Neurology
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Correspondence:
Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Correspondence are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Younger, D. S.
Right arrow Articles by Kim, T. S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Younger, D. S.
Right arrow Articles by Kim, T. S.
NEUROLOGY 1990;40:595
© 1990 American Academy of Neurology

Motor neuron disease and amyotrophic lateral sclerosis

Relation of high CSF protein content to paraproteinemia and clinical syndromes

D. S. Younger, MD, L. P. Rowland, MD, N. Latov, MD, PhD, W. Sherman, MD, M. Pesce, PhD, D. J. Lange, MD, W. Trojaborg, MD, J. R. Miller, MD, R. E. Lovelace, MD, A. P. Hays, MD and T. S. Kim, MS

Departments of Neurology (Drs. Younger, Rowland, Latov, Lange, Trojaborg, Miller, Lovelace, and Kim); Medicine (Division of Oncology) (Dr. Sherman); Pathology, Divisions of Clinical Pathology (Dr. Pesce), and Neuropathology (Dr. Hays), Presbyterian Hospital, Columbia University, College of Physicians and Surgeons, New York, NY.

From 1984 to 1988, 11 of 120 patients (9%) with motor neuron disease (MND) had paraproteins detected by serum immunofixation electrophoresis (IFE), compared with 4 (3%) by cellulose acetate gels: 1 patient had progressive spinal muscular atrophy, 5 patients had amyotrophic lateral sclerosis (ALS), and 5 patients had ALS with probable upper motor neuron signs. Four of 5 patients (80%) with cerebrospinal fluid (CSF) protein content above 75 mg/dl had paraproteins, as did 6 of 30 with values above 50 mg/dl. Four of 14 patients with cerebrospinal oligoclonal bands (OCB) also had paraproteins. Two patients with ALS, CSF protein content above 75 mg/dl, and paraproteinemia had lymphoma. We conclude the following about patients with MND: high CSF protein content (especially above 75 mg/dl) or CSF OCB makes paraproteinemia more likely; some of these patients may have lymphoma; there is an inordinately high occurrence of paraproteinemia in MND; and IFE on agarose is more sensitive than electrophoresis on cellulose acetate in detecting paraproteins. Syndromes of paraproteinemia and high CSF protein are not restricted to the lower motor neuron but qualify as "ALS" with coexisting upper motor neuron signs.

Address correspondence and reprint requests to Dr. David S. Younger, Neurological Institute of the Columbia-Presbyterian Medical Center, Box 53, 710 West 168th Street, New York, NY 10032-3784.

Supported by the Center Grants from the Muscular Dystrophy Association and NIH (NINCDS-NS11766) and GCRC (RR-00645).

Received June 23, 1989. Accepted for publication in final form September 15, 1989.




This article has been cited by other articles:


Home page
 NEJMHome page
W. J. Triggs and D. Cros
Case 40-2007 -- A 38-Year-Old Man with Weakness in the Hands
N. Engl. J. Med., December 27, 2007; 357(26): 2707 - 2715.
[Full Text] [PDF]

Home page
 NeurologyHome page
P. Kaufmann, S. L. Pullman, D. C. Shungu, S. Chan, A. P. Hays, M. L. Del Bene, M. A. Dover, M. Vukic, L. P. Rowland, and H. Mitsumoto
Objective tests for upper motor neuron involvement in amyotrophic lateral sclerosis (ALS)
Neurology, May 25, 2004; 62(10): 1753 - 1757.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurol. Neurosurg. PsychiatryHome page
J Kuipers-Upmeijer, A E J de Jager, J M Hew, J W Snoek, and T W van Weerden
Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findings
J. Neurol. Neurosurg. Psychiatry, November 1, 2001; 71(5): 615 - 620.
[Abstract] [Full Text] [PDF]

Home page
 Arch NeurolHome page
R. Dabby, D. J. Lange, W. Trojaborg, A. P. Hays, R. E. Lovelace, T. H. Brannagan, and L. P. Rowland
Inclusion Body Myositis Mimicking Motor Neuron Disease
Arch Neurol, August 1, 2001; 58(8): 1253 - 1256.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
W. J. Triggs, D. Menkes, J. Onorato, R. S.- H. Yan, M. S. Young, K. Newell, H. W. Sander, O. Soto, K. H. Chiappa, and D. Cros
Transcranial magnetic stimulation identifies upper motor neuron involvement in motor neuron disease
Neurology, August 1, 1999; 53(3): 605 - 605.
[Abstract] [Full Text]

Home page
 NEJMHome page
D. A. Chad and N. L. Harris
Case 16-1999- A 71-Year-Old Man with Progressive Weakness and a Gammopathy
N. Engl. J. Med., May 27, 1999; 340(21): 1661 - 1669.
[Full Text] [PDF]

Home page
 NEJMHome page
A. H. Ropper and K. C. Gorson
Neuropathies Associated with Paraproteinemia
N. Engl. J. Med., May 28, 1998; 338(22): 1601 - 1607.
[Full Text] [PDF]

Home page
 Arch NeurolHome page
D. J. Lange, P. L. Murphy, B. Diamond, V. Appel, E. C. Lai, D. S. Younger, and S. H. Appel
Selegiline Is Ineffective in a Collaborative Double-blind, Placebo-Controlled Trial for Treatment of Amyotrophic Lateral Sclerosis
Arch Neurol, January 1, 1998; 55(1): 93 - 96.
[Abstract] [Full Text] [PDF]

Home page
 NEJMHome page
W.J. Triggs and M.A. Edgar
Case 36-1995- A 61-year-old man with increasing weakness and atrophy of all extremities
N. Engl. J. Med., November 23, 1995; 333(21): 1406 - 1412.
[Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 1990 by AAN Enterprises, Inc.