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Neurological complications and myelographic features of achondroplasia

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Summary

The neurological complications of achondroplasia are discussed based on the literature and two of our cases. Thoracolumbar kyphosis hypertrophic osteoarthritis, and intervertebral disc herniations may cause early neurological disturbances to adult achondroplasts owing to the spinal stenosis. The symptoms range from nerve root compression syndromes to complete transverse myelopathy. The radiographic features are characteristic, but the value of myelography for etiological clarification and location of the pathological process is limited. The significance of early symptoms and signs must be stressed and early decompression performed, as results are very disappointing once extensive neurological damage has occurred. An extensive laminectomy is usually indicated.

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References

  1. Albrecht, O., Ranzi, E.: Kompressionsmyelitis bei Chondrodystrophie. Wiener klin. Wschr.. 39, 1241 (1926)

    Google Scholar 

  2. Alexander, E.: Significance of the small lumbar spinal canal: Cauda equina compression syndromes due to spondylosis. 5. Achondroplasia. J. Neurosurg. 31, 513 (1969)

    Google Scholar 

  3. Ambrozy, G., Palvölgyi, R.: Neurologische Krankheitser-scheinungen bei Chondrodystrophie. Psychiatr. Neurol. Med. Psychol. (Leipzig,) 20, 354 (1969)

    Google Scholar 

  4. Bergstroem K., Laurent, U., Lundberg P. O.: Neurological symptoms in achondroplasia. Acta Neurol. Scand. 47, 59 (1971)

    Google Scholar 

  5. Blom, S., Ekbom, K. A.: Early clinical signs of meningiomas of the foramen magnum. J. Neurosurg. 19, 661 (1962)

    Google Scholar 

  6. Bösch, P., Reisner, T.: Das Querschnittssyndrom bei chondrodystrophischen Zwergen Z. Orthop. 114, 861 (1977)

    Google Scholar 

  7. Brish A., Lerner, M. A., Braham, J.: Intermittent claudication from compression of cauda equina by a narrowed spinal canal. J. Neurosurg 21, 207 (1964)

    Google Scholar 

  8. Caffey, J.: Achondroplasia of pelvis and lumbosacral spine. Amer. J. Roentgenol. 80, 449 (1958)

    Google Scholar 

  9. Clark, K.: Significance of the small lumbar spinal canal: Cauda equina compression syndromes due to spondylosis. 2. Clinical and surgical significance. J. Neurosurg. 31, 495 (1969)

    Google Scholar 

  10. Cressmann M. R., Pawl, R. P.: Serpentine myelographic defect caused by redudant nerve root. J. Neurosurg. 28, 391 (1968)

    Google Scholar 

  11. Cohen, M. E., Rosenthal, A. D., Matson, D. D.: Neurological abnormalities in achondroplastic children. J. Pediat. 71, 367 (1967)

    Google Scholar 

  12. Critchley E.: Neurological abnormalities in achondroplasia. Develop. Med. Child. Neurol. 10, 104 (1968)

    Google Scholar 

  13. Dennis, J. P., Rosenberg, H. S., Alvord, E. C.: Megalencephaly, internal hydrocephalus and other neurological aspects of achondroplasia. Brain 84, 427 (1961)

    Google Scholar 

  14. Donath, J. Vogl, A.: Untersuchungen, über den chondrodystrophischen Zwergwuchs. Z. ges. Neurol. Psychiatr. 111, 333 (1927)

    Google Scholar 

  15. Duvoisin, R. C., Yahr, L. D.: Compressive spinal cord and root syndromes in achondroplastic dwarfs. Neurology (Minn.) 12, 202 (1962)

    Google Scholar 

  16. Ehni, G.: Significance of the small lumbar spinal canal: Cauda equina compression syndromes due to spondylosis., J. Neurosurg. 31, 490 (1969)

    Google Scholar 

  17. Epstein, J. A., Malis, L. I.: Compression of spinal cord and cauda equina in achondroplastic dwarfs. Neurology (Minn.) 5, 875 (1955)

    Google Scholar 

  18. Epstein, B. S. Epstein, J. A., Jones, M. D.: Lumbar spinal stenosis. Radiol. Clin. North Amer. 15, 227 (1977)

    Google Scholar 

  19. Ferrari, G., Pellone, M.: Complicanze neurologiche dell'achondroplasia G. Psichiatr. Neuropatol. 95, 243 (1969)

    Google Scholar 

  20. Freund, E.: Spastic paraplegia in achondroplasia. Arch. Surg. 27, 859 (1933)

    Google Scholar 

  21. Gulati, D. R., Rout, D.: Atlantoaxial dislocation with quadriparesis in achondroplasia. J. Neurosurg. 40, 394 (1974)

    Google Scholar 

  22. Hancock, D. O., Philips, D. G.: Spinal compression in achondroplasia. Paraplegia 3, 22 (1966)

    Google Scholar 

  23. Jones, R. A. C.: The narrow lumbar canal. J. Bone Joint Surg. 50-B, 595 (1968)

    Google Scholar 

  24. Kissel, P., Hartemann, P., barrucand, D., Montaut, J. Compression medullaire et achondroplasie. Rev. neurol. 109, 489 (1963)

    Google Scholar 

  25. Kopits, S. E.: Orthopedic complications of dwarfism. Clin. Orthop 114, 153 (1976)

    Google Scholar 

  26. Langer, L. O., Baumann, P. A., Gorlin, R. J.: Achondroplasia. Am. J. Roentgenol. 100, 12 (1967)

    Google Scholar 

  27. Lutter, L. D., Langer, L. O.: Neurological symptoms in achondroplastic dwarfs. Surgical treatment. J. Bone Joint Surg. 59-A, 87 (1977)

    Google Scholar 

  28. Nelson, N. A.: Spinal stenosis in achondroplasia. Proc. Roy Soc. Med. 65, 1028 (1973)

    Google Scholar 

  29. Ozer, F. L.: Achondroplasia with spinal neurologic complications in mother and son. Birth Defects 10, 351 (1975)

    Google Scholar 

  30. Roberson, G. H., Llewellyn, H. J., Taveras, J. M.: The narrow lumbar spinal canal syndrome. Radiology 107, 89 (1973)

    Google Scholar 

  31. Roth, M., Krkoska, J., Toman, I.: Morphogenesis of the spinal canal, normal and stenotic. Neuroradiology 10, 277 (19676)

    Google Scholar 

  32. Sarnecka-Stefanowicz, D.: Inferior limb paraparesis in a cases of achondroplasia. Chir. Narzadow Ruchu Ortop. Poi. 42, 89 (1977)

    Google Scholar 

  33. Schreiber, F., Rosenthal, H.: Paraplegia from ruptured lumbar discs in achondroplastic dwarfs. J. Neurosurg. 9, 648 (1952)

    Google Scholar 

  34. Scott, C. I.: Achondroplastic and hypochondroplastic dwarfism. Clin. Orthop. 114, 18 (1976)

    Google Scholar 

  35. Sculpo, T. P., Levine, D. B.: Thoracolumbar kyphosis in achondroplasia with spinal cord compression. N. Y. State Med. J. 76, 426 (1976)

    Google Scholar 

  36. Stroobandt, G., Laterre, E. C., Vincent, A., Cornelis, G.: Compression radiculo-médullaire d'origine rachidienne chez une achondroplase. Neurochirurgie 16, 295 (1971)

    Google Scholar 

  37. Verbiest, H.: Further experiences on the pathological influence of a developmental narrowness of the bony lumbar vertebral canal J. Bone Joint Surg. 37-B, 576 (1955)

    Google Scholar 

  38. Vogl, A., Osborne, R. L.: Lesions of the spinal cord (transverse myelopathy) in achondroplasia. Arch. Neurol. Psychiatr. 61, 644 (1949)

    Google Scholar 

  39. Weber, G.: Rückenmarkskompression bei Chondrodystrophie. Schweiz. Arch. Neurol. Psychiatr. 71, 291 (1953)

    Google Scholar 

  40. Wilson, C. B.: Significance of the small lumbar spinal canal: Cauda equina compression syndromes due to spondylosis. 3. Intermittent claudication. J. Neurosurg. 31, 499 (1969)

    Google Scholar 

  41. Yang, S. S., Corbett, D. P., Brough, A. J., Heidelberger, K. P.: Upper cervical myelopathy in achondroplasia. Am. J. Clin. Pathol. 68, 68 (1977)

    Google Scholar 

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Galanski, M., Herrmann, R. & Knoche, U. Neurological complications and myelographic features of achondroplasia. Neuroradiology 17, 59–63 (1978). https://doi.org/10.1007/BF00345272

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  • DOI: https://doi.org/10.1007/BF00345272

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