Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findings

Journal of Neurology, Neurosurgery, and Psychiatry
J Kuipers-UpmeijerT W van Weerden

Abstract

To describe the clinical, neurophysiological, and MRI findings in 10 patients with primary lateral sclerosis (PLS). The course of the disease was very slowly progressive. Spasticity due to upper motor neuron dysfunction was the most prominent sign, but EMG showed slight lower motor neuron signs, such as a mixed pattern on maximal voluntary contraction and enlarged motor unit potentials. One patient had clinically mild lower motor neuron involvement. Central motor conduction times (CMCT) were more prolonged in PLS than is the case in ALS. Minor sensory signs were found on neurophysiological examination, comparable with those in ALS. In four patients serum creatine kinase activity was raised. On MRI cortical atrophy was seen, most pronounced in the precentral gyrus and expanding into the parietal-occipital region. PLS is a distinct clinical syndrome, part of the range of motor neuron diseases. Besides pronounced upper motor neuron symptoms, mild lower motor neuron symptoms can also be found, as well as (subclinical) sensory symptoms. PLS can be distinguished from ALS by its slow clinical course, a severely prolonged MEP, and a more extensive focal cortical atrophy.

References

May 1, 1992·Muscle & Nerve·W F BrownJ Veitch
Apr 1, 1992·Brain : a Journal of Neurology·C E PringleG C Ebers
Aug 1, 1991·Electroencephalography and Clinical Neurophysiology·M D CaramiaP M Rossini
Oct 1, 1986·Journal of Neurology, Neurosurgery, and Psychiatry·J M Pearce
Mar 1, 1986·Archives of Physical Medicine and Rehabilitation·M Sinaki, D W Mulder
Nov 1, 1995·Neurology·R J CaselliD Osborne
Jun 1, 1995·Journal of Neurology, Neurosurgery, and Psychiatry·R P BruynJ M de Jong
Aug 1, 1994·Brain : a Journal of Neurology·J A Kiernan, A J Hudson
Aug 1, 1993·Journal of the Neurological Sciences·F NorrisH Norris
May 1, 1996·Fortschritte der Neurologie-Psychiatrie·R HeeneH Knisatschek
Feb 1, 1996·Journal of Neurology·N MeucciG Scarlato
Mar 4, 1999·Neuromuscular Disorders : NMD·J Desai, M Swash
Aug 6, 1999·Stroke; a Journal of Cerebral Circulation·G SulterJ De Keyser
Dec 14, 1999·Journal of the Neurological Sciences·M SwashV P Misra
Apr 20, 2001·Journal of the Neurological Sciences·N Le ForestierV Meininger

❮ Previous
Next ❯

Citations

Jun 10, 2010·Der Nervenarzt·P P UrbanC Pohlmann
Mar 25, 2005·Journal of Neurology·Jerônimo Buzetti MilanoErasmo Barros
Feb 13, 2007·Journal of Neurology·Martin R TurnerP Nigel Leigh
Feb 19, 2009·Journal of Neurology·Julie Phukan, Orla Hardiman
Mar 3, 2011·Brain : a Journal of Neurology·Maaike M van der GraaffMarianne de Visser
Nov 2, 2012·Brain : a Journal of Neurology·Christel DentelLuc Dupuis
Mar 1, 2003·Journal of Clinical Neuromuscular Disease·Judah Lindenberg, David C Preston
Sep 1, 2005·Journal of Clinical Neuromuscular Disease·Mike A SingerGil I Wolfe
Dec 22, 2010·The British Journal of Radiology·L C TzarouchiM I Argyropoulou
Sep 28, 2002·Current Opinion in Neurology·Christian Krarup
Nov 30, 2004·Expert Opinion on Investigational Drugs·Michael J Strong
Jul 23, 2005·Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases·Lewis P Rowland
Jul 9, 2008·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Gianni SorarùCorrado Angelini
Nov 26, 2009·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Gianni SorarùCorrado Angelini
Aug 28, 2015·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Malcolm ProudfootMartin R Turner
May 18, 2013·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Shannon KolindMartin R Turner
Feb 6, 2010·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Edward D HueyMary Kay Floeter
Jul 23, 2005·Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases·Michael J Strong, Paul H Gordon
May 21, 2010·Seminars in Ultrasound, CT, and MR·Juan A Guzmán-De-VilloriaPilar Fernández-García
Oct 10, 2008·Revue neurologique·N Le Forestier, V Meininger
Feb 12, 2008·Clinical Neurology and Neurosurgery·Barbara TomikAndrzej Szczudlik
Oct 31, 2015·Neurologic Clinics·Jeffrey M StatlandHiroshi Mitsumoto
Jul 31, 2007·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·A BeckerA Czaplinski
Jan 11, 2007·Muscle & Nerve·Mike A SingerRichard J Barohn
Oct 22, 2005·Annals of Neurology·Frans BrugmanLeonard H van den Berg
Jan 4, 2008·Human Brain Mapping·Olga CiccarelliStephen M Smith
Aug 19, 2007·Movement Disorders : Official Journal of the Movement Disorder Society·Ibrahim M NorlinahNiall P Quinn
Mar 28, 2006·Annals of Neurology·Ou BaiMary Kay Floeter
Sep 24, 2013·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·H M ClarkK A Josephs
May 2, 2013·Human Brain Mapping·Federica AgostaMassimo Filippi
Aug 16, 2005·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Cathy L ShortSimon A Koblar
Feb 17, 2015·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·N GeevasingaS Vucic
Jul 1, 2003·Expert Review of Neurotherapeutics·Wolfgang GrisoldAndrea Vass
Nov 12, 2013·Neurobiology of Aging·Federica AgostaMassimo Filippi
Feb 22, 2012·Journal of the Neurological Sciences·Tommaso BocciFerdinando Sartucci
Jul 26, 2013·Neurorehabilitation and Neural Repair·Francois BethouxErik P Pioro
Apr 19, 2018·Expert Review of Neurotherapeutics·Thanuja DharmadasaMatthew C Kiernan
Jan 11, 2018·Neurodegenerative Disease Management·Ramnath Santosh Ramanathan, Sandeep Rana
Jan 19, 2019·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Eoin FineganPeter Bede
Jan 23, 2020·Journal of Clinical Medicine·Tereza FilipiAnd Miroslava Anderova
Feb 8, 2020·Journal of Neurology, Neurosurgery, and Psychiatry·Martin R TurnerHiroshi Mitsumoto
Jun 23, 2020·Expert Review of Neurotherapeutics·Pamela A McCombeRobert D Henderson
Nov 20, 2016·Acta Neurologica Scandinavica·Verena WaisJohannes Dorst
Jun 6, 2018·Journal of Neurology·Smriti AgarwalMatthew C Kiernan
Mar 21, 2006·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Raquel Gil GouveiaMamede de Carvalho

❮ Previous
Next ❯

Related Concepts

Related Feeds

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.

Cardiac Conduction System

The cardiac conduction system is a specialized tract of myocardial cells responsible for maintaining normal cardiac rhythm. Discover the latest research on the cardiac conduction system here.

Related Papers

Journal of the Neurological Sciences
Nadine Le ForestierVincent Meininger
Journal of the Neurological Sciences
M SwashV P Misra
© 2021 Meta ULC. All rights reserved