Progressive supranuclear palsy: new concepts

Barsottini, Orlando Graziani Póvoas; Felı́cio, André Carvalho; Aquino, Camila C.; Pedroso, José Luiz

doi:10.1590/s0004-282x2010000600020

Cited by 33 publications

(34 citation statements)

References 52 publications

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“…Invasive surgical procedures were not immediately performed because of his general clinical condition and comorbidities, which deserved prioritary medical and nutritional management, as well as functional rehabilitation (physiotherapy, hand therapy, and occupational therapy). The patient also had PSP, starting after the age of 40, and the features were strongly suggestive of Richardson syndrome [6] but the coexistece of DC with this syndrome seems to be a casual phenomenon. Moreover, his neurological symptoms, including falls, could be mistaken as an unsuspected epilepsy, which is a well-known predisposing factor for DC [1,7].…”

Section: Answer To Photo Quiz Dupuytren's Contracturementioning

confidence: 95%

“…Other disorders related to DC include myocardial disease and reflex sympathetic dystrophy [1], hepatic disease, arthritis, fibromatosis, use of antiepileptic drugs, and compulsive personality. Differential diagnoses involve other conditions associated with diabetes, as multiple "trigger fingers" and "ulnar tunnel" syndrome, but these entities were ruled out based on clinical features [1,6,7].…”

Section: Answer To Photo Quiz Dupuytren's Contracturementioning

confidence: 99%

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A 61-year-old Diabetic And Alcoholist Male With Bilateral

Santos¹,

Ferrer²,

Sales³

et al. 2013

MMJ

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A 61-year-old white right-handed man was admitted because of recent loss of weight and difficulty in swallowing solid foods. There was gradually progressive apathy, postural instability with falls backwards and cognitive deficit. His past history included controlled diabetes mellitus and arterial hypertension, tobacco smoking and heavy alcoholism. During the last two years, an unexplained bilateral contraction developed in some of his fingers. He was not a manual worker nor a guitar or piano player, and denied previous hand trauma or similar disturbance in members of his family. Physical examination confirmed postural instability and revealed vertical gaze palsy, and the suspicion of progressive supranuclear palsy (PSP) was confirmed, based on clinical data and the study of brain images. Moreover, there was bilateral contraction of the middle, ring and little fingers, and thickened linear structures were palpated over the third, fourth and fifth flexor tendons on both palms. Furthermore, the patient was not able to lay his palms flat on a tabletop; and conspicuous hypotrophy of the lumbrical and interosseus muscles was observed (Figure 1). The evaluation of the shoulders, plantar and genital regions showed no abnormality. Laboratory tests were unremarkable, and specific studies were made to clear the hand changes. The patient was treated with conservative clinical, nutritional, and physiotherapy procedures, before being referred for specialized treatment and outpatient surveillance.What is your diagnosis?

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Section: Answer To Photo Quiz Dupuytren's Contracturementioning

confidence: 95%

Section: Answer To Photo Quiz Dupuytren's Contracturementioning

confidence: 99%

A 61-year-old Diabetic And Alcoholist Male With Bilateral

Santos¹,

Ferrer²,

Sales³

et al. 2013

MMJ

View full text Add to dashboard Cite

show abstract

“…The prevalence of PSP is agedependent and estimated at 6 to 10% of that of PD, or 6 to 7 cases per 100,000. 9 Nearly half of all patients are markedly disabled or wheelchair bound within 4 years of onset. Early onset, the presence of falls, slowness, and inability to move the eyes downward early in the development of the disease predict poor survival time.…”

Section: Progressive Supranuclear Palsy-parkinsonism (32%)mentioning

confidence: 99%

A Case of Progressive Supranuclear Palsy/Steele—Richardson—Olszewski Syndrome

Shende

Choudhari

Kelkar

et al. 2017

MGM Journal of Medical Sciences

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Progressive supranuclear palsy (PSP) is an uncommon neurological disorder, the hallmark of which is supranuclear ophthalmoplegia involving vertical gaze. Other clinical features include pseudobulbar palsy (dysphagia and dysarthria), neck dystonia (retrocollis), bradykinesia, postural instability, and repeated falls occurring early in the course of the disease, personality changes, a staring unblinking facies, mild dementia, and cerebellar and corticospinal tract signs. Magnetic resonance imaging (MRI) of the brain on midsagittal images may reveal a characteristic atrophy of the midbrain in a shape that suggests a bird, particularly a humming bird. The PSP may resemble Parkinson's disease (PD), but the pathophysiology is distinct from PD. Here, we report a rare case of a 72-year-old man who came with difficulty in naming objects and persons, dysphagia, dysarthria, difficulty in vertical gaze, and history of recurrent fall even while in sitting down position. He was diagnosed as PSP based on clinical examination and neuroimaging. The PSP has poor prognosis.

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“…O desenvolvimento da doença geralmente se dá na terceira/quarta década de vida e não há relatos na literatura da doença em pessoas abaixo dos 40 anos, apresentando sintomas progressivos de complicações como perda de equilíbrio, levando "[...] a quedas, disfagia, problemas nutricionais, pneumonia aspiratória, redução na capacidade de piscar, paralisia vertical, salivação excessiva e apraxia da abertura palpebral", dentre outras, levando aos quadros de diminuição das funções perceptivo-motoras (Ribeiro, et al, 2012;Barsottini, Felício, Aquino, & Pedroso, 2010).…”

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“…Normalmente, o diagnóstico correto é confirmado entre 3-6 e 4-9 anos após apresentarem-se os sintomas iniciais clínicos, tornando-se uma agravante o retardo para o tratamento, apesar de este ainda ser bastante limitado, muitas vezes por falta de conhecimento dos especialistas envolvidos (Barsottini, Felício, Aquino, & Pedroso, 2010). dificuldades de diagnóstico e a atuação da equipe multidisciplinar quanto aos cuidados à pessoa afetada por esta doença.…”

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Síndrome da Paralisia Supranuclear Progressiva: As dificuldades de diagnóstico e a atuação da Equipe multidisciplinar quanto aos cuidados à pessoa afetada por esta doença

2017

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A Síndrome da Paralisia Supranuclear Progressiva (PSP) é doença neurodegenerativa do Sistema Nervoso Central (SNC), rara, e de difícil diagnóstico, afetando principalmente o tronco cerebral e os núcleos da base. O quadro clínico se caracteriza por oftalmoparesia supranuclear, instabilidade postural e demência. O objetivo do estudo foi investigar a fisiopatologia, diagnóstico, tratamento e assistência da equipe multidisciplinar às pessoas com PSP. Revisão integrativa de 15 artigos publicados na base de dados da Biblioteca Virtual da Saúde, BVS, envolvendo estudos de casos e pesquisa de campo. O estudo revelou pouca publicação acerca da doença e, por ser rara, não existe fármaco eficiente e eficaz; o diagnóstico é limitado nas primeiras manifestações, e somente possível por meio de exames mecanicistas. Em razão de existir parco material sobre a assistência a estes casos, sugere-se que os Conselhos, Associações de Neurologia e demais especialidades envolvidas no tratamento desenvolvam, divulguem mais detalhes sobre a doença, a fim de se criar um protocolo de atendimento integral aos afetados pela síndrome, bem como o necessário apoio aos familiares e cuidadores, que auxilie nas práticas da assistência ambulatorial e familiar.

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Progressive supranuclear palsy: new concepts

Cited by 33 publications

References 52 publications

A 61-year-old Diabetic And Alcoholist Male With Bilateral

A 61-year-old Diabetic And Alcoholist Male With Bilateral

A Case of Progressive Supranuclear Palsy/Steele—Richardson—Olszewski Syndrome

Síndrome da Paralisia Supranuclear Progressiva: As dificuldades de diagnóstico e a atuação da Equipe multidisciplinar quanto aos cuidados à pessoa afetada por esta doença

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