Chapter 53. Primary Lateral Sclerosis

  1. Robert P. Lisak MD, FAAN, FRCP Parker Webber Chair Professor Chair Neurologist-in-Chief Chief3,4,
  2. Daniel D. Truong MD, FAAN Head5,
  3. William M. Carroll MBBS, MD, FRACP, FRCP(E) Head6 and
  4. Roongroj Bhidayasiri MD, FRCP Director7,8
  1. Yvonne D. Rollins MD, PhD Assistant Professor of Neurology1,
  2. Björn Oskarsson MD Assistant Professor2 and
  3. Steven P. Ringel MD Professor of Neurology1

Published Online: 18 MAY 2010

DOI: 10.1002/9781444317008.ch53

International Neurology: A Clinical Approach

International Neurology: A Clinical Approach

How to Cite

Rollins, Y. D., Oskarsson, B. and Ringel, S. P. (2009) Primary Lateral Sclerosis, in International Neurology: A Clinical Approach (eds R. P. Lisak, D. D. Truong, W. M. Carroll and R. Bhidayasiri), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444317008.ch53

Editor Information

  1. 3

    Wayne State University School of Medicine, Detroit, MI, USA

  2. 4

    Detroit Medical Center, Harper University Hospital, Detroit, MI, USA

  3. 5

    The Parkinson and Movement Disorder Institute, Memorial Neuroscience Institute, Orange Coast Memorial Medical Center, Fountain Valley, CA, USA

  4. 6

    Sir Charles Gairdner Hospital, Nedlands, Perth, Australia

  5. 7

    Chulalongkorn Comprehensive Movement Disorders Center, Chulalongkorn University Hospital, Bangkok, Thailand

  6. 8

    University of California at Los Angeles, School of Medicine, Los Angeles, CA, USA

Author Information

  1. 1

    University of Colorado, Denver, Aurora, CO, USA

  2. 2

    Clinical Neurology, University of California, Davis, Sacramento, CA, USA

Publication History

  1. Published Online: 18 MAY 2010
  2. Published Print: 11 SEP 2009

ISBN Information

Print ISBN: 9781405157384

Online ISBN: 9781444317008



  • primary lateral sclerosis;
  • primary lateral sclerosis (PLS), primary upper motor neuron (UMN) disorder;
  • clinical syndrome of PLS - rare, accounting for 1–3% of patients with motor neuron disease;
  • progressive lower extremity spasticity without significant loss of strength - classic initial symptom;
  • etiology of motor neuron degeneration - remaining unknown;
  • PLS, a diagnosis of exclusion - other disorders, including ALS;
  • no curative treatment - available for PLS;
  • EMG, useful in distinguishing PLS from ALS - based on absence of diffuse chronic denervation


This chapter contains sections titled:

  • Introduction

  • Epidemiology

  • Pathophysiology

  • Clinical features

  • Investigations

  • Treatment/management

  • Further reading