Navegando por Palavras-chave "parkinsonismo atípico"
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- ItemSomente MetadadadosAvaliação dos aspectos cognitivo, autonômico e do sono em pacientes com atrofia de múltiplos sistemas(Universidade Federal de São Paulo (UNIFESP), 2016-11-29) Barcelos, Lorena Broseghini [UNIFESP]; Ferraz, Henrique Ballalai Ferraz [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Objective: To evaluate the cognitive impairment, autonomic and sleep disturbances in subtype MSA-P versus MSA-C. Introduction: Multiple system atrophy (MSA) is a progressive neurodegenerative disorder of unknown etiology that occurs sporadically, characterized by autonomic failure, parkinsonian, cerebellar and pyramidal features in various combinations. Based on the main symptoms, MSA is divided into MSA-P (parkinsonism subtype) and MSA-C (cerebellar subtype). Disease onset is usually in the sixth decade of life, with both sexes equally affected. MSA is a synucleinopathy characterized by a relentless worsening of motor and nonmotor symptoms. Sleep disturbances are nonmotor features often associated with MSA. Cognitive impairment is an exclusion criterion for the diagnosis of MSA, according to the second consensus statement; however, frontal-lobe and cerebellar dysfunction with executive deficits have been reported. Methods: This cross-sectional study consisted 14 patients diagnosed with MSA, 4 patients with MSA-C, 10 patients with MSA-P. The presence of motor symptoms was determined by applying the Unified MSA Rating Scale (UMSARS). Nonmotor aspects were evaluated by applying the Short Form (36) Health Survey (SF-36), Scales for Outcomes in Parkinson?s disease e Autonomic (SCOPA-AUT), Restless Legs Syndrome Rating Scale (RLSRS), Hospital Anxiety and Depression Scale (HADS), Beck Depression Inventory (BDI) Parkinson?s disease Sleep Scale (PDSS), Pittsburgh Sleep Quality Index (PSQI) and Epworth Sleepiness Scale (ESS). The neuropsychological tests examined general cognition, verbal and visual memory, working memory, visuospatial and constructional ability, language, and executive function. Results: The study enrolled 14 patients with MSA, 10 patients showed a predominance of parkinsonian signs (MSA-P), and 4 showed a predominance of cerebellar dysfunction (MSA-C). The mean age was 63.5 ± 2.4 years, and the mean disease duration was 4.4 ± 2.4 years. The education level of the sample was 9.8 ± 6.3 years. The mean Mini Mental State Examination (MMSE) was 25 ± 3.5 points and Mattis Dementia Rating Scale (MDRS) was 125.3 ± 14.5. Comparing MSA subtypes the quality of life questionnaire SF-36 bodily pain in the section showed worse results in MSA-P (p<0.05), and neuropsychological testing in patients with MSA-C did not differ significantly from MSA-P, except the MDRS in the field of attention, which showed worse performance in MSA-C (p=0.029). The comparison of patients for the presence or absence of Restless Legs Syndrome (RLS) showed a statistically significant difference in the immediate memory performed better in the group with RLS (p=0.046), and the belated recognition was better in the group with RLS (p=0.032). An inverse correlation was found between PDSS total score and autonomic, anxiety and depression aspects. Conclusion: Comparative study of cognitive impairment in MSA-P and MSA-C showed that patients with MSA have cognitive impairment in executive and visuospatial functions, while the attentional involvement was predominant only in MSA-C. These findings support the concept that the cognitive deficit originates from striatofrontal dysfunction and also degeneration cerebellar. SPI is a prevalent condition in MSA and its presence is not related to a poorer performance in cognitive assessment. Autonomic scale showed greater severity correlated with poorer sleep quality assessed by PDSS. Our study suggests that cognitive and sleep involvement are relevant in MSA and clinical neurologist should not neglect evaluation of these aspects in their daily clinical practice.
- ItemAcesso aberto (Open Access)Clinical features of dystonia in atypical parkinsonism(Academia Brasileira de Neurologia - ABNEURO, 2008-12-01) Godeiro-Junior, Clecio [UNIFESP]; Felício, André Carvalho [UNIFESP]; Barsottini, Orlando Graziani Povoas [UNIFESP]; Aguiar, Patricia M. de Carvalho [UNIFESP]; Silva, Sonia M.a. [UNIFESP]; Borges, Vanderci [UNIFESP]; Ferraz, Henrique B. [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)BACKGROUND: The association between Dystonia and Parkinson's disease (PD) has been well described especially for foot and hand dystonia. There is however few data on dystonic postures in patients with atypical parkinsonism. OBJECTIVE: To evaluate the frequency and pattern of dystonia in a group of patients with atypical parkinsonism (multiple system atrophy - MSA, progressive supranuclear palsy - PSP, and corticobasal degeneration - CBD) and to investigate whether dystonia could be the first presenting symptom at disease onset in those patients. METHOD: A total of 38 medical charts were reviewed (n=23/MSA group; n=7/CBD group; n=8/PSP group) and data values were described as means/standard deviations. The variables evaluated were sex, age at onset, disease duration, first symptom, clinical features of dystonia and other neurological signs, response to levodopatherapy, Hoehn&Yahr scale >3 after three years of disease, and magnetic resonance imaging findings. RESULTS: The overall frequency of dystonia in our sample was 50% with 30.4% (n=7) in the MSA group, 62.5% (n=5) in the PSP group, and 100% (n=8) in the CBD group. In none of these patients, dystonia was the first complaint. Several types of dystonia were found: camptocormia, retrocollis, anterocollis, blepharoespasm, oromandibular, and foot/hand dystonia. CONCLUSION: In our series, dystonia was a common feature in atypical parkinsonism (overall frequency of 50%) and it was part of the natural history although not the first symptom at disease onset. Neuroimaging abnormalities are not necessarily related to focal dystonia, and levodopa therapy did not influence the pattern of dystonia in our group of patients.
- ItemAcesso aberto (Open Access)Progressive supranuclear palsy: new concepts(Academia Brasileira de Neurologia - ABNEURO, 2010-12-01) Barsottini, Orlando Graziani Povoas [UNIFESP]; Felício, André Carvalho [UNIFESP]; Aquino, Camila Catherine Henriques de [UNIFESP]; Pedroso, José Luiz [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Progressive supranuclear palsy (PSP) is a distinctive form of neurodegenerative disease which affects the brainstem and basal ganglia. Patients present supranuclear ophthalmoplegia, postural instability and mild dementia. PSP is defined neuropathologically by the accumulation of neurofibrillary tangles in the subthalamic nucleus, pallidum, red nucleus, substantia nigra, striatum, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. Over the last decade many lines of investigations have helped refine PSP in many aspects and it is the purpose of this review to help neurologists identify PSP, to better understand its pathophysiology and to provide a more focused, symptom-based treatment approach.