Myasthenic Disorders and ALS

February 2009, Volume 15, Issue 1

Myasthenic Disorders and ALS

February 2009, Vol.15, No.1

Guest Editor:

Matthew N. Meriggioli, MD


Aaron E. Miller, MD

ISSN: 1080-2371

Online ISSN: 1538-6899

Faculty: PDF Only
CONTINUUM: Lifelong Learning in Neurology
February 2009 - Volume 15 - Issue 1, Myasthenic Disorders and ALS - p 1-4
doi: 10.1212/01.CON.0000300012.87469.e2
Editor's Preface
Key Points
Issue Overview
Aaron E. Miller, MD


Aaron E. Miller, MD


CONTINUUM: Lifelong Learning in Neurology February 2009 - Volume 15 - Issue 1, Myasthenic Disorders and ALS -p 11-12 doi: 10.1212/01.CON.0000300013.69543.fd

When I assumed the position of editor of CONTINUUM approximately 6 years ago, we introduced a 3-year curriculum cycle, covering four core topics each year. The other two issues of CONTINUUM annually review topics that are less heavily represented in the American Board of Psychiatry and Neurology recertification examination but are nonetheless important. Among the core subjects is neuromuscular disorders, and in previous cycles we have covered peripheral neuropathies and muscle diseases, respectively. In this issue, we address two very important neuromuscular topics-myasthenia and amyotrophic lateral sclerosis (ALS)-that have not been reviewed thoroughly in CONTINUUM recent years.

Myasthenia gravis (MG) and ALS are critically important subjects for neurologists despite the relatively small numbers of individuals afflicted with these disorders. MG is the prototypic disorder of the neuromuscular junction and also focuses our attention on autoimmunity as an important determinant of neurologic disease. ALS not only commands our attention as an example of a neurodegenerative disease of the motor system, but it also provides critically important lessons about the management of a chronic, progressive, and ultimately fatal disorder.

For this issue, Dr Matthew Meriggioli graciously accepted the challenge of addressing these two seemingly disparate subjects. However, in the first chapter, Dr Julie Rowin reminds us that, at first presentation, patients with MG and ALS may not always be so easy to distinguish. She offers guidance to the clinician approaching patients suspected of having one of these disorders and provides many useful clinical pearls. In the next chapter, Dr Meriggioli himself reviews the immunopathogenesis, diagnosis, and management of autoimmune MG. Other forms of myasthenia exist, however. In the subsequent chapter Dr C. Michel Harper addresses the subject of congenital myasthenic syndromes. This is a fascinating review of a variety of genetic disorders that cause neuromuscular junction dysfunction. Although most neurologists will seldom encounter patients with these defects, understanding them provides us with a better grasp of the physiology of all neuromuscular junction disorders.

The second half of this CONTINUUM issue switches gears to tackle ALS. Drs Richard Bedlack and Swati Aggarwal first address classic ALS, providing a hopeful discussion that highlights progress in our understanding of this disorder while also covering diagnostic and management issues. Following this, Dr Richard Barohn reviews the clinical spectrum of motor neuron disorders, paying particular attention to the variants of primary lateral sclerosis and pure lower motor neuron disorders, respectively. In the final formal chapter of this issue, Dr Steven Vernino discusses paraneoplastic disorders affecting both the neuromuscular junction and the anterior horn cell. This class of autoimmune diseases, while uncommon, is always intriguing, and the spectrum of these disorders continues to expand.

As usual, we have included a section of Ethical Perspectives in Neurology related to our subject. In this issue, Drs Philippe Couillard and A. Keith Brownell address some critically important ethical issues related to end-oflife decision making for a patient with advanced ALS. In addition, Dr Steven Lewis joins attorney Alyssa Stamatakos in writing the Practice Issues in Neurology section that reviews the practical issues of informed consent. Finally, your mastery of the subjects of myasthenia and ALS will be both tested and enhanced by working through the patient management problem devised by Dr Andrea Swenson and the multiple-choice questions, prepared for this issue by Drs Douglas Gelb and D. Joanne Lynn.

Encountering a patient with myasthenia or ALS will not be a very frequent experience for most readers. But the occurrence will not be rare, and when you are challenged to provide the care for these very interesting patients, you will be far better prepared as a result of digesting this issue of CONTINUUM. We are indebted to Dr Meriggioli and his talented faculty for guiding our way.

-Aaron E. Miller, MD

© 2009 American Academy of Neurology