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CSF Oligoclonal Bands, Immunoglobulins, and Viral Antibodies in Progressive Myoclonus Epilepsy

CSF Oligoclonal Bands, Immunoglobulins, and Viral Antibodies in Progressive Myoclonus Epilepsy Abstract • We studied CSF and serum samples from 16 patients with progressive myoclonus epilepsy (PME). These patients had juvenile-onset PME with evidence of autosomal recessive inheritance and no Lafora bodies. Twelve of the 16 patients with PME had immunologic abnormalities. Oligoclonal γ bands were seen in six of the eight patients from whom sufficient CSF was available. The CSF albumin and serum/CSF albumin ratios were normal in all 16 patients, indicating the presence of intact blood-brain barriers. Six of the 16 patients showed increased CSF IgG levels and five had an increased CNS IgG synthesis. All patients had normal serum and CSF IgM and IgA levels. Three patients, all with bands, had reduced measles and/ or vaccinia serum/CSF antibody ratios. The findings suggest altered immune response of the CNS of some patients with PME apparently caused by nonspecific immunostimulation. References 1. Diebold K: Vier Erbtypen oder Krankheitsformen der progressiven Myoklonusepilepsien . Arch Psychiat Nervenkr 1972;215:362-375.Crossref 2. Koskiniemi M, Donner M, Majuri H, et al: Progressive myoclonus epilepsy: A clinical and histopathological study . Acta Neurol Scand 1974;50:307-332.Crossref 3. Taskinen E, Iivanainen M: Lymphoid cell reaction and immunoglobulins in cerebrospinal fluid in progressive myoclonus epilepsy, abstracted, in Abstracts of the 11th World Congress of Neurology, Excerpta Medica International Congress Series No. 427. Amsterdam, Excerpta Medica, 1977, No. 26, p 9. 4. Iivanainen M: New medical treatment of progressive myoclonus epilepsy, abstracted . Acta Neurol Scand 1976;54:98. 5. Johnson P, Arrigo SC, Nelson BJ, et al: Agarose electrophoresis of cerebrospinal fluid in multiple sclerosis: A simplified method for demonstrating cerebrospinal fluid oligoclonal immunoglobulin bands . Neurology 1977;27:273-277.Crossref 6. Tourtellotte WW: What is multiple sclerosis? Laboratory criteria for diagnosis , in Davison AN, Humphrey JH, Liversedge AL (eds): Multiple Sclerosis Research . New York, American Elsevier Publishing Co, 1975, pp 9-26. 7. Leinikki PO, Pässilä S: Quantitative, semiautomated, enzyme-linked immunosorbent assay for viral antibodies . J Infect Dis 1977;136 ( (suppl) ):294-299.Crossref 8. Iivanainen MV, Leinikki PO, Shekarchi IV, et al: Cerebrospinal fluid antibody pattern in multiple sclerosis by ELISA, abstracted . Ann Neurol 1979;6:155. 9. Link H: Immunoglobulin G and low molecular weight proteins in human cerebrospinal fluid: Chemical and immunological characterization with special reference to multiple sclerosis . Acta Neurol Scand 1967;43( (suppl 28) ):1-136. 10. Link H, Panelius M, Salmi AA: Immunoglobulins and measles antibodies in subacute sclerosing panencephalitis . Arch Neurol 1973;28:23-30.Crossref 11. Laterre EC, Callewaert A, Heremans JF, et al: Electrophoretic morphology of gamma globulins in cerebrospinal fluid of multiple sclerosis and other diseases of the nervous system . Neurology 1970;20:982-990.Crossref 12. Adornato BT, Houff HA, Engel WK, et al: Abnormal immunoglobulin bands in cerebrospinal fluid in myasthenia gravis . Lancet 1978;2:367-368.Crossref 13. Laurenzi MA, Link H: Comparison between agarose gel electrophoresis and isoelectric focusing of CSF for demonstration of oligoclonal immunoglobulin bands in neurological disorders . Acta Neurol Scand 1978;58:148-156.Crossref 14. Link H: Demonstration of oligoclonal immunoglobulin G in Guillain-Barre syndrome . Acta Neurol Scand 1975;52:111-120.Crossref 15. Vandvik B, Norrby E: Oligoclonal IgG antibody response in the central nervous system to different measles virus antigens in subacute sclerosing panencephalitis . Proc Natl Acad Sci USA 1973;70:1060-1063.Crossref 16. Vandvik B, Norrby E, Nordal HJ, et al: Oligoclonal measles virus-specific IgG antibodies isolated from cerebrospinal fluids, brain extracts and sera from patients with subacute sclerosing panencephalitis and multiple sclerosis . Scand J Immunol 1976;5:979-992.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

CSF Oligoclonal Bands, Immunoglobulins, and Viral Antibodies in Progressive Myoclonus Epilepsy

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References (19)

Publisher
American Medical Association
Copyright
Copyright © 1981 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1981.00510040032003
Publisher site
See Article on Publisher Site

Abstract

Abstract • We studied CSF and serum samples from 16 patients with progressive myoclonus epilepsy (PME). These patients had juvenile-onset PME with evidence of autosomal recessive inheritance and no Lafora bodies. Twelve of the 16 patients with PME had immunologic abnormalities. Oligoclonal γ bands were seen in six of the eight patients from whom sufficient CSF was available. The CSF albumin and serum/CSF albumin ratios were normal in all 16 patients, indicating the presence of intact blood-brain barriers. Six of the 16 patients showed increased CSF IgG levels and five had an increased CNS IgG synthesis. All patients had normal serum and CSF IgM and IgA levels. Three patients, all with bands, had reduced measles and/ or vaccinia serum/CSF antibody ratios. The findings suggest altered immune response of the CNS of some patients with PME apparently caused by nonspecific immunostimulation. References 1. Diebold K: Vier Erbtypen oder Krankheitsformen der progressiven Myoklonusepilepsien . Arch Psychiat Nervenkr 1972;215:362-375.Crossref 2. Koskiniemi M, Donner M, Majuri H, et al: Progressive myoclonus epilepsy: A clinical and histopathological study . Acta Neurol Scand 1974;50:307-332.Crossref 3. Taskinen E, Iivanainen M: Lymphoid cell reaction and immunoglobulins in cerebrospinal fluid in progressive myoclonus epilepsy, abstracted, in Abstracts of the 11th World Congress of Neurology, Excerpta Medica International Congress Series No. 427. Amsterdam, Excerpta Medica, 1977, No. 26, p 9. 4. Iivanainen M: New medical treatment of progressive myoclonus epilepsy, abstracted . Acta Neurol Scand 1976;54:98. 5. Johnson P, Arrigo SC, Nelson BJ, et al: Agarose electrophoresis of cerebrospinal fluid in multiple sclerosis: A simplified method for demonstrating cerebrospinal fluid oligoclonal immunoglobulin bands . Neurology 1977;27:273-277.Crossref 6. Tourtellotte WW: What is multiple sclerosis? Laboratory criteria for diagnosis , in Davison AN, Humphrey JH, Liversedge AL (eds): Multiple Sclerosis Research . New York, American Elsevier Publishing Co, 1975, pp 9-26. 7. Leinikki PO, Pässilä S: Quantitative, semiautomated, enzyme-linked immunosorbent assay for viral antibodies . J Infect Dis 1977;136 ( (suppl) ):294-299.Crossref 8. Iivanainen MV, Leinikki PO, Shekarchi IV, et al: Cerebrospinal fluid antibody pattern in multiple sclerosis by ELISA, abstracted . Ann Neurol 1979;6:155. 9. Link H: Immunoglobulin G and low molecular weight proteins in human cerebrospinal fluid: Chemical and immunological characterization with special reference to multiple sclerosis . Acta Neurol Scand 1967;43( (suppl 28) ):1-136. 10. Link H, Panelius M, Salmi AA: Immunoglobulins and measles antibodies in subacute sclerosing panencephalitis . Arch Neurol 1973;28:23-30.Crossref 11. Laterre EC, Callewaert A, Heremans JF, et al: Electrophoretic morphology of gamma globulins in cerebrospinal fluid of multiple sclerosis and other diseases of the nervous system . Neurology 1970;20:982-990.Crossref 12. Adornato BT, Houff HA, Engel WK, et al: Abnormal immunoglobulin bands in cerebrospinal fluid in myasthenia gravis . Lancet 1978;2:367-368.Crossref 13. Laurenzi MA, Link H: Comparison between agarose gel electrophoresis and isoelectric focusing of CSF for demonstration of oligoclonal immunoglobulin bands in neurological disorders . Acta Neurol Scand 1978;58:148-156.Crossref 14. Link H: Demonstration of oligoclonal immunoglobulin G in Guillain-Barre syndrome . Acta Neurol Scand 1975;52:111-120.Crossref 15. Vandvik B, Norrby E: Oligoclonal IgG antibody response in the central nervous system to different measles virus antigens in subacute sclerosing panencephalitis . Proc Natl Acad Sci USA 1973;70:1060-1063.Crossref 16. Vandvik B, Norrby E, Nordal HJ, et al: Oligoclonal measles virus-specific IgG antibodies isolated from cerebrospinal fluids, brain extracts and sera from patients with subacute sclerosing panencephalitis and multiple sclerosis . Scand J Immunol 1976;5:979-992.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Apr 1, 1981

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