Motorneuron Sprouting and Spinal Plasticity in Amyotrophic Lateral Sclerosis: The “Window of Opportunity” for a Ganglioside Treatment

  • P. Pinelli
  • C. Pasetti
  • L. Mazzini
  • F. Pisano
  • A. Villani
Part of the FIDIA Research Series book series (FIDIA, volume 6)


Amyotrophic Lateral Sclerosis (ALS) is a disease of unknown pathogenesis affecting the first and the second motorneuron with a progressive course which leads to the death of the patient within a very few years (Rowland, 1982). Abnormal ganglioside composition has been found in spinal cord by Dawson et al., (1986) who have hypothesized that in ALS abnormal gangliosides build up in motorneurons and lead to their eventual degeneration. On the other hand, a biochemical abnormality occurring early in the disease is reflected in the impairment of thiamine phosphorylation, detected at the level of cerebral spinal fluid (Poloni et al. 1982; 1983); this finding seems to parallel a lowered energetic metabolism of the motorneurons in the earliest phase of the pathogenetic process.


Amyotrophic Lateral Sclerosis Motor Unit Amyotrophic Lateral Sclerosis Patient Cerebral Spinal Fluid Motor Neuron Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



amyotrophic lateral sclerosis




bovina brain gangliosides


motor units.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Borenstein S, Desmedt JE (1973) Electromyographical signs of collateral reinnervation. In: Desmedt JE (ed): New developments in electromyography and clinical neurophysiology. Brussels, vol. 1, pp. 130–140.Google Scholar
  2. Bradley WG, Hedlund W, Cooper C, Desousa GJ, Gabbai A, Mora JS, Munsat TL, Scheife R (1984) A double-blind controlled trial of bovine brain gangliosides in ALS. Neurology 34: 1079–1082.PubMedCrossRefGoogle Scholar
  3. Buchthal F (1957) An introduction to electromyography. Scandinavian University Books, Copenhagen.Google Scholar
  4. Dawson G, Hancock LW, Horwitz AL, Stefansson L, Antel J (1986) Possible association of degenerative motor neuron disease (ALS) with abnormal ganglioside metabolism. This volume.Google Scholar
  5. Dunbar GL, Butler W, Fass B, Stein DG (1985) Gangliosides enhance behavioral recovery and neuronal repair in brain damaged rats. This volume.Google Scholar
  6. Fuxe K, Agnati LF, Benfenati F, Zoli M, Diemer NH, Owman C, Toffano G, Ruggeri M, Jansson AM, Goldstein M (1966) Effects of ganglioside GM1 treatment on lesioned nigral dopamine neurons and on striatal energy metabolism, striatal blood flow and cyclic AMP and Ca+ + induced protein phosphorylation. This volume.Google Scholar
  7. Gorio A, Carmignoto G, Ferrari G (1981) Axon sprouting stimulated by gangliosides: a new model for elongation and sprouting. In: Rapport MM, Gorio A (eds): Gangliosides in neurological and neuromuscular function, development and repair. Raven Press, New York, pp. 177–195.Google Scholar
  8. Gurney M (1985) Initial steps toward a molecular biology of ALS. In: Kato (ed): Proceedings of ALS International Congress: Therapeutic Psychological and Research Aspects of ALS. Varese, Italy, March 27–31.Google Scholar
  9. Pinelli P, Mazzini L, Villani A (1985) A follow-up electromyographic investigations of ALS patients treated with high dosage gangliosides. In: Kato (ed): Proceedings of ALS International Congress: Therapeutic Psychological and Research Aspect of ALS. Varese, Italy, March 27–31.Google Scholar
  10. Poloni M, Rocchelli B, Patrini C, Rindi G (1982) Thiamin monophosphate in patients with ALS. Arch Neurol 39: 507.PubMedCrossRefGoogle Scholar
  11. Poloni M, Patrini C, Pinelli P (1983) Cerebrospinal fluid, plasma and erythrocytes thiamine content in ALS. A.L.S. Workshop, Munich, October 14–15.Google Scholar
  12. Rowland LP (1982) Diverse forms of motor neuron disease. In: Rowland LP (ed): Advances in Neurology. Human Motor Neuron Disease, pp. 1–13.Google Scholar
  13. Stalberg E, Ekstedt J (1973) Single fiber EMG and microphysiology of the motor unit in normal and diseased human muscle. In: Desmedt JE (ed): New developments in electromyography and clinical neurophysiology. Brussels, vol. 1, pp. 113–129.Google Scholar
  14. Swash M, Schwarts MS (1983) Staging motor neuron disease: single fiber EMG studies of assymetry, progression and compensatory reinnervation. In: Clifford Rose F (ed): Research progress in motor neuron disease. Pittman.Google Scholar
  15. Varon S, Skaper SD, Katoh-Semba R (1986) Neuritic responses to GM1 ganglioside in several in vitro systems. This volume.Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1986

Authors and Affiliations

  • P. Pinelli
    • 1
  • C. Pasetti
    • 2
  • L. Mazzini
    • 2
  • F. Pisano
    • 2
  • A. Villani
    • 2
  1. 1.Neurological ClinicUniversity of MilanMilanItaly
  2. 2.Dept. of NeurologyMedical Center of RehabilitationVeruno, NovaraItaly

Personalised recommendations