Atypical Progressive Supranuclear Palsy With Corticospinal Tract Degeneration

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Atypical progressive supranuclear palsy with corticospinal tract degeneration.

Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), sporadic multisystem tauopathy, and some forms of frontotemporal dementia with Parkinsonism linked to chromosome 17 are characterized by neuronal and glial lesions accumulating tau protein containing 4 conserved repeats in microtubule-binding domain (4R tau). Corticospinal tract degeneration is not a common feature of 4R tau...

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A case of atypical progressive supranuclear palsy

BACKGROUND Progressive supranuclear palsy (PSP) is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progressive apraxia of speech (AOS) and aphasia. AIM We aimed to investigate the clinical presentation of PSP, using a comprehensive multidimensional evaluation, and the disease response to va...

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Pure akinesia: an atypical manifestation of progressive supranuclear palsy.

Two patients with "pure akinesia" who showed the characteristic changes of progressive supranuclear palsy (PSP) at necropsy are described. They had akinesia but no rigidity or tremor, and ophthalmoplegia was not observed during the course of illness. The symptoms of "pure akinesia" was not improved by levodopa therapy but was considerably improved by L-threo-3,4-dihydroxy-phenylserine. At necro...

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Procerus sign in progressive supranuclear palsy and corticobasal degeneration.

The procerus sign was described by Romanoand Colosimo (1) in two patients with progressive supranuclear palsy (PSP), being defined as the appearance of vertical wrinkles in the glabellar region and the bridge of the nose due to dystonic contractions of the procerus muscle, associated with a combination of reduced blinking, spasticity, lid retraction, and gaze palsy. This occurs because the proc...

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Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options

OPINION STATEMENT There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case ...

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ژورنال

عنوان ژورنال: Journal of Neuropathology and Experimental Neurology

سال: 2006

ISSN: 0022-3069

DOI: 10.1097/01.jnen.0000218446.38158.61