A case of atypical progressive supranuclear palsy

Simona Spaccavento, Marina Del Prete, Angela Craca, Anna Loverre IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy Background: Progressive supranuclear palsy (PSP) is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical d...

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Autores principales:	Spaccavento S, Del Prete M, Craca A, Loverre A
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2013
Materias:	Progressive supranuclear palsy pharmacological treatments neurosychological deficits. Geriatrics RC952-954.6
Acceso en línea:	https://doaj.org/article/67c9d4640c37403d8799dfa74882db0c
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spelling	oai:doaj.org-article:67c9d4640c37403d8799dfa74882db0c2021-12-02T00:52:10ZA case of atypical progressive supranuclear palsy1178-1998https://doaj.org/article/67c9d4640c37403d8799dfa74882db0c2013-12-01T00:00:00Zhttps://www.dovepress.com/a-case-of-atypical-progressive-supranuclear-palsy-peer-reviewed-article-CIAhttps://doaj.org/toc/1178-1998Simona Spaccavento, Marina Del Prete, Angela Craca, Anna Loverre IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy 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 various pharmacological treatments. Methods: A 72-year-old right-handed male, with 17 years education, who first presented with aphasia, AOS, depression, apathy, and postural instability at 69 years; a complete neuropsychological evaluation, tapping the different cognitive domains, was performed. Results: Testing revealed a moderate global cognitive deficit (Mini-Mental State Examination test score =20), low memory test scores (story recall, Rey’s 15-word Immediate and Delayed Recall), and poor phonemic and semantic fluency. The patient’s language was characterized by AOS, with slow speech rate, prolonged intervals between syllables and words, decreased articulatory accuracy, sound distortions, and anomia. Behavioral changes, such as depression, anxiety, apathy, and irritability, were reported. The neurological examination revealed supranuclear vertical gaze palsy, poor face miming, and a mild balance deficit. Magnetic resonance imaging showed only widespread cortical atrophy. Single photon emission computed tomography demonstrated left > right frontotemporal cortical abnormalities. After 6 months, a further neuropsychological assessment showed a progression in cognitive deficits, with additional attention deficits. The patient reported frequent falls, but the neurological deficits remained unchanged. Neuroimaging tests showed the same brain involvement. Conclusion: Our case highlights the heterogeneity of the clinical features in this syndrome, demonstrating that atypical PSP can present as AOS and aphasia, without the classical features or involvement of the subcortical gray and brainstem region, commonly affected in typical PSP. Keywords: pharmacological treatments, neuropsychological deficitsSpaccavento SDel Prete MCraca ALoverre ADove Medical PressarticleProgressive supranuclear palsypharmacological treatmentsneurosychological deficits.GeriatricsRC952-954.6ENClinical Interventions in Aging, Vol Volume 9, Pp 31-39 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Progressive supranuclear palsy pharmacological treatments neurosychological deficits. Geriatrics RC952-954.6
spellingShingle	Progressive supranuclear palsy pharmacological treatments neurosychological deficits. Geriatrics RC952-954.6 Spaccavento S Del Prete M Craca A Loverre A A case of atypical progressive supranuclear palsy
description	Simona Spaccavento, Marina Del Prete, Angela Craca, Anna Loverre IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy 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 various pharmacological treatments. Methods: A 72-year-old right-handed male, with 17 years education, who first presented with aphasia, AOS, depression, apathy, and postural instability at 69 years; a complete neuropsychological evaluation, tapping the different cognitive domains, was performed. Results: Testing revealed a moderate global cognitive deficit (Mini-Mental State Examination test score =20), low memory test scores (story recall, Rey’s 15-word Immediate and Delayed Recall), and poor phonemic and semantic fluency. The patient’s language was characterized by AOS, with slow speech rate, prolonged intervals between syllables and words, decreased articulatory accuracy, sound distortions, and anomia. Behavioral changes, such as depression, anxiety, apathy, and irritability, were reported. The neurological examination revealed supranuclear vertical gaze palsy, poor face miming, and a mild balance deficit. Magnetic resonance imaging showed only widespread cortical atrophy. Single photon emission computed tomography demonstrated left > right frontotemporal cortical abnormalities. After 6 months, a further neuropsychological assessment showed a progression in cognitive deficits, with additional attention deficits. The patient reported frequent falls, but the neurological deficits remained unchanged. Neuroimaging tests showed the same brain involvement. Conclusion: Our case highlights the heterogeneity of the clinical features in this syndrome, demonstrating that atypical PSP can present as AOS and aphasia, without the classical features or involvement of the subcortical gray and brainstem region, commonly affected in typical PSP. Keywords: pharmacological treatments, neuropsychological deficits
format	article
author	Spaccavento S Del Prete M Craca A Loverre A
author_facet	Spaccavento S Del Prete M Craca A Loverre A
author_sort	Spaccavento S
title	A case of atypical progressive supranuclear palsy
title_short	A case of atypical progressive supranuclear palsy
title_full	A case of atypical progressive supranuclear palsy
title_fullStr	A case of atypical progressive supranuclear palsy
title_full_unstemmed	A case of atypical progressive supranuclear palsy
title_sort	case of atypical progressive supranuclear palsy
publisher	Dove Medical Press
publishDate	2013
url	https://doaj.org/article/67c9d4640c37403d8799dfa74882db0c
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A case of atypical progressive supranuclear palsy

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