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1/5. A contemporary case of encephalitis lethargica.

    We present a clinicopathological report of a recent fatal case of a 27-year-old woman whom we consider to have had encephalitis lethargica. Clinical features of note were a presentation with vertigo, persistent vomiting and sleep disturbance including marked daytime somnolence and vivid nightmares. On examination, she had impaired slow pursuit vertical eye movements, dysarthria, an expressionless face and slow tongue movements. She went on to develop gross supranuclear gaze palsy, neck rigidity, bradykinesia, blepharospasm, profound somnolence and anarthria but no tremor, weakness or impairment of cognition. She died after an illness lasting 12 months. On investigation, the cerebrospinal fluid was found to contain a very high level of IgG with oligoclonal bands but no cells. Post-mortem examination revealed an active encephalitis, mainly centered on the upper brainstem and diencephalon with extensive Purkinje cell loss and marked plasma cell infiltrates and morula cells. No virus was recovered.
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ranking = 1
keywords = supranuclear, palsy
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2/5. Progressive supranuclear palsy: new disease or variant of postencephalitic parkinsonism?

    We review the etiological importance of the epidemic encephalitis for progressive supranuclear palsy (PSP) and addresses the question of whether the explosion of PSP literature in the mid-20th century reflects the appearance of a new disease. We examined 2,000 studies on Parkinson's disease from 1861 to 1963 and found PSP-like cases in the past, before the epidemic encephalitis era. It can be assumed that PSP is neither a new disease nor a variant of postencephalitic parkinsonism.
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ranking = 9.6254944592563
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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3/5. Frequency of Alzheimer's neurofibrillary tangle in the brains of progressive supranuclear palsy, postencephalitic parkinsonism, Alzheimer's disease, senile dementia and non-demented elderly person.

    The frequency and the distribution of Alzheimer's neurofibrillary tangles were examined in those cases of progressive supranuclear palsy (one case), postencephalitic parkinsonism (one case), Alzheimer's disease (two cases), senile dementia (two cases) and non-demented elderly person (three cases). The light microscopic pattern of Alzheimer's neurofibrillary tangles seen in progressive supranuclear palsy was similar to those in postencephalitic parkinsonism, Alzheimer's disease, senile dementia and non-demented elderly person. Some differences in the frequency and the distribution of Alzheimer's neurofibrillary tangles were demonstrated.
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ranking = 24.35946186784
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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4/5. Pathological overlap in cases of parkinsonism associated with neurofibrillary tangles. A study of recent cases of postencephalitic parkinsonism and comparison with progressive supranuclear palsy and Guamanian parkinsonism-dementia complex.

    In recent years a number of patients suffering from long-standing postencephalitic parkinsonism have donated their brains to the United Kingdom Parkinson's disease Society brain Bank, in london. In view of the paucity of detailed neuropathological reports of the disease since the 1940s, we have carried out a clinicopathological study of eight recent cases. A spectrum of pathological change was seen, with highly variable involvement of cortical, subcortical and brainstem structures. There was no correlation between severity of disease and severity of pathology. The anatomical distribution of lesions was compared with that seen in progressive supranuclear palsy and Guamanian Parkinson-dementia complex. No definite histological features were identified that could distinguish any of the three disease entities. While the pathogenesis of postencephalitic parkinsonism, progressive supranuclear palsy and Guamanian Parkinson-dementia complex remains unknown, the presence of similar pathologies in these conditions suggests a common disease mechanism, despite the distinctive clinical features.
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ranking = 24.35946186784
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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5/5. Supranuclear gaze palsy and eyelid apraxia in postencephalitic parkinsonism.

    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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ranking = 5.0605361465276
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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