This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Marinos C. Dalakas, MD; Daniel P. Stein, MD; Carlos Otero, MD; Elizabeth Sekul, MD; Edward J. Cupler, MD; Susan McCrosky, RN

Arch Neurol. 1994;51(9):861-864.

Abstract
Objective
To determine if high-dose intravenous immunoglobulin therapy is effective in improving muscle strength or in arresting the pace of disease progression in patients with rapidly progressive amyotrophic lateral sclerosis.

Design
An open-label pilot study of intravenous infusions of high-dose immunoglobulin administered once a month for 3 months in nine patients with classic amyotrophic lateral sclerosis. Selected patients had a rapidly progressive course with signs of worsening noticeably evident every 6 weeks prior to therapy. A patient with multifocal motor neuropathy with conduction block that presented as a lower motor neuron syndrome was concurrently treated to document the efficacy of the same preparation of immunoglobulin in a potentially treatable disease that simulates lower motor neuron syndrome. The efficacy of high-dose intravenous immunoglobulin infusions was assessed by objective measurement of maximum voluntary isometric contraction in all muscle groups of two limbs before and after therapy.

Setting
The Clinical Center of the National Institutes of Health, Bethesda, Md.

Results
All patients with amyotrophic lateral sclerosis worsened during the study. By the end of the third month, their mean total muscle scores (megascores) had declined by 71.2 points, from a mean of 369.7 (range, 200 to 605) to 298.5 (range, 130 to 552) points. The pace of progression did not change during the 4-month observation period. In contrast, the patient with multifocal motor neuropathy responded to intravenous immunoglobulin therapy and increased his megascores by 146 points after 3 months. The GM₁ antibody titers were normal in all the patients.

Conclusions
High-dose intravenous immunoglobulin, a prohibitively expensive drug, has no apparent therapeutic role in improving the symptoms or arresting the pace of progression in patients with amyotrophic lateral sclerosis. In contrast, multifocal motor neuropathy is an immunopathologically different disease that responds to intravenous immunoglobulin therapy.

Author Affiliations
From the Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Md.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Activity-dependent conduction block in multifocal motor neuropathy: magnetic fatigue test.
Nodera et al.
Neurology 2006;67:280-287.
ABSTRACT | FULL TEXT  

Axon loss is an important determinant of weakness in multifocal motor neuropathy.
Van Asseldonk et al.
J. Neurol. Neurosurg. Psychiatry 2006;77:743-747.
ABSTRACT | FULL TEXT  

Seroconversion of anti-GM1 antibodies in patients with amyotrophic lateral sclerosis
Haggiag et al.
Neurology 2004;63:755-756.
FULL TEXT  

Intravenous Immunoglobulin in Autoimmune Neuromuscular Diseases
Dalakas
JAMA 2004;291:2367-2375.
ABSTRACT | FULL TEXT  

Intravenous immunoglobulin in neurological disease: a specialist review
Wiles et al.
J. Neurol. Neurosurg. Psychiatry 2002;72:440-448.
ABSTRACT | FULL TEXT  

Is the Routine Use of Intravenous Immunoglobulin Treatment in Neurologic Disorders Justified?: No
Karussis and Abramsky
Arch Neurol 1999;56:1028-1032.
FULL TEXT