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The concept and diagnostic criteria of primary lateral sclerosis.

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Title: The concept and diagnostic criteria of primary lateral sclerosis.
Authors: Wais, Verena, Rosenbohm, Angela, Petri, Susanne, Kollewe, Katja, Hermann, Andreas, Storch, Alexander, Hanisch, Frank, Zierz, Stephan, Nagel, Gabriele, Kassubek, Jan, Weydt, Patrick, Brettschneider, Johannes, Weishaupt, Jochen H., Ludolph, Albert C., Dorst, Johannes
Source: Acta Neurologica Scandinavica. Sep2017, Vol. 136 Issue 3, p204-211. 8p.
Subjects: Amyotrophic lateral sclerosis, Disease progression, Diagnostic imaging, Symptoms, Electrophysiology, Diagnosis
Abstract: Objectives Primary lateral sclerosis ( PLS) is commonly considered as a motor neuron disease ( MND) variant which almost exclusively affects upper motor neurons ( UMN). There is still no consensus whether PLS should be regarded as an independent disease entity separate from amyotrophic lateral sclerosis ( ALS) or as a comparatively slowly progressive variant of ALS. Given these different views, clinical diagnosis of PLS is a challenge. In this multicenter study, we analyzed clinical features of patients diagnosed with PLS in four specialized MND centers. Material and Methods We retrospectively analyzed clinical, laboratory, imaging, and electrophysiological data of 76 patients with PLS diagnosed in four specialized ALS centers. We analyzed the concept of the disease based on our findings and an extensive review of the literature. Results We found that 79% of patients showed asymmetrical symptoms, 60% showed clinical or electrophysiological signs of lower motor neuron ( LMN) involvement after a mean of 8.4 ± 5.0 years, and extrapyramidal and/or non-motoric symptoms were frequently observed. Interestingly, none of the patients diagnosed with PLS fulfilled the diagnostic criteria proposed by Pringle et al. in 1992. Conclusions Our data show that PLS as a disease entity is still not well enough defined and that there are different concepts about its clinical presentation. We believe that further prospective longitudinal studies are needed in order to refine diagnostic criteria to reflect current clinical practice. Furthermore, neuropathological and neuroimaging approaches might help to arrange PLS in the MND spectrum and its classification. [ABSTRACT FROM AUTHOR]
Copyright of Acta Neurologica Scandinavica is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
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  Data: The concept and diagnostic criteria of primary lateral sclerosis.
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  Data: <searchLink fieldCode="AR" term="%22Wais%2C+Verena%22">Wais, Verena</searchLink><br /><searchLink fieldCode="AR" term="%22Rosenbohm%2C+Angela%22">Rosenbohm, Angela</searchLink><br /><searchLink fieldCode="AR" term="%22Petri%2C+Susanne%22">Petri, Susanne</searchLink><br /><searchLink fieldCode="AR" term="%22Kollewe%2C+Katja%22">Kollewe, Katja</searchLink><br /><searchLink fieldCode="AR" term="%22Hermann%2C+Andreas%22">Hermann, Andreas</searchLink><br /><searchLink fieldCode="AR" term="%22Storch%2C+Alexander%22">Storch, Alexander</searchLink><br /><searchLink fieldCode="AR" term="%22Hanisch%2C+Frank%22">Hanisch, Frank</searchLink><br /><searchLink fieldCode="AR" term="%22Zierz%2C+Stephan%22">Zierz, Stephan</searchLink><br /><searchLink fieldCode="AR" term="%22Nagel%2C+Gabriele%22">Nagel, Gabriele</searchLink><br /><searchLink fieldCode="AR" term="%22Kassubek%2C+Jan%22">Kassubek, Jan</searchLink><br /><searchLink fieldCode="AR" term="%22Weydt%2C+Patrick%22">Weydt, Patrick</searchLink><br /><searchLink fieldCode="AR" term="%22Brettschneider%2C+Johannes%22">Brettschneider, Johannes</searchLink><br /><searchLink fieldCode="AR" term="%22Weishaupt%2C+Jochen+H%2E%22">Weishaupt, Jochen H.</searchLink><br /><searchLink fieldCode="AR" term="%22Ludolph%2C+Albert+C%2E%22">Ludolph, Albert C.</searchLink><br /><searchLink fieldCode="AR" term="%22Dorst%2C+Johannes%22">Dorst, Johannes</searchLink>
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  Data: <searchLink fieldCode="JN" term="%22Acta+Neurologica+Scandinavica%22">Acta Neurologica Scandinavica</searchLink>. Sep2017, Vol. 136 Issue 3, p204-211. 8p.
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  Data: <searchLink fieldCode="DE" term="%22Amyotrophic+lateral+sclerosis%22">Amyotrophic lateral sclerosis</searchLink><br /><searchLink fieldCode="DE" term="%22Disease+progression%22">Disease progression</searchLink><br /><searchLink fieldCode="DE" term="%22Diagnostic+imaging%22">Diagnostic imaging</searchLink><br /><searchLink fieldCode="DE" term="%22Symptoms%22">Symptoms</searchLink><br /><searchLink fieldCode="DE" term="%22Electrophysiology%22">Electrophysiology</searchLink><br /><searchLink fieldCode="DE" term="%22Diagnosis%22">Diagnosis</searchLink>
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  Data: Objectives Primary lateral sclerosis ( PLS) is commonly considered as a motor neuron disease ( MND) variant which almost exclusively affects upper motor neurons ( UMN). There is still no consensus whether PLS should be regarded as an independent disease entity separate from amyotrophic lateral sclerosis ( ALS) or as a comparatively slowly progressive variant of ALS. Given these different views, clinical diagnosis of PLS is a challenge. In this multicenter study, we analyzed clinical features of patients diagnosed with PLS in four specialized MND centers. Material and Methods We retrospectively analyzed clinical, laboratory, imaging, and electrophysiological data of 76 patients with PLS diagnosed in four specialized ALS centers. We analyzed the concept of the disease based on our findings and an extensive review of the literature. Results We found that 79% of patients showed asymmetrical symptoms, 60% showed clinical or electrophysiological signs of lower motor neuron ( LMN) involvement after a mean of 8.4 ± 5.0 years, and extrapyramidal and/or non-motoric symptoms were frequently observed. Interestingly, none of the patients diagnosed with PLS fulfilled the diagnostic criteria proposed by Pringle et al. in 1992. Conclusions Our data show that PLS as a disease entity is still not well enough defined and that there are different concepts about its clinical presentation. We believe that further prospective longitudinal studies are needed in order to refine diagnostic criteria to reflect current clinical practice. Furthermore, neuropathological and neuroimaging approaches might help to arrange PLS in the MND spectrum and its classification. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of Acta Neurologica Scandinavica is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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        Value: 10.1111/ane.12713
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        Text: English
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      – SubjectFull: Amyotrophic lateral sclerosis
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      – SubjectFull: Diagnostic imaging
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