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Syringomyelia and neuropathic pain, its treatment

The next patient is suffering from severe pain due to cervical syringomyelia. Here his story:

Syringomyelia is a pathological proces most often seen in the cervical part of the spinal cord, it causes neuropathic pain (neuropathy, neuritis, neuralgia) such as burning pain, tingling (paraesthesias), numbness, and muscle atrophy in the upper limbs.  

References on pain and syringomyelia

  1. Beric A, Dimitrijevic MR, Lindblom U: Central dysesthesia syndrome in spinal cord injury patients. Pain 34:109-116, 1988
  2. Charnay Y, Paulin C, Chayvialle JA, et al: Distribution of substance P-like immunoreactivity in the spinal cord and dorsal root ganglia of the human foetus and infant. Neuroscience 10: 41-55, 1983
  3. Chung K, Briner RP, Carlton SM, et al: Immunohistochemical localization of seven different peptides in the human spinal cord. J Comp Neurol 280:158-170, 1989
  4. Cooper PE, Fernstrom MH, Rorstad OP, et al: The regional distribution of somatostatin, substance P and neurotensin in human brain. Brain Res 218:219-232, 1981
  5. Das A, Puvanendran K: Syringomyelia and complex regional pain syndrome as complications of multiple sclerosis. ArchNeurol 56:1021-1024, 1999
  6. Davidoff G, Roth E, Guarracini M, et al: Function-limiting dysesthetic pain syndrome among traumatic spinal cord injury patients: a cross-sectional study. Pain 29:39-48, 1987
  7. de Lanerolle NC, LaMotte CC: The human spinal cord: substance P and methionine-enkephalin immunoreactivity. J Neurosci2:1369-1386, 1982
  8. Epstein FJ, Farmer JP, Freed D: Adult intramedullary spinal cord ependymomas: the result of surgery in 38 patients. J Neurosurg79:204-209, 1993
  9. Furst S: Transmitters involved in antinociception in the spinal cord. Brain Res Bull 48:129-141, 1999
  10. Gamache FW Jr, Ducker TB: Syringomyelia: a neurological and surgical spectrum. J Spinal Disord 3:293-298, 1990
  11. Gibson SJ, Polak JM, Katagiri T, et al: A comparison of the distributions of eight peptides in spinal cord from normal controls and cases of motor neurone disease with special reference to Onuf's nucleus. Brain Res 474:255-278, 1988
  12. Gillberg PG, Aquilonius SM, Eckernas SA, et al: Choline acetyltransferase and substance P-like immunoreactivity in the human spinal cord: changes in amyotrophic lateral sclerosis. Brain Res 250:394-397, 1982
  13. Harmar A, Keen P: Chemical characterization of substance P-like immunoreactivity in primary afferent neurones. Brain Res220:203-207, 1981
  14. Helme RD, White DM: Immunohistochemical localization of substance P in postmortem rat and human spinal cord. J NeuropatholExp Neurol 42:99-105, 1983
  15. Henry JL: Effects of substance P on functionally identified units in cat spinal cord. Brain Res 114:439-451, 1976
  16. Henry JL: Relation of substance P to pain transmission: neurophysiological evidence. Ciba Found Symp 206-224, 1982
  17. Hida K, Iwasaki Y, Koyanagi I, et al: Surgical indication and results of foramen magnum decompression versus syringosubarachnoid shunting for syringomyelia associated with Chiari I malformation. Neurosurgery 37:673-679, 1995
  18. Hokfelt T, Vincent S, Dalsgaard CJ, et al: Distribution of substance P in brain and periphery and its possible role as a cotransmitter. Ciba Found Symp 84-106, 1982
  19. Iversen LL, Hanley MR, Sandberg BE, et al: Substance P receptors in the nervous system and possible receptor subtypes. CibaFound Symp 186-205, 1982
  20. Jessell TM: Substance P in nociceptive sensory neurons. CibaFound Symp 225-248, 1982
  21. Konishi S, Tsunoo A, Yanaihara N, et al: Peptidergic excitatory and inhibitory synapses in mammalian sympathetic ganglia: roles of substance P and enkephalin. Biomed Res 1:528-536, 1980
  22. LaMotte CC, Shapiro CM: Ultrastructural localization of substance P, metenkephalin, and somatostatin immunoreactivity in lamina X of the primate spinal cord. J Comp Neurol 306: 290-306, 1991
  23. McCormick PC, Torres R, Post KD, et al: Intramedullary ependymoma of the spinal cord. J Neurosurg 72:523-532, 1990
  24. Milhorat TH, Chou MW, Trinidad EM, et al: Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005-1017, 1999
  25. Milhorat TH, Johnson RW, Milhorat RH, et al: Clinicopathological correlations in syringomyelia using axial magnetic resonance imaging. Neurosurgery 37:206-213, 1995
  26. Milhorat TH, Kotzen RM, Mu HT, et al: Dysesthetic pain in patients with syringomyelia. Neurosurgery 38:940-947, 1996
  27. Milhorat TH, Mu HT, LaMotte CC, et al: Distribution of substance P in the spinal cord of patients with syringomyelia. JNeurosurg 84:992-998, 1996
  28. Naftchi NE, Abrahams SJ, St. Paul HM, et al: Localization and changes of substance P in spinal cord of paraplegic cats. BrainRes 153:507-513, 1978
  29. Nomura H, Shiosaka S, Inagaki S, et al: Distribution of substance P-like immunoreactivity in the lower brainstem of the human fetus: an immunohistochemical study. Brain Res 252: 315-325, 1982
  30. Otsuka M, Konishi S, Yanagisawa M, et al: Role of substance P as a sensory transmitter in spinal cord and sympathetic ganglia. Ciba Found Symp 13-34, 1982
  31. Pearson J, Brandeis L, Cuello AC: Depletion of substance P-containing axons in substantia gelatinosa of patients with diminished pain sensitivity. Nature 295:61-63, 1982
  32. Perl ER: Causalgia, pathological pain, and adrenergic receptors. Proc Natl Acad Sci USA 96:7664-7667, 1999
  33. Schoenen J, Lotstra F, Vierendeels G, et al: Substance P, enkephalins, somatostatin, cholecystokinin, oxytocin, and vasopressin in human spinal cord. Neurology 35:881-890, 1985
  34. van Gijn J, Giesbergen PC, Hoogenraad TU: Attacks of pain in the leg from classic syringomyelia. J Neurol Neurosurg Psychiatry63:410, 1997 (Letter)
  35. Vita G, Haun CK, Hawkins EF, et al: Effects of experimental spinal cord transection on substance P receptors: a quantitative autoradiography study. Neuropeptides 17:147-153, 1990
  36. Williams B: Orthopaedic features in the presentation of syringomyelia. J Bone Joint Surg (Br) 61:314-323, 1979
  37. Yezierski RP: Pain following spinal cord injury: the clinical problem and experimental studies. Pain 68:185-194, 1996

March 2010, Prof. dr. J.M. Keppel Hesselink, MD, PhD, revision sept 2011

 
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