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Focal spinal muscular atrophy in two German Shepherd pups

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Summary

Focal spinal muscular atrophy evolved rapidly in two German Shepherd pups 2 weeks after birth. By 1 month, the male and female littermates had developed a valgus deformity of the right forelimb, a flexed carpus, and arm and forearm muscle weakness and atrophy. In the male, less severe left forelimb weakness and wasting also occured, and bilateral forelimb denervation potentials were recorded. Postmortem study of the male revealed asymmetric loss and degeneration of motoneurons in the cervical spinal cord intumescence. Degenerating neurons appeared vacuolated or chromatolytic. Chromatolysis was often peripheral and resulted from dispersion and loss of the free and attached ribosomes which normally from Nissl bodies. Although this focal neuronopathy was unlike motoneuron diseases described previously in animals, it resembled the asymmetric and unilateral, benign spinal muscular atrophies found in humans.

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References

  1. Adornato BT, Engel WK, Kucera J, Bertorini TE (1978) Benign focal amyotrophy. Neurology 28:399

    Google Scholar 

  2. Cork LC, Griffin JW, Munnell JF, Lorenz M Adams RJ, Price DL (1979) Hereditary canine spinal muscular atrophy. J Neuropathol Exp Neurol 38:209–221

    Google Scholar 

  3. Cork LC, Griffin JW, Choy C, Pedula CA, Price DL (1982) Pathology of motor neurons in accelerated hereditary canine spinal muscular atrophy. Lab Invest 46:89–99

    Google Scholar 

  4. Cummings JF, de Lahunta A, Moore JJ III (1988) Multisystemic chromatolytic neuronal degeneration in a cairn terrier pup. Cornell Vet 78:301–314

    Google Scholar 

  5. de Lahunta A, Shively JN (1975) Neurofibrillary accumulation in a puppy. Cornell Vet 65:240–247

    Google Scholar 

  6. de Visser M, de Visser BWO, Verbeeten B Jr (1988) Electromyographic and computed tomographic findings in five patients with monomelic spinal muscular atrophy. Eur Neurol 28:135–138

    Google Scholar 

  7. Gourie-Devi M, Suresh TG, Shankar SK (1984) Monomelic amyotrophy. Arch Neurol 41:388–394

    Google Scholar 

  8. Harding AE, Bradbury PG, Murray NMF (1983) Chronic asymmetrical spinal muscular atrophy. J Neurol Sci 59:69–83

    Google Scholar 

  9. Higgins RJ, Vandevelde M, Hoff EJ, Jagar JE, Cook LC, Silberman MS (1977) Neurofibrillary accumulation in the zebra (Equus burchelli). Acta Neuropathol (Berl) 37:1–5

    Google Scholar 

  10. Higgins RJ, Rings DM, Fenner WR, Stevenson S (1983) Spontaneous lower motor neuron disease with neurofibrillary accumulation in young pigs. Acta Neuropathol (Berl) 59:288–294

    Google Scholar 

  11. Hirayama K, Tsubaki T, Toyokura Y, Okinata S (1963) Juvenile muscular atrophy of unilateral upper extremity. Neurology 13:373–380

    Google Scholar 

  12. Inada S, Sakamoto H, Haruta H, Miyazona Y, Sasaki H (1978) A clinical study on hereditary progressive muscular atrophy in Pointer dogs. Jpn J Vet Sci 40:539–547

    Google Scholar 

  13. Inada S, Yamauchi C, Igata A, Osame M, Izumo S (1986) Canine storage disease characterized by hereditary progressive neurogenic muscular atrophy: breeding experiments and clinical manifestation. Am J Vet Res 47:2294–2299

    Google Scholar 

  14. Izumo S, Ikuta F, Igata A, Osame M, Yamauchi C, Inada S (1983) Morphological study on the hereditary neurogenic amyotrophic dogs: accumulation of lipid compound-like structures in the lower motor neuron. Acta Neuropathol (Berl) 61:270–274

    Google Scholar 

  15. O'Sullivan DJ, McLeod JG (1978) Distal chronic spinal muscular atrophy involving the hands. J Neurol Neurosurg Psychiatry 41:653–658

    Google Scholar 

  16. Palmer AC, Blakemore WF (1989) A progressive neuronopathy in the young cairn terrier. J Small Anim Pract 30:101–106

    Google Scholar 

  17. Riggs JE, Schochet SS Jr, Gutmann L (1984) Benign focal amyotrophy: variant of chronic spinal muscular atrophy. Arch Neurol 41:678–679

    Google Scholar 

  18. Rousseaux CG, Klavano GG, Johnson ES, Shnitka TK, Harries WN, Snyder FF (1985) “Shaker” calf syndrome. A newly recognized inherited neurodegenerative disorder of horned Hereford calves. Vet Pathol 22:104–111

    Google Scholar 

  19. Sandefeldt EV, Cummings JF, de Lahunta A, Bjock G, Kook L (1973) Hereditary neuronal abiotrophy in the Swedish Lapland dog. Cornell Vet [Suppl 3] 63:1–71

    Google Scholar 

  20. Shell LG, Jortner BS, Leib MS (1987) Familial motor neuron disease in Rottweiler dogs. Vet Pathol 24:135–139

    Google Scholar 

  21. Shields RP, Vandevelde M (1978) Spontaneous lower motor neuron disease in rabbits (Oryctolagus cuniculus). Acta Neuropathol (Berl) 44:85–90

    Google Scholar 

  22. Singh N, Sachdiv KK, Susheela AK (1980) Juvenile muscular atrophy localized to arms. Arch Neurol 37:297–299

    Google Scholar 

  23. Sobue I, Sarta N, Iida M, Ando K (1978) Juvenile type of distal and segmental muscular atrophy of upper extremities. Ann Neurol 3:429–432

    Google Scholar 

  24. Tan CT (1985) Juvenile muscular atrophy of distal upper extremities. J Neurol Neurosurg Psychiatry 48:285–286

    Google Scholar 

  25. Vandevelde M, Greene CE, Hoff EJ (1976) Lower motor neuron disease with accumulation of neurofilaments in a cat. Vet Pathol 13:428–435

    Google Scholar 

  26. Virmani V, Mohan PK (1985) Non-familial, spinal segmental muscular atrophy in juvenile and young subjects. Acta Neurol Scand 72:336–340

    Google Scholar 

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Supported by a grant from Zypporah S. Fleisher for Canine Neurological Research

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Cummings, J.F., George, C., de Lahunta, A. et al. Focal spinal muscular atrophy in two German Shepherd pups. Acta Neuropathol 79, 113–116 (1989). https://doi.org/10.1007/BF00308967

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  • DOI: https://doi.org/10.1007/BF00308967

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