Acta Neuropathologica

, Volume 71, Issue 3–4, pp 267–277 | Cite as

Tremors in Samoyed pups with oligodendrocyte deficiencies and hypomyelination

  • J. F. Cummings
  • B. A. Summers
  • A. de Lahunta
  • C. Lawson
Original Works


Over a 3-year period a breeder of Samoyed dogs observed six litters in which one or more pups at 3 weeks of age showed generalized tremors and an inability to stand. One affected pup, a 5-week-old male, was referred for clinical and pathological evaluation. Gross and microscopic studies including immunocytochemical demonstration of myelin basic protein, revealed a lack of myelin throughout the central nervous system. Ultrastructural studies revealed that a total absence of normal myelin was associated with oligodendroglial deficiencies. Oligodendrocytes were immature in appearance, greatly reduced in number, and incapable of forming compact myelin. Astrocytosis and an increase in the third type of neuroglial cells were observed. The changes differed from those reported in other canine forms of hypomyelinogenesis and were compared with hypomyelinating diseases in other species. The findings suggested that this hypomyelination was the result of retarded gliogenesis.

Key words

Comparative neuropathology Hypomyelination Oligodendroglia Type III neuroglia 


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  1. Barlow RM, Gardiner AC (1969) Experiments in Border disease. I. Transmission, pathology, and some serological aspects of the experimental disease. J Comp Pathol 79:397–405Google Scholar
  2. Barlow RM, Storey IJ (1977a) Myelination of the ovine CNS with special reference to Border disease. I. Qualitative aspects. Neuropathol Appl Neurobiol 3:237–253Google Scholar
  3. Barlow RM, Storey IJ (1977b) Myelination of the ovine CNS with special reference to Border disease. II. Quantitative aspects. Neuropathol Appl Neurobiol 3:255–265Google Scholar
  4. Blakemore WF, Harding JDJ (1974) Ultrastructural observation of the spinal cords of piglets affected with congenital tremor type AIV. Res Vet Sci 17:248–255Google Scholar
  5. Blakemore WF, Harding JDJ, Done JT (1974) Ultrastructural observations on the spinal cord of a Landrace pig with congenital tremor type A III. Res Vet Sci 17:174–178Google Scholar
  6. Bray GM, Duncan ID, Griffiths IR (1983) Shaking pups: a disorder of central myelination in the spaniel dog. IV. Freeze-fracture electron microscope studies of axons, oligodendrocytes and astrocytes in the spinal cord white matter. Neuropathol Appl Neurobiol 9:369–378Google Scholar
  7. Csiza CK, de Lahunta A (1979) Myelin deficiency (md), a neurologic mutant in the Wistar rat. Am J Pathol 95:215–219Google Scholar
  8. de Lahunta A (1983) Veterinary neuroanatomy and clinical neurology, 2nd edn. Saunders, Philadelphia, pp 149–150Google Scholar
  9. Dentinger MP, Barron KD, Csiza CK (1982) Ultrastructure of the central nervous system in a myelin deficient rat. J Neurocytol 11:671–691Google Scholar
  10. Dentinger MP, Barron KD, Csiza CK (1985) Glial and axonal development in optic nerve of myelin deficient rat mutant. Brain Res 344:255–266Google Scholar
  11. Done JT (1968) Congenital nervous diseases of pigs. A review. Lab Anim 2:207–217Google Scholar
  12. Duncan ID, Griffiths IR, Munz M (1983) Shaking pups: a disorder of central myelination in the spaniel dog. III. Quantitative aspects of glia and myelin in the spinal cord and optic nerve. Neuropathol Appl Neurobiol 9:355–368Google Scholar
  13. Greene CE, Vandevelde M, Hoff EJ (1977) Congenital cerebrospinal hypomyelinogenesis in a pup. J Am Vet Med Assoc 171:534–536Google Scholar
  14. Griffiths IR, Duncan ID, McCulloch M, Harvey MJA (1981) Shaking pups: a disorder of central myelination in the spaniel dog. Part 1. Clinical, genetic, light microscopical observations. J Neurol Sci 50:423–433Google Scholar
  15. Harding JDJ, Done JT, Harbourne JF, Randall CJ, Gilbert FR (1973) Congenital tremor type A III in pigs: an hereditary sex-linked cerebrospinal hypomyelinogenesis. Vet Rec 92:527–529Google Scholar
  16. Kunishita T, Tabera R, Umezawa H, Mizutani M, Katsuie Y (1986) A new myelin-deficient mutant hamster: biochemical and morphological studies. J Neurochem 4:105–111Google Scholar
  17. Mason RW, Hartley WJ, Randall M (1979) Spongiform degeneration of the white matter in a Samoyed pup. Aust Vet Practitioner 9:11–13Google Scholar
  18. Mayhew IG, Blakemore WF, Palmer AC, Clarke CJ (1984) Tremor syndrome and hypomyelination in lurcher pups. J Small Anim Pract 25:551–559Google Scholar
  19. Meier C, Bischoff A (1974) Dysmyelination in jimpy mouse: electron microscopic study. J Neuropathol Exp Neurol 33:343–353Google Scholar
  20. Meier C, Bischoff A (1975) Oligodendroglia cell development in jimpy mice and controls. J Neurol Sci 6:517–528Google Scholar
  21. Mori S, Leblond CP (1969) Identification of microglia in light and electron microscopy. J Comp Neurol 135:57–80Google Scholar
  22. Mori S, Leblond CP (1970) Electron microscopic identification of three classes of oligodendrocytes and a preliminary study of their proliferative activity in the corpus callosum of young rats. J Comp Neurol 139:1–30Google Scholar
  23. Peters A, Palay SL, Webster HF (1976) The fine structure of the nervous system: the neurons and supporting cells. Saunders, Philadelphia, pp 254–263Google Scholar
  24. Phillips DE (1973) An electron microscopic study of macroglia and microglia in the lateral funiculus of the developing spinal cord in the fetal monkey. Z Zellforsch Mikrosk Anat 140:145–167Google Scholar
  25. Privat A, Robain O, Mandel P (1972) Aspects ultrastructuraux du corps calleux chez la soures jimpy. Acta Neuropathol (Berl) 21:282–295Google Scholar
  26. Raff MC, Miller RH, Noble M (1983) A glial progenitor cell that develops in vitro into an astrocyte or an oligodendrocyte depending on culture medium. Nature 303:390–396Google Scholar
  27. Schelper RL, Adrian EK (1986) Monocytes become macrophages: they do not become microglia. A light and electron microscopic autoradiographic study using 12-iododeoxy-uridine. J Neuropathol Exp Neurol 45:1–19Google Scholar
  28. Sidman RL, Dickie MM, Appel SH (1979) Mutant mice (quaking and jimpy) with deficient myelination in the central nervous system. Science 144:309–311Google Scholar
  29. Skoff RP (1976) Myelin deficit in the jimpy mouse may be due to cellular abnormalities in astroglia. Nature 264:560–562Google Scholar
  30. Skoff RP (1982) Increased proliferation of oligodendrocytes in the hypomyelinated mouse mutant — jimpy. Brain Res 248:19–31Google Scholar
  31. Skoff RP, Price DL, Stocks A (1976) Electron microscopic autoradiographic studies of gliogenesis in rat optic nerve. II. Time of origin. J Comp Neurol 169:313–334Google Scholar
  32. Sternberger LA (1979) Immunocytochemistry, 2nd edn. Wiley, New York, pp 104–169Google Scholar
  33. Vandevelde M, Braund KG, Walker TL, Kornegay JN (1978) Dysmyelination of the central nervous system in the Chow-Chow dog. Acta Neuropathol (Berl) 42:211–215Google Scholar
  34. Vaughn JE (1969) An electron microscopic analysis of gliogenesis in rat optic nerves. Z Zellforsch Mikrosk Anat 94:293–324Google Scholar
  35. Vaughn JE, Pease DC (1970) Electron microscopic studies of Wallerian degeneration in rat optic nerves. II. Astrocytes, oligodendrocytes and adventitial cells. J Comp Neurol 140:207–226Google Scholar
  36. Vaughn JE, Peters A (1967) Electron microscopy of the early postnatal development of fibrous astrocytes. Am J Anat 121:131–152Google Scholar
  37. Vaughn JE, Peters A (1986) A third neuroglial cell type. An electron microscope study. J Comp Neurol 133:269–288Google Scholar
  38. Vaughn JE, Hinds PL, Skoff RP (1970) Electron microscopic studies of Wallerian degeneration in rat optic nerve. I. The multipotential glia. J Comp Neurol 140:175–206Google Scholar

Copyright information

© Springer-Verlag 1986

Authors and Affiliations

  • J. F. Cummings
    • 1
  • B. A. Summers
    • 2
  • A. de Lahunta
    • 1
  • C. Lawson
    • 3
  1. 1.Department of Anatomy, New York State College of Veterinary MedicineCornell UniversityIthacaUSA
  2. 2.Department of Pathology, New York State College of Veterinary MedicineCornell UniversityIthacaUSA
  3. 3.Derry Animal HospitalDerryUSA

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