Does the absence of clinical expression of choreoathetosis, despite severe striatal atrophy, correlate with plasticity of neuropeptide synthesis?

  • S. N. Schiffmann
  • J.-J. Vanderhaeghen
Part of the Journal of Neural Transmission book series (NEURAL SUPPL, volume 33)


Neuropeptide and neurotransmitter plasticity has been demonstrated in the central nervous system. Modifications of their synthesis occur following receptor blockade or deafferentiation by surgical lesions. This concept should provid answers to some remaining open questions in human pathology especially in degenerative diseases of the basal ganglia. In a severely atrophied striatum we observed a selective increase in the number of detectable striatal substance P and met-enkephalin neurones which exhibited a striking increase in the intensity of labelling. This increase, instead of the well established reduction of substance P and enkephalins in the atrophied striatum of Huntington’s disease, could explain the absence of choreoathetosis which was replaced by rigidity and bradykinesia in the patient. The absence of choreoathetosis, despite severe striatal atrophy, is described in several basal ganglia diseases and could also be related to neurotransmitter or neuropeptide plasticity rather than due to the primary lesion.


Basal Ganglion Disease Dopamine Antagonist Haloperidol Thin Nerve Fibre Basal Ganglion Degeneration Tamic Acid Decarboxylase 
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  1. Adams RD, Van Bogaert L, Vendereecken H (1961) Dégénérescences nigro-striées et cérebellonigro-striées. Psychiat Neurol 142: 219–259CrossRefGoogle Scholar
  2. Aronin N, Cooper PE, Lorenz LJ, Bird ED, Sagar SM, Leeman SE, Martin JB (1983) Somatostatin is increased in the basal ganglia in Huntington’s disease. Ann Neurol 13: 519–526PubMedCrossRefGoogle Scholar
  3. Bannon MJ, Lee J-M, Giraud P, Young A, Affloter H-U, Bonner TI (1986) Dopamine antagonist haloperidol decreases substance P, substance K and preprotachykinin mRNAs in rat striatonigral neurons. J Biol Chem 261: 6640–6642PubMedGoogle Scholar
  4. Bird ED, Iversen LL (1974) Hungtington’s chorea: post-mortem measurement of glutamic acid decarboxylase, choline acetyltransferase and dopamine in basal ganglia. Brain 97: 457–472PubMedCrossRefGoogle Scholar
  5. Bird MT, Pauslon GW (1971) The rigid form of Huntington’s chorea. Neurology 21: 271–276PubMedGoogle Scholar
  6. Bruyn GW, Went LN (1986) Huntington’s chorea. In: Vincken PJ, Bruyn GW, Klawans HL (eds) Handbook of clinical neurology, vol 5. Extra-pyramidal disorders. Elsevier, Amsterdam, pp 267–313Google Scholar
  7. Dawbarn D, De Quidt ME, Emson PC (1985) Survival of basal ganglia neuropeptide Y-somatostain neurones in Huntington’s disease. Brain Res 340: 251–260PubMedCrossRefGoogle Scholar
  8. Emson PC, Arregui A, Clement-Jones V, Sandberg BEB, Rossor M (1980) Regional distribution of methionine-enkephalin and substance P-like immunoreactivity in normal human brain and in Huntington’s disease. Brain Res 199: 147–160PubMedCrossRefGoogle Scholar
  9. Ferrante RJ, Beal MF, Kowa11 NW, Richardson EP Jr, Martin JB (1987a) Sparing of acetylcholinesterase-containing neurons in Huntington’s disease. Brain Res 411: 162–166PubMedCrossRefGoogle Scholar
  10. Ferrante RJ, Kowa11 NW, Richardson EP Jr, Bird ED, Martin JB (1987b) Topography of enkephalin, substance P and acetylcholinesterase staining in Huntington’s disease striatum. Neurosci Lett 71: 283–288CrossRefGoogle Scholar
  11. Greenfield J, Wolfsohn J (1922) The pathology of Syndenham’s chorea. Lancet II: 603–606CrossRefGoogle Scholar
  12. Gusella JF, Wexler NS, Conneally PM, Naylor SL, Anderson MA, Tanzi RE, Watkins PC, Ottina K, Wallace MR, Sakaguchi AY, Young AB, Shoulson I, Bonnilla E, Martin JB (1983) A polymorphic DNA marker genetically linked to Huntington’s disease. Nature 306: 234–238PubMedCrossRefGoogle Scholar
  13. Normand E, Popovici T, Onteniente B, Fellman D, Piatier-Tonneau D, Auffray C, Bloch B (1988) Dopaminergic neurons of the substantia nigra modulate preproenkephalin A gene expression in rat striatal neurons. Brain Res 439: 39–46PubMedCrossRefGoogle Scholar
  14. Peters ACB, Vielvoye GJ, Versteeg J, Bots GT, Lindeman J (1979) ECHO 25 focal encephalitis and subacute hemichorea. Neurology 29: 678–681Google Scholar
  15. Roos RAC (1986) Neuropathology of Huntington’s chorea. In: Vincken PJ, Bruyn GW, Klawans HL (eds) Handbook of clinical neurology, vol 5. Extrapyramidal disorders. Elsevier, Amsterdam, pp 315–326Google Scholar
  16. Schiffmann SN, Vanderhaeghen J-J (1989) Increase of substance P and met-enkephalin in a severely atrophied striatum without clinical expression of chorea. Neurochem Int 14: 175–183PubMedCrossRefGoogle Scholar
  17. Spokes EGS (1980) Neurochemical alterations in Huntington’s chorea. A study of postmortem brain tissue. Brain 103: 179–210PubMedCrossRefGoogle Scholar
  18. Wilson SAK (1912) Progressive lenticular degeneration: a familial nervous system disease associated with cirrhosis of the liver. Brain 34: 295–507CrossRefGoogle Scholar
  19. Wolff G, Deuschl G, Wienker TF, Hummel K, Bender K, Lucking CH, Schumacher M, Hammer J, Oepen G (1989) New mutation to Huntington’s disease. J Med Genet 26: 18–27PubMedCrossRefGoogle Scholar
  20. Zweig RM, Koven SJ, Hedreen JC, Maestri NE, Kazazian Jr HH, Folstein SE (1989) Linkage to the Huntington’s disease locus in a fanily with unusual clinical and pathological features. Ann Neurol 26: 78–84PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • S. N. Schiffmann
    • 2
  • J.-J. Vanderhaeghen
    • 1
  1. 1.Laboratory of Neuropathology and Neuropeptide Research, Faculty of MedicineErasme and Brugmann Hospitals, Université Libre de BruxellesBrusselsBelgium
  2. 2.Laboratory of Neuropathology and Neuropeptide ResearchUniversité Libre de Bruxelles, Campus ErasmeBrusselsBelgium

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