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Intravenous immunoglobulin therapy in amyotrophic lateral sclerosis

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Abstract

Seven consecutive patients with amyotrophic lateral sclerosis (ALS) were treated with intravenous immunoglobulins (IVIg; 0.4 g/kg per day for 5 consecutive days followed by monthly 2-day infusions at the same daily dosage) continued with oral cyclophosphamide (1–2 mg/kg per day), for 4–13 months (mean 8.1). Response to treatment was assessed by means of the Medical Research Council (MRC) rating scale for muscle strength on 40 muscles (10 per limb), a clinical scale for bulbar function and a modified Rankin disability scale. All patients continued to deteriorate during treatment on as regards both their MRC score and either their bulbar or Rankin score or both. The progression of the disease during treatment, expressed as the monthly variation in MRC score (mean=−2.71; SD=1.36), was no slower than that estimated before therapy (mean=−1.81; SD=0.93). Even if the results of this small, uncontrolled study do not permit the exclusion of an effect of IVIg on the progression of ALS, they also do not provide any evidence that this expensive form of therapy consistently slows the course of the disease.

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References

  1. Appel SH, Smith RG, Engelhardt JI, Stefani E (1993) Evidence of autoimmunity in amyotrophic lateral sclerosis. J Neurol Sci 118:169–174

    Article  PubMed  CAS  Google Scholar 

  2. Azulay JP, Blin O, Pouget J, Boucraut J, Bille’-Turc F, Carles G, Serratrice G (1994) Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double blind,placebo controlled study. Neurology 44: 429–432

    PubMed  CAS  Google Scholar 

  3. Dalakas M, Stein DP, Otero C, Sekul E, Cupler EJ, McCrosky S (1994) Effect of high-dose intravenous immunoglobulin on amyotrophic lateral sclerosis and multifocal motor neuropathy. Arch Neurol 51:861–864

    PubMed  CAS  Google Scholar 

  4. Drachman DB, Chaudry V, Cornblath DR, Kuncl RW, Pestronk A, Clawson L, Mellits ED, Quaskey S, Quinn T, Calkins A, Order S (1994) Trial of immunosuppression in amyotrophic lateral sclerosis using total lymphoid irradiation. Ann Neurol 35:142–150.

    Article  PubMed  CAS  Google Scholar 

  5. Engel KW, Hanna CJ (1992) Intravenous immunoglobulin: excellent benefit in otherwise refractory progressive muscular atrophy with IgM monoclonal gammopathy (abstract). Ann Neurol 32:279

    Google Scholar 

  6. European ALS Collaborative Group (1992) Criteria for diagnosis of familial ALS. Neuromusc Dis 2:7–9

    Article  Google Scholar 

  7. Kinsella LJ, Lange DJ, Trojaburg W, Sadiq SA, Younger DS, Latov N (1994) Clinical and electrophysiological correlates of elevated anti-GM1 antibody titers. Neurology 44:1278–1282

    PubMed  CAS  Google Scholar 

  8. Latov N, Hays AP, Donofrio PD, Liao J, Ito H, McGinnis S, Manoussos K, Freddo L, Shy ME, Sherman WH, Chang HW, Greenberg HS, Albers JW Alessi AG, Keren D, Yu RK, Rowland LP, Kabat EA (1988) Monoclonal IgM with unique specificity to gangliosides GM1 and GD1b and to lacto-N-tetraose associated with human motor neuron disease. Neurology 38: 763–768

    PubMed  CAS  Google Scholar 

  9. Mitsumoto H, Hanson MR, Chad DA (1988) Amyotrophic lateral sclerosis: recent advances in pathogenesis and therapeutic trials. Arch Neurol 45: 189–202

    PubMed  CAS  Google Scholar 

  10. Mitsumoto H, Kumar S, Levin KH, Shields RW, Secic M, Wilbourn AJ, Desai RG (1992) Intravenous immunoglobulin treatment in amyotrophic lateral sclerosis (abstract). Ann Neurol 32:252

    Google Scholar 

  11. Nobile-Orazio E, Meucci N, Barbieri S, Carpo M, Scarlato G (1993) High dose intravenous immunoglobulin therapy in multifocal motor neuropathy. Neurology 43:537–544

    PubMed  CAS  Google Scholar 

  12. Pestronk A (1991) Motor neuropathies, motor neuron disorders and antiglycolipid antibodies. Muscle Nerve 14: 927–936

    Article  PubMed  CAS  Google Scholar 

  13. Pestronk A, Lopate G, Kornberg AJ, Elliot JL, Blume G, Yee W-C, Goodnough LT (1994) Distal lower motor neuron syndrome with high titer serum IgM anti-GM1 antibodies: improvement following immunotherapy with monthly plasma exchange and intravenous cyclophosphamide. Neurology 44:2027–2031

    PubMed  CAS  Google Scholar 

  14. Rowland LP (1991) Ten central themes in a decade of ALS research. In: Rowland LP (ed) Advances in neurology, vol 56. Amyotrophic lateral scerosis and other motor neuron diseases. Raven Press, New York, pp 3–23

    Google Scholar 

  15. Shy ME, Heiman-Patterson T, Parry GJ, Tahmoush A, Evans VA, Schick PK (1990) Lower motor neuron disease in a patient with autoantibodies against GaP(β1–3)GalNAc in gangliosides GM1 and GD1b: improvement following immunotherapy. Neurology 40: 842–844

    PubMed  CAS  Google Scholar 

  16. Tsai C-P, Lin KP, Liao K-K, Wang S-J, Wang V, Kao K-P, Wu Z-A (1993) Immunosuppressive treatment in lower motor neuron syndrome with autoantibodies against GM1 gangliosides. Eur Neurol 33:446–449

    PubMed  CAS  Google Scholar 

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Meucci, N., Nobile-Orazio, E. Intravenous immunoglobulin therapy in amyotrophic lateral sclerosis. J Neurol 243, 117–120 (1996). https://doi.org/10.1007/BF02444000

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  • DOI: https://doi.org/10.1007/BF02444000

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