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1.
Disabil Rehabil ; 44(13): 3095-3103, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-33295820

RESUMO

PURPOSE: Healthcare decision making in motor neurone disease (MND) focuses on symptom management and quality of life. Decision making may be affected by personal approach to receiving information, decision making style, and disease symptoms. This study explored decision making from the perspectives of people living with motor neurone disease (plwMND). The issues impacting engagement and involvement in healthcare decisions were investigated. METHODS: Semi-structured interviews were conducted with 19 plwMND and 15 carers. Interview data was inductively analysed to identify and describe patterns and themes. RESULTS: Data analysis identified six overarching themes: Dimensions of decision making; Window of opportunity for choice; Intrinsic influences on decision making; Extrinsic influences impacting decision making; Planning in uncertainty; and, Communication is core. Many participants did not identify a process of "decision-making" except if considering early gastrostomy placement. Information provision requires a balance between ensuring patients are informed but not overwhelmed. Communication impairment impacts involvement. Healthcare professionals' communication style influences engagement in decision making. CONCLUSION: PlwMND perceive a lack of clinical decisions to make because disease symptoms and clinical phenotypes dictate necessary interventions. PlwMND describe communication impairment as a barrier to involvement in decision making and extra support is required to ensure they maintain engagement.IMPLICATIONS FOR REHABILITATIONPeople living with MND (plwMND) perceive they have few clinical decisions to make and viewed this process as "accepting a recommendation", rather than "making a decision" although early gastrostomy placement is the exception with considerable deliberation evident.Specialist multidisciplinary clinic advice is especially helpful for plwMND without dysphagia (swallowing problems) when considering early gastrostomy placement.Communication impairment may be a barrier to involvement in healthcare decisions and extra support to remain engaged is required.Some plwMND choose not to involve others in their decisions, and patients/families with medical or scientific backgrounds are more likely to collaborate with each other outside the context of clinic appointments.


Assuntos
Transtornos de Deglutição , Doença dos Neurônios Motores , Cuidadores , Tomada de Decisões , Transtornos de Deglutição/etiologia , Atenção à Saúde , Humanos , Doença dos Neurônios Motores/complicações , Pesquisa Qualitativa , Qualidade de Vida , Incerteza
2.
J Neurol Sci ; 423: 117358, 2021 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-33653604

RESUMO

Reactivation of Human Endogenous Retrovirus K (HERV-K), subtype HML-2, has been associated with pathophysiology of amyotrophic lateral sclerosis (ALS). We aimed to assess the efficacy of antiretroviral therapy in inhibiting HML-2 in patients with ALS and a possible association between the change in HML-2 levels and clinical outcomes. We studied the effect of 24-weeks antiretroviral combination therapy with abacavir, lamivudine, and dolutegravir on HML-2 levels in 29 ALS patients. HML-2 levels decreased progressively over 24 weeks (P = 0.001) and rebounded within a week of stopping medications (P = 0.02). The majority of participants (82%), defined as "responders", experienced a decrease in HML-2 at week 24 of treatment compared to the pre-treatment levels. Differences in the evolution of some of the clinical outcomes could be seen between responders and non-responders: FVC decreased 23.69% (SE = 11.34) in non-responders and 12.71% (SE = 8.28) in responders. NPI score decreased 91.95% (SE = 6.32) in non-responders and 53.05% (SE = 10.06) in responders (P = 0.01). Thus, participants with a virological response to treatment showed a trend for slower progression of the illness. These findings further support the possible involvement of HML-2 in the clinical course of the disease.


Assuntos
Esclerose Lateral Amiotrófica , Retrovirus Endógenos , Infecções por HIV , Esclerose Lateral Amiotrófica/tratamento farmacológico , Esclerose Lateral Amiotrófica/genética , Infecções por HIV/tratamento farmacológico , Humanos
3.
Radiography (Lond) ; 23(3): 181-186, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-28687283

RESUMO

INTRODUCTION: The development of diagnostic imaging services manifests features specific to the Scottish environment, in particular development of the radiographic workforce through implementing skills mix and role developments to enhance outcomes for patients. A component of a College of Radiographers Industry Partnership Scheme (CoRIPS) supported study, this research investigates perspectives of strategic service managers with Health Board responsibility for service delivery. METHOD: A questionnaire survey was administered to strategic service managers across Scotland (N = 14), followed up with telephone interviews. There was a return rate of 57% (n = 8) for the questionnaires and n = 4 agreed to be interviewed. Data collected related to radiographer roles across their Board area; awareness and understanding of service development issues and features as well as perspective on opportunities and barriers in the context of Scottish Government policy, workforce logistics, attitudes and inter-professional relationships. RESULTS: The results indicate evidence of financial, logistical and political barriers to service evolution, offset by a sense of optimism that scope for beneficial change may be approaching. There are a range of significant initiatives in place and an appetite exists to pursue the development of radiographer roles and skill mix for the benefit of service users more generally. CONCLUSION: The difficulties in achieving change are well understood and there are basic issues related to finance and industrial relations. There are also however, cultural elements to contend with in the form of attitudes demonstrated by some radiographers and significantly, the radiological community whose influence on the practice of independently regulated radiographers seems incongruent.


Assuntos
Pessoal Técnico de Saúde/estatística & dados numéricos , Diagnóstico por Imagem , Descrição de Cargo , Humanos , Entrevistas como Assunto , Escócia , Inquéritos e Questionários
4.
Neurology ; 72(12): 1087-94, 2009 Mar 24.
Artigo em Inglês | MEDLINE | ID: mdl-19307543

RESUMO

OBJECTIVE: We sought to define the significance of brachial amyotrophic diplegia (flail arm syndrome [FA]) and the pseudopolyneuritic variant (flail leg syndrome [FL]) of amyotrophic lateral sclerosis (ALS; motor neuron disease). METHODS: We analyzed survival in clinic cohorts in London, UK (1,188 cases), and Melbourne, Australia (432 cases). Survival from disease onset was analyzed using the Kaplan- Meier method and Cox proportional hazards model. RESULTS: In the London cohort, the FA syndrome represented 11% and the FL syndrome 6% of the sample. Median survival was 35 months for limb onset and 27 months for bulbar onset ALS, whereas this was 61 months for FA syndrome (p < 0.001) and 69 months for FL syndrome (p < 0.001). Five-year survival in this cohort was 8.8% for bulbar onset, 20% for limb onset, 52% for FA syndrome, and 64% for FL syndrome. The ratio of men to women was 4:1 in the FA group compared to 2:1 in other limb onset cases. Excluding lower motor neuron FA and FL cases, progressive muscular atrophy comprised 4% of the sample and had a prognosis similar to typical limb onset ALS. In the Melbourne cohort, median survival for limb onset ALS was 31 months, bulbar onset 27 months, FA syndrome 66 months (p < 0.001), and FL syndrome 71 months (p = 0.001). CONCLUSIONS: The flail arm (FA) and flail leg (FL) syndromes had significantly better survival than typical amyotrophic lateral sclerosis (ALS) or progressive muscular atrophy cases that were not classified as FA or FL. Our findings underline the clinical and prognostic importance of the FA and FL variants of ALS.


Assuntos
Esclerose Lateral Amiotrófica/epidemiologia , Esclerose Lateral Amiotrófica/fisiopatologia , Braço/fisiopatologia , Perna (Membro)/fisiopatologia , Adolescente , Adulto , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Esclerose Lateral Amiotrófica/diagnóstico , Estudos de Coortes , Progressão da Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Atrofia Muscular Espinal/diagnóstico , Atrofia Muscular Espinal/epidemiologia , Atrofia Muscular Espinal/fisiopatologia , Prognóstico , Modelos de Riscos Proporcionais , Distribuição por Sexo , Taxa de Sobrevida , Adulto Jovem
5.
Artigo em Inglês | MEDLINE | ID: mdl-11771768

RESUMO

INTRODUCTION: The p75 neurotrophin receptor has been recognized as a death-signalling molecule under certain circumstances. Its role in motor neuron degeneration in amyotrophic lateral sclerosis (ALS) was analysed in SOD1-G93A transgenic mice and in spinal cords from human amyotrophic lateral sclerosis. METHOD: The precise loss of motor neurons in SOD1-G93A transgenic mice from birth to adulthood was established using the unbiased fractionator/optical dissector neuronal counting technique. RESULTS: This study showed an early trend in the loss of lumbar motor neurons in SOD1-G93A mice, beginning at birth and progressing to a massive 80% reduction by 4 months of age, when the disease is severe. This study also found that the p75 neurotrophin receptor was expressed in lumbar motor neurons in symptomatic SOD1-G93A mice and in motor neurons in the cervical spinal cords of patients with ALS. CONCLUSIONS: The murine and human ALS data suggest that the p75 neurotrophin receptor may play a death-signalling role in the pathogenesis of motor neuron degeneration. The precise mechanism by which this receptor drives the apoptotic process, both in murine SOD1-G93A motor neuron degeneration and in human amyotrophic lateral sclerosis, remains to be determined.


Assuntos
Esclerose Lateral Amiotrófica/metabolismo , Neurônios Motores/metabolismo , Degeneração Neural/metabolismo , Receptores de Fator de Crescimento Neural/fisiologia , Substituição de Aminoácidos/genética , Esclerose Lateral Amiotrófica/genética , Esclerose Lateral Amiotrófica/patologia , Animais , Animais Recém-Nascidos , Método Duplo-Cego , Feminino , Humanos , Masculino , Camundongos , Camundongos Transgênicos , Doença dos Neurônios Motores/genética , Doença dos Neurônios Motores/metabolismo , Doença dos Neurônios Motores/patologia , Neurônios Motores/patologia , Degeneração Neural/genética , Degeneração Neural/patologia , Receptor de Fator de Crescimento Neural , Receptores de Fator de Crescimento Neural/genética , Superóxido Dismutase/genética , Superóxido Dismutase/metabolismo
6.
J Neurol Sci ; 180(1-2): 107-13, 2000 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-11090874

RESUMO

Different clinical criteria for diagnosing dementia were compared in a sample of 69 patients with motor neurone disease (MND). Participants' performances on a computerised battery of neuropsychological tests were evaluated to assess the usefulness of these tests in predicting dementia in MND. The results indicated that when diagnostic criteria for frontotemporal (FTD) were used as part of a questionnaire method of diagnosing dementia the incidence of dementia in MND was considerably greater than traditional estimates suggest. Through a series of logistic and multiple regressions the results demonstrated that neuropsychological test performance related well to diagnostic classifications of dementia. MND patients with a clinical diagnosis of dementia were likely to demonstrate impaired new learning; poor working memory and planning; slowness in information processing and rigidity in thinking. These features, which are typical of cases of FTD, suggest that the dementia of MND is usefully characterised as a form of FTD. The finding that neuropsychological impairment correlated with behavioural features of dysexecutive impairment in daily living, indicates that the management focus in MND must be broadened to include cognitive/behavioural issues.


Assuntos
Demência/etiologia , Demência/psicologia , Doença dos Neurônios Motores/complicações , Doença dos Neurônios Motores/psicologia , Índice de Gravidade de Doença , Adulto , Idoso , Idoso de 80 Anos ou mais , Demência/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Análise de Regressão
7.
J Chromatogr Sci ; 37(3): 61-4, 1999 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-10089595

RESUMO

A simple high-performance liquid chromatography assay using fluorescence detection for the major metabolite of the gastric prokinetic drug cisapride, norcisapride, is presented. Analysis is performed using an Alltech Platinum EPS C8 column with a mobile phase made up of methanol and 0.02M sodium dihygrogen phosphate (45:55, v/v) containing triethylamine (1 g/L). Complete resolution is achieved among norcisapride, the internal standard (metoclopramide), and endogenous urinary components. The assay is linear over the range 50-2000 ng/mL with a mean recovery of 71.2% across the analytical range following solvent extraction with toluene-isoamyl alcohol (95:5, v/v). Intraday coefficients of variation (precision) determined at 200 and 1000 ng/mL are 6.0 and 9.8%, respectively, and interday coefficients of variation are 8.8 and 6.6%, respectively. Intra- and interassay accuracy (as mean relative error) determined at the same concentrations is within 10% in all cases. An analysis of urine samples from a healthy volunteer following the administration of a single 10-mg oral dose of cisapride is shown.


Assuntos
Cisaprida/análogos & derivados , Cromatografia Líquida de Alta Pressão , Cisaprida/urina , Humanos , Indicadores e Reagentes , Reprodutibilidade dos Testes
8.
J Psychol ; 133(1): 125-8, 1999 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-10022080

RESUMO

Extraversion has been suggested as a factor associated with addiction. This claim was tested in relation to exercise addiction. Twelve exercise addicts were compared with 12 nonaddicted individuals who were committed to regular exercise and with 12 nonexercising individuals drawn from the same student population. Addicted exercisers did not differ from nonaddicted exercisers in extraversion, although exercisers as a group were more extraverted than nonexercisers. The results are interpreted as evidence against the claim that extraversion is a component of the addictive personality profile.


Assuntos
Comportamento Aditivo/psicologia , Exercício Físico/psicologia , Extroversão Psicológica , Adolescente , Adulto , Feminino , Humanos , Masculino , Inventário de Personalidade , Psicometria , Estudantes/psicologia
10.
Int J Psychophysiol ; 23(1-2): 121-8, 1996.
Artigo em Inglês | MEDLINE | ID: mdl-8880372

RESUMO

Jacobs' (1986) proposition that dependency is the result of two sets of predisposing factors (one of which is either an excessively depressed or excited resting physiological state) was examined in relation to exercise dependency by investigating EEG characteristics of subjects committed to fitness regimes. EEG records of three groups of subjects who differed in terms of degree of dependency were studied under resting conditions both pre- and post-exercise. The results indicated that although there were no differences between groups in terms of relative power in each of five EEG bandwidths covering the range 0 to 30 Hz, the power distribution within the alpha (7.5-13 Hz) bandwidth was significantly different for high dependent and non-dependent groups under both the pre- and post-exercise conditions. While the distribution was skewed toward the lower alpha frequencies for the low dependent group, it was skewed toward the higher frequencies for the high dependent group with the distribution for a group with high involvement but low dependency falling between these extremes. This finding is taken as partial support for Jacobs' notion that dependency is associated with greater physiological excitation in the resting state.


Assuntos
Eletroencefalografia , Exercício Físico/fisiologia , Adulto , Ritmo alfa , Feminino , Humanos , Masculino , Aptidão Física
11.
Br J Surg ; 77(12): 1358-60, 1990 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-2276018

RESUMO

We have studied the pelvic floor musculature and its innervation in 14 of 24 (58 per cent) multiparous women who had been recruited into a study of the effect of childbirth on the pelvic floor as part of a prospective investigation that began in 1983. These 24 women had all delivered by the vaginal route without forceps assistance. Five of the 14 had developed clinical symptoms of stress incontinence 5 years later; two of them had had a further uncomplicated vaginal delivery during this time. There was manometric and neurophysiological evidence of weakness because of partial denervation of the pelvic floor striated sphincter musculature, with pudendal neuropathy, which was more marked in those women with incontinence. These findings provide direct evidence for the hypothesis that pudendal neuropathy due to vaginal delivery persists and may worsen with time.


Assuntos
Parto Obstétrico , Trabalho de Parto , Pelve/fisiopatologia , Transtornos Puerperais/etiologia , Adulto , Canal Anal/fisiopatologia , Feminino , Seguimentos , Genitália Feminina/inervação , Humanos , Neurônios Motores/fisiologia , Contração Muscular/fisiologia , Músculos/inervação , Doenças do Sistema Nervoso/etiologia , Paridade , Gravidez , Pressão , Estudos Prospectivos , Incontinência Urinária por Estresse/etiologia , Vagina
12.
J Neurol Neurosurg Psychiatry ; 53(11): 955-60, 1990 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-2178181

RESUMO

The central and peripheral motor pathways serving striated sphincter muscle function were studied using cortical and lumbar transcutaneous electrical stimulation, pudendal nerve stimulation and sphincter electromyography in 23 patients with multiple sclerosis (MS), and sphincter disturbance, including incontinence of urine or faeces, urinary voiding dysfunction, or constipation. The central motor conduction time was significantly increased in the MS group compared to controls (p less than 0.05). Damage to both the upper and lower motor neuron pathways can contribute to sphincter disturbance in MS. The latter may be due to coexisting pathology or to involvement of the conus medullaris by MS.


Assuntos
Canal Anal/inervação , Constipação Intestinal/fisiopatologia , Incontinência Fecal/fisiopatologia , Neurônios Motores/fisiologia , Tratos Piramidais/fisiopatologia , Uretra/inervação , Bexiga Urinaria Neurogênica/fisiopatologia , Adulto , Cauda Equina/fisiopatologia , Estimulação Elétrica , Eletromiografia , Feminino , Humanos , Masculino , Córtex Motor/fisiopatologia , Tempo de Reação/fisiologia , Transmissão Sináptica/fisiologia
13.
Arch Neurol ; 46(10): 1061-4, 1989 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-2803065

RESUMO

Striated anal sphincter function was studied electrophysiologically and radiologically in six patients with Parkinson's disease and chronic constipation. In five cases, there was paradoxic anal sphincter muscle contraction during simulated defecation straining resembling anismus-type pelvic outlet obstruction. Radiologic studies showed functional improvement of the defecatory mechanism following the administration of the dopamine receptor agonist apomorphine in four patients. Dysfunction of the striated anal sphincter musculature may be a significant cause of constipation in some parkinsonian patients, occurring as part of the generalized extrapyramidal motor disorder.


Assuntos
Canal Anal/fisiopatologia , Constipação Intestinal/etiologia , Doença de Parkinson/fisiopatologia , Defecação/fisiologia , Eletromiografia , Feminino , Fluoroscopia/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/complicações
14.
J Neurol Neurosurg Psychiatry ; 51(12): 1503-7, 1988 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-3221217

RESUMO

Anismus, or constipation due to functional obstruction at the pelvic outlet by paradoxical contraction of the striated sphincter muscles during defaecation straining, is described in ten constipated patients and four patients with Parkinson's disease and constipation. The dysfunctional pattern of muscle recruitment resembled that characteristic of dystonia elsewhere in the body and was indistinguishable in patients with idiopathic anismus and those with extrapyramidal motor disturbance due to Parkinson's disease. These findings suggest that anismus may be a focal dystonic phenomenon.


Assuntos
Constipação Intestinal/fisiopatologia , Distonia/fisiopatologia , Contração Muscular , Doença de Parkinson/fisiopatologia , Reto/fisiopatologia , Adulto , Idoso , Canal Anal/fisiopatologia , Eletromiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
16.
Clin Exp Neurol ; 23: 67-70, 1987.
Artigo em Inglês | MEDLINE | ID: mdl-3665181

RESUMO

Binswanger, in his 1894 dissertation on the differential diagnosis of general paresis of the insane, described a slowly progressive dementia associated with macroscopic loss of white matter. In recent years interest in Binswanger's disease was rekindled with CT demonstration of extensive white matter low densities in some patients. To define the clinical spectrum, we reviewed 22 consecutive cases in which the CT appearances suggested a diagnosis of Binswanger's disease. Two patients had focal neurological deficits at presentation, but recent anoxic or hypoglycaemic insults could not be excluded as the cause of the CT abnormalities. The 20 remaining patients were demented and showed variable combinations of corticobulbar dysfunction and gait dyspraxia. The duration of symptoms ranged from a few months to several years. Sixty per cent of this group gave a history of discrete stroke events and focal cortical and/or lacunar infarcts were a frequent CT finding. Binswanger's disease is probably due to chronic or acute-on-chronic white matter ischaemia. The association with lacunar and cortical infarctions suggests that a combination of large and small vessel disease produces diffuse ischaemia maximal in white matter watershed zones. Binswanger's disease is clinically differentiated from multi-infarct dementia by its time course.


Assuntos
Arteriosclerose Intracraniana/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Idoso , Idoso de 80 Anos ou mais , Infarto Cerebral/diagnóstico por imagem , Demência/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Risco
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