Ocular Manifestations in Progressive Supranuclear Palsy

Pfaffenbach, David D.; Layton, Donald D.; Kearns, Thomas P.

doi:10.1016/0002-9394(72)90740-4

Cited by 58 publications

(31 citation statements)

References 8 publications

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“…Signal abnormalities are mild and consist of hyperintensity in the periaqueductal region on T 2 -weighted sequences ("Morning Glory sign") 18,19 . The most widely reported dystonic manifestation on PSP is blepharospasm 20 , but none of our patients presented such manifestation. Two of our patients (patients 2 and 4) presented limb dystonia.…”

Section: Discussioncontrasting

confidence: 48%

Clinical features of dystonia in atypical parkinsonism

Godeiro-Júnior

Felı́cio

Barsottini

et al. 2008

Arq. Neuro-Psiquiatr.

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-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.KeY worDS: dystonia, atypical parkinsonism, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, Parkinson's disease. Características clínicas da distonia no parkinsonismo atípicoResumo -Introdução: A associação de distonia e doença de Parkinson (DP) já foi bem estabelecida, principalmente para distonia focal em pé ou mão. entretanto, há poucos dados quanto a distonia em pacientes com parkinsonismo atípico. Objetivo: Avaliar a freqüência e o padrão da distonia em um grupo de pacientes com parkisnonismo atípico (atrofia de múltiplos sistemas -AMS; paralisia supranuclear progressiva -PSP; degeneração corticobasal -DCB) e investigar se a distonia pode ser a manifestação inicial neste grupo. Método: Um total de 38 prontuários médicos foi revisado (n=23/grupo AMS; n=8/grupo PSP; n=7/grupo PSP) e os dados foram apresentados em médias/desvios padrões. As variaveis avaliadas foram: sexo, idade de início, duração da doença, primeiro sintoma, características clínicas da distonia e outros sinais neurológicos, resposta ao tratamento com levodopa, escala de Hoehn & Yahr >3 em 3 anos de doença, e achados de ressonância magnética. Resultados: A frequência total de distonia em nosso grupo foi 50%, sendo 30,4% (n=7) no grupo AMS, 62.5% (n=5) no grupo PSP e 100% (n=8) no grupo DCB. em nenhum dos pacientes, distonia foi o primeiro sintoma...

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Section: Discussioncontrasting

confidence: 48%

Clinical features of dystonia in atypical parkinsonism

Godeiro-Júnior

Felı́cio

Barsottini

et al. 2008

Arq. Neuro-Psiquiatr.

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“…A lower cut off would be appropriate under different observation conditions or where higher specificity was required. It must be noted that there is considerable overlap in the range of blink rates for patients Previous studies have indicated that blink rate is reduced in other extra-pyramidal disorders [25] but not in other types of movement disorder [8]. Consequently blink rate is of little value in distinguishing between the various extrapyramidal disorders.…”

Section: Discussionmentioning

confidence: 97%

Case–control study of blink rate in Parkinson’s disease under different conditions

et al. 2011

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Standard neurology texts list a reduced blink rate as one of the clinical features of Parkinson's disease. However, there are few clinical studies which have quantified this clinical sign. Here we present the results of a quantified study in a cohort of cases and controls using a standard protocol. Cases meeting standard criteria for a diagnosis of Parkinson's disease were studied together with age- and sex-matched controls. Baseline data included age, sex, duration of disease, Hoehn and Yahr stage, mini-mental state examination and treatment. Subjects were videoed undertaking three different tasks: being interviewed, watching a video, and reading from a book. Blink rates were calculated as a mean 'per minute' figure for each of the three tasks. A meta-analysis of previous studies of blink rate was undertaken. A total of 20 cases and 41 controls were studied. A decline in blink rate with increasing age was seen for cases but not controls. A significant reduction in blink rate was seen in cases when compared with controls for each of the test conditions. Blink rates were highest in subjects when being interviewed and were lowest whilst reading a passage in both cases and controls. No effect of disease duration, severity or treatment was observed. We have quantified the reduction in blink rate which has long been recognised as a feature of Parkinson's disease. We have identified factors which determine blink rate within individuals. We have also been able to define normal and abnormal levels for blink rate which may be of value clinically and for future research.

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“…Although clinical findings were based on neurological bedside testing and detailed studies of eye movements were not undertaken, the pattern of gaze palsy was similar to that observed in PSP, with severely affected vertical saccades and smooth pursuit and initially preserved horizontal gaze (Dix et al, 1971;Pfaffenbach et al, 1972;Cogan, 1974;Troost and Daroff, 1977;Chu et al, 1979;PierrotDeseilligny et al, 1989). As PSP progresses, horizontal supranuclear ophthalmoplegia develops, followed by total ophthalmoplegia with loss of vestibuloocular reflexes.…”

Section: Clinical Findingsmentioning

confidence: 95%

Supranuclear gaze palsy and eyelid apraxia in postencephalitic parkinsonism

Wenning

Jellinger

Litvan

1997

J. Neural Transmission

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We describe six patients with clinicopathologically confirmed post-encephalitic parkinsonism (PEP) in whom oculomotor abnormalities developed several years after suffering the initial episode of encephalitis lethargica. Four of the cases had vertical supranuclear gaze palsy and two eyelid apraxia, features typically associated with progressive supranuclear palsy (PSP). Our findings indicate that the presence of gaze palsy alone may not be a reliable clinical discriminator between PEP and PSP. Involvement of the dorsal central gray nucleus, nucleus centralis pontis oralis, nucleus dorsal raphe interpositus, rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), nucleus interstitialis of Cajal, nucleus of the posterior commissure, pedunculopontine nuclei and frontal cortex was observed in several of our PEP cases and may contribute to the oculomotor abnormalities in this disorder. Whether the dorsal tegmental nucleus, caudal to the supratrochlear nucleus, severely affected in all our PEP cases, has a role in vertical gaze needs to be further studied.

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Ocular Manifestations in Progressive Supranuclear Palsy

Cited by 58 publications

References 8 publications

Clinical features of dystonia in atypical parkinsonism

Clinical features of dystonia in atypical parkinsonism

Case–control study of blink rate in Parkinson’s disease under different conditions

Supranuclear gaze palsy and eyelid apraxia in postencephalitic parkinsonism

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