelation (0.60), factor analysis, and the magnitude and pattern of correlations with four other rating scales, disease severity, and disease duration. The psychometric properties of the new scale were similar in the United Kingdom and North America, and in clinic- and community-based samples studied. CONCLUSIONS: The Progressive Supranuclear Palsy Quality of Life scale (PSP-QoL) may be a helpful patient-reported scale for clinical trials and studies in PSP.

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PubMed2005

The European Multiple System Atrophy-Study Group (EMSA-SG).

Introduction. The European Multiple System Atrophy-Study Group (EMSA-SG) is an academic network comprising 23 centers across Europe and Israel that has constituted itself already in January 1999. This international forum of established experts under the guidance of the University Hospital of Innsbruck as coordinating center is supported by the 5th framework program of the European Union since March 2001 (QLK6-CT-2000-00661). Objectives. Primary goals of the network include (1) a central Registry for European multiple system atrophy (MSA) patients, (2) a decentralized DNA Bank, (3) the development and validation of the novel Unified MSA Rating Scale (UMSARS), (4) the conduction of a Natural History Study (NHS), and (5) the planning or implementation of interventional therapeutic trials. Methods. The EMSA-SG Registry is a computerized data bank localized at the coordinating centre in Innsbruck collecting diagnostic and therapeutic data of MSA patients. Blood samples of patients and controls are recruited into the DNA Bank. The UMSARS is a novel specific rating instrument that has been developed and validated by the EMSA-SG. The NHS comprises assessments of basic anthropometric data as well as a range of scales including the UMSARS, Unified Parkinson's Disease Rating Scale (UPDRS), measures of global disability, Red Flag list, MMSE (Mini Mental State Examination), quality of live measures, i.e. EuroQoL 5D (EQ-5D) and Medical Outcome Study Short Form (SF-36) as well as the Beck Depression Inventory (BDI). In a subgroup of patients dysautonomic features are recorded in detail using the Queen Square Cardiovascular Autonomic Function Test Battery, the Composite Autonomic Symptom Scale (COMPASS) and measurements of residual urinary volume. Most of these measures are repeated at 6-monthly follow up visits for a total study period of 24 months. Surrogate markers of the disease progression are identified by the EMSA-SG using magnetic resonance and diffusion weighted imaging (MRI and DWI, respectively). Results. 412 patients have been recruited into the Registry so far. Probable MSA-P was the most common diagnosis (49% of cases). 507 patients donated DNA for research. 131 patients have been recruited into the NHS. There was a rapid deterioration of the motor disorder (in particular akinesia) by 26.1% of the UMSARS II, and - to a lesser degree - of activities of daily living by 16.8% of the UMSARS I in relation to the respective baseline scores. Motor progression was associated with low motor or global disability as well as low akinesia or cerebellar subscores at baseline. Mental function did not deteriorate during this short follow up period. Conclusion. For the first time, prospective data concerning disease progression are available. Such data about the natural history and prognosis of MSA as well as surrogate markers of disease process allow planning and implementation of multi-centre phase II/III neuroprotective intervention trials within the next years more effectively. Indeed, a trial on growth hormone in MSA has just been completed, and another on minocycline will be completed by the end of this year.

Animals↗
PubMed2005

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

The development of psychometrically sound health-related quality of life (Hr-QoL) instruments has made it possible to assess subjectively experienced Hr-QoL quantitatively, and to incorporate Hr-QoL as a measure in medical research and clinical trials. Hr-QoL in patients with progressive supranuclear palsy (PSP) has been measured using generic (not disease-specific) instruments, or Parkinson's disease-specific scales. We give an overview of the development of a disease-specific Hr-QoL instrument for patients with PSP. Based on interviews with 27 patients with PSP a preliminary Hr-QoL instrument was developed and administered to over 300 people with PSP in the UK and US. Following psychometric analysis a rating scale with a physical and a mental subscale emerged. In this patient sample, both subscales satisfied criteria for scaling assumptions, acceptability, reliability and validity (correlations with other measures consistent with a priori hypotheses). The psychometric properties of this questionnaire are undergoing further evaluation.

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PubMed2004

Anti-basal ganglia antibodies in patients with atypical dystonia and tics: a prospective study.

Anti-basal ganglia antibodies (ABGA) are associated with movement disorders in children, but have not been assessed in adult onset movement disorders. In a prospective assessment ABGA were positive in 65% of a group of 65 patients with atypical movement disorders, but were very rare in healthy adults and adults with idiopathic dystonia. An autoimmune mechanism may underlie a proportion of cases of atypical movement disorders.

Adolescent↗
PubMed2004

Patterns of EMG-EMG coherence in limb dystonia.

Dystonia of the limbs may be due to a wide range of aetiologies and may cause major functional limitation. We investigated whether the previously described pathological 4 to 7 Hz drive to muscles in cervical dystonia is present in patients with aetiologically different types of dystonia of the upper and lower limbs. To this end, we studied 12 symptomatic and 4 asymptomatic carriers of the DYT1 gene, 6 patients with symptomatic dystonia due to focal basal ganglia lesions, and 11 patients with fixed dystonia, a condition assumed to be mostly psychogenic in aetiology. We evaluated EMG-EMG coherence in the tibialis anterior (TA) of these and 15 healthy control subjects. Ten of 12 (83%) of symptomatic DYT1 patients had an excessive 4 to 7 Hz common drive to TA, evident as an inflated coherence in this band. This drive also involved the gastrocnemius, leading to co-contracting electromyographic bursts. In contrast, asymptomatic DYT1 carriers, patients with symptomatic dystonia, patients with fixed dystonia, and healthy subjects showed no evidence of such a drive or any other distinguishing electrophysiological feature. Moreover, the pathological 4 to 7 Hz drive in symptomatic DYT1 patients was much less common in the upper limb, where it was only present in 2 of 6 (33%) patients with clinical involvement of the arms. We conclude that the nature of the abnormal drive to dystonic muscles may vary according to the muscles under consideration and, particularly, with aetiology.

Adult↗
PubMed2004

Reliability of self-reported diagnoses in patients with neurologically unexplained symptoms.

BACKGROUND: Patients with neurologically unexplained symptoms (NUS) often have a previous history of other medically unexplained symptoms. A past history of such symptoms can help make a positive diagnosis of a somatoform or affective disorder, and enable appropriate management strategies. However, information on past medical diagnoses is primarily obtained from patient interviews and may be inaccurate, particularly in patients with NUS. OBJECTIVE: To assess the reliability of past medical diagnoses reported by patients with NUS compared with patients with confirmed neurological disease (ND) without suspicion of somatoform illness. METHODS: 21 patients with NUS and 16 patients with ND were interviewed about their current and past medical problems and diagnoses. The accuracy of the reported diagnoses was assessed through examination of their complete general practice notes. RESULTS: The median number of previous diagnoses reported by patients with NUS was significantly higher than in controls (7 v 3, p = 0.001). There was no difference in the median number of confirmed diagnoses between the two groups (2 v 2.5); however, the median percentage of reported diagnoses confirmed by investigations was significantly smaller in the NUS group (22% v 80%, p = 0.001). The additional diagnoses reported by patients with NUS not only comprised functional syndromes such as irritable bowel syndrome or non-cardiac chest pain (6% v 0%, p = 0.01), but also organic diagnoses which had either been unequivocally excluded (5% v 0%, p = 0.006), were based on equivocal findings often found after multiple investigations (9% v 0%, p = 0.01), or had not been investigated before a clinical diagnosis was made (50% v 18%, p = 0.04). CONCLUSION: Reported previous diagnoses should not be taken at face value when the current differential diagnosis includes a functional/somatoform neurological syndrome, particularly if the list of past medical diagnoses is long. Confirmation of previous diagnoses from alternative sources may contribute to a diagnosis of somatoform disorder, allowing appropriate management strategies for the current (and past) complaints to be initiated.

Adult↗
PubMed2003

Quality of life in patients with orthostatic tremor.

We assessed health-related quality of life (QoL) and depression, using the SF-36 and the Beck Depression Inventory (BDI), in 20 orthostatic tremor (OT) patients. All dimensions of the SF-36 were markedly reduced in OT and depression was found in 11 patients. The BDI score correlated significantly with several SF-36 subscores. We conclude that OT strongly impacts on QoL. The results highlight the importance of recognizing and treating depression in patients with OT.

Aged↗
PubMed2002

Novel three-stage ascertainment method: prevalence of PD and parkinsonism in South Tyrol, Italy.

BACKGROUND: A number of community-based studies on the prevalence of PD have been conducted worldwide, but they are often extremely costly and time consuming. OBJECTIVE: To assess the prevalence of PD and parkinsonism for the population aged between 60 and 85 years in South Tyrol, Northern Italy, using a novel population-based three-stage ascertainment method. METHODS: Seven hundred fifty persons aged 60 to 85 years from South Tyrol received a validated screening mail questionnaire for parkinsonism. In the second stage of the ascertainment method, trained primary care physicians (PCP) identified all persons with possible parkinsonism among those screened positive. In the third stage, movement disorders specialists excluded or confirmed the diagnosis in all identified people. RESULTS: The response rate was 87.6%. The prevalence rate per 100 population over 65 years of age was 1.5 (95% CI 0.6 to 2.3) for PD and 2.2 (95% CI 1.2 to 3.3) for parkinsonism after having been adjusted to the 1991 European standard population. Overall, 78% (95% CI 59 to 96%) of patients with parkinsonism were newly detected through the survey. CONCLUSIONS: The prevalence of PD and parkinsonism in people aged over 65 in South Tyrol was similar to that observed in door-to-door surveys in other European countries. The novel three-stage case ascertainment method employed proved a useful tool to substitute for expensive door-to-door surveys for prevalence studies of parkinsonism, detecting a high number of undiagnosed cases, particularly in geographically remote areas.

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PubMed2002

Ropinirole for the treatment of tremor in early Parkinson's disease.

The effect of Ropinirole on tremor in early Parkinson's disease (PD) was assessed. The results of three multicentre, randomized, double-blind trials comparing ropinirole monotherapy with levodopa, bromocriptine and placebo treatment were analysed retrospectively with respect to improvement of resting tremor and postural/action tremor as measured by items 20 and 21 of the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS). Improvements in resting tremor were significantly better with ropinirole than placebo. There were no significant differences between the effect of ropinirole and those of levodopa (L-dopa) or bromocriptine on resting tremor. Postural/action tremor was mild in these early therapy studies, and there were no significant differences between treatment groups. These results suggest that ropinirole monotherapy is effective in treating resting tremor in early PD. On the other hand, response of postural/action tremor to dopaminergic treatment in early PD was not significantly better than to placebo at the dosages used in these trials.

Aged↗
PubMed2002

How valid is the clinical diagnosis of Parkinson's disease in the community?

BACKGROUND: Many patients diagnosed with Parkinson's disease are later found to have an erroneous diagnosis, often only when they come to necropsy; conversely, many patients with Parkinson's disease in the community remain undiagnosed. OBJECTIVE: To assess the validity of a clinical diagnosis of parkinsonism in the general population according to strict published criteria. METHODS: As part of a population based study on the prevalence of Parkinson's disease in London, all patients were identified with a diagnosis of parkinsonism, tremor with onset over age 50 years, or who had ever received antiparkinsonian drugs. All patients who agreed to participate were diagnosed according to strict clinical diagnostic criteria, after a detailed neurological interview and examination and discussion of the findings with examination of their video recordings. Follow up information was obtained over a period of at least one year, and atypical cases were reviewed at the end of the study. RESULTS: A diagnosis of probable Parkinson's disease was confirmed in 83% of patients with this diagnosis, including three (2%) in whom atypical features were found that were insufficient to discard a diagnosis of Parkinson's disease. Two additional patients (2%) were found to have possible Parkinson's disease. However, in 15% of patients the diagnosis was unequivocally rejected. Conversely, 13 patients who had previously not been diagnosed with Parkinson's disease (19%) were found to have this disorder. CONCLUSIONS: At least 15% of patients with a diagnosis of Parkinson's disease in the population do not fulfil strict clinical criteria for the disease, and approximately 20% of patients with Parkinson's disease who have already come to medical attention have not been diagnosed as such.

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PubMed2001

Effect of ApoE and tau on age of onset of progressive supranuclear palsy and multiple system atrophy.

In addition to allelic association between genetic polymorphisms and diseases, modulation of age of onset is a well recognised effect of disease susceptibility genes. The ApoE epsilon4 allele is associated with an earlier age of onset of sporadic and familial Alzheimer's disease. It has been suggested that the tau genotype influences the age of onset of progressive supranuclear palsy (PSP), a form of atypical parkinsonism caused by tau neurofibrillary tangle deposition. We have therefore analysed the relationship between tau and ApoE genotypes and the age of onset of PSP and another form of atypical parkinsonism, multiple system atrophy (MSA). We have not found any effect of possession of the tau H1 haplotype or the ApoE epsilon4 allele on the age of onset of MSA or PSP.

Age of Onset↗
PubMed2001

Poetic talent unmasked by treatment of Parkinson's disease.

Parkinson's disease is typically characterised by loss of function and previous abilities, often accompanied by complications of treatment in later stages of the disease. We report on a patient who newly developed an artistic skill after starting treatment for Parkinson's disease. This case offers insight into the neurology of the artistic process as well as into the pathophysiology of psychiatric adverse reactions to the treatment of PD.

Antiparkinson Agents↗
PubMed2001

What contributes to depression in Parkinson's disease?

BACKGROUND: Depression is a common problem in patients with Parkinson's disease, but its mechanism is poorly understood. It is thought that neurochemical changes contribute to its occurrence, but it is unclear why some patients develop depression and others do not. Using a community-based sample of patients with Parkinson's disease, we investigated the contributions of impairment, disability and handicap to depression in Parkinson's disease. METHODS: Ninety-seven patients seen in a population-based study on the prevalence of Parkinson's disease completed the Beck Depression Inventory (BDI). Clinical and historical information on symptoms and complications of Parkinson's disease were obtained from the patients by a neurologist. In addition, clinician and patient ratings of disability on the Schwab and England scale were obtained and a quality of life questionnaire was completed. RESULTS: Moderate to severe depression (BDI > or = 18) was reported by 19.6% of the patients. Higher depression scores were associated with advancing disease severity, recent self-reported deterioration, higher akinesia scores, a mini-mental score of < 25 and occurrence of falls. Disability as rated by the neurologist accounted for 34% of the variance of depression scores. Self-reported impairment of cognitive function and the feeling of stigmatization accounted for > 50% of the variance of depression scores. CONCLUSIONS: Depression in patients with Parkinson's disease is associated with advancing disease severity, recent disease deterioration and occurrence of falls. Regression analysis suggests that depression in Parkinson's disease is more strongly influenced by the patients' perceptions of handicap than by actual disability. The treatment of depression should therefore be targeted independently of treatment of the motor symptoms of Parkinson's disease, and consider the patients' own perception of their disease.

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