Verena Wais1, Angela Rosenbohm1, Susanne Petri2, Katja Kollewe2, Andreas Hermann3,4, Alexander Storch3,4,5, Frank Hanisch6, Stephan Zierz7, Gabriele Nagel8, Jan Kassubek1, Patrick Weydt1, Johannes Brettschneider9, Jochen H Weishaupt1, Albert C Ludolph1, Johannes Dorst1. 1. Department of Neurology, University of Ulm, Ulm, Germany. 2. Department of Neurology, University of Hannover, Hannover, Germany. 3. Department of Neurology, Division for Neurodegenerative Diseases, Dresden University of Technology, Dresden, Germany. 4. German Center for Neurodegenerative Diseases (DZNE), Dresden, Germany. 5. Department of Neurology, University Medical Center Rostock, Rostock, Germany. 6. Department of Neurology, Evangelisches Krankenhaus Königin Elisabeth Herzberge, Berlin, Germany. 7. Department of Neurology, University of Halle, Halle, Germany. 8. Institute for Epidemiology and Medical Biometry, University of Ulm, Ulm, Germany. 9. CaritasKlinikum, Saarbrücken, Germany.
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.
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.
Authors: Johannes Dorst; Lu Chen; Angela Rosenbohm; Dong-Sheng Fan; Albert C Ludolph; Jens Dreyhaupt; Annemarie Hübers; Joachim Schuster; Jochen H Weishaupt; Jan Kassubek; Burkhard Gess; Thomas Meyer; Ute Weyen; Andreas Hermann; Jürgen Winkler; Torsten Grehl; Tim Hagenacker; Paul Lingor; Jan C Koch; Anne Sperfeld; Susanne Petri; Julian Großkreutz; Moritz Metelmann; Joachim Wolf; Andrea S Winkler; Thomas Klopstock; Matthias Boentert; Siw Johannesen; Alexander Storch; Bertold Schrank; Daniel Zeller; Xiao-Lu Liu; Lu Tang Journal: J Neurol Date: 2019-03-28 Impact factor: 4.849
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Authors: Martin R Turner; Richard J Barohn; Philippe Corcia; John K Fink; Matthew B Harms; Matthew C Kiernan; John Ravits; Vincenzo Silani; Zachary Simmons; Jeffrey Statland; Leonard H van den Berg; Hiroshi Mitsumoto Journal: J Neurol Neurosurg Psychiatry Date: 2020-02-06 Impact factor: 10.154
Authors: Hava Özlem Dede; Nermin Görkem Şırın; Elif Kocasoy-Orhan; Halil Atilla Idrısoğlu; Mehmet Barış Baslo Journal: Noro Psikiyatr Ars Date: 2019-08-16 Impact factor: 1.339