Skip to main content

Advertisement

SpringerLink
Log in

Acute brainstem dissection of syringomyelia associated with cervical intramedullary neurinoma

  • Case Report
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

Intramedullary tumors and syringomyelia typically present with slowly progressing deficits. More rarely, they are characterized by acute presentation or worsening, at times mimicking other more common etiologies. The acute onset of syringomyelia is most likely attributable to an acute increase in cerebrospinal fluid and epidural venous pressure that results in impulsive fluid movement and, ultimately, in the rupture of the syrinx and dissection into the spinal cord or brainstem. Reported here is a case of acute presentation of a small cervical intramedullary neurinoma due to the upward dissection of its associated syrinx. Critical questions are: (1) how can a small tumor produce a large syrinx? and (2) in the absence of craniospinal interferences, which mechanism underlies the acute expansion of the cavity, resulting in a rapid onset? The authors examined the pathophysiology of syrinx formation and enlargement in intramedullary tumors and reviewed the literature, emphasizing the relationship between spinal cord movements and intramedullary pressure. On the basis of current pathogenetic concepts, the authors concluded that tumor-related syringomyelia might be caused by an association of mechanisms, both from within (obstruction of perivascular spaces; increase in extracellular fluid viscosity due to the tumor itself; intramedullary pressure gradients among different cord levels and between the cord and the subarachnoid space) and from without (the cerebrospinal fluid entering the tissue). All these factors may be amplified, as in the reported case, by a tumor located dorsally at the cervical level. Abnormal postures of the spine, such as a prolonged and excessive flexed neck position, may ultimately contribute to the acute dissection of the syrinx.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Anwer UE, Fisher M (1996) Acute and atypical presentations of syringomyelia. Eur Neurol 36:215–218

    CAS  PubMed  Google Scholar 

  2. Barnett HJM, Newcastle NB (1973) Syringomyelia and tumors of the nervous system. In: Barnett HJM, Foster JB, Hudgson P (eds) Syringomyelia. Major problems in neurology, vol 1. Saunders, London, pp 261–301

    Google Scholar 

  3. Batzdorf U (1991) Syringomyelia: current concepts in diagnosis and treatment. Williams & Wilkins, Baltimore

    Google Scholar 

  4. Cserr HF, Ostrach LH (1974) Bulk flow of interstitial fluid following intracranial injection of blue Dextran 2000. Exp Neurol 45:50–60

    Article  CAS  PubMed  Google Scholar 

  5. Delpirou C, Héroum C, Blard JM, Pagès M (2001) Acute onset of syringomyelia: two cases. Rev Neurol (Paris) 157:692–694

    CAS  Google Scholar 

  6. DiChiro G (1964) Movement of the cerebrospinal fluid in human beings. Nature 204:290–294

    CAS  PubMed  Google Scholar 

  7. Ellertsson AB, Greitz T (1969) Myelocystographic and fluorescein studies to demonstrate communication between intramedullary cysts and the cerebrospinal fluid space. Acta Neurol Scand 45:418–430

    CAS  PubMed  Google Scholar 

  8. Ellertsson AB, Greitz T (1970) The distending force in the production of communicating syringomyelia. Lancet 1:1234

    Article  CAS  Google Scholar 

  9. Fischbein NJ, Dillon WP, Cobbs C, Winstein PR (1999) The “presyrinx” state: a reversible myelopathic condition that may precede syringomyelia. Am J Neuroradiol 20:7–20

    CAS  PubMed  Google Scholar 

  10. Fuller R, Stanners A (2000) A cough, then respiratory failure. Lancet 355:284

    Article  CAS  PubMed  Google Scholar 

  11. Hall P, Turner M, Aichinger S, Bendick P, Campbell R (1980) Experimental syringomyelia: the relationship between intraventricular and intrasyrinx pressures. J Neurosurg 52:812–817

    CAS  PubMed  Google Scholar 

  12. Harrison DE, Cailliet R, Harrison DD, Troyanovich SJ, Harrison SO (1999) A review of biomechanics of the central nervous system. Part III: spinal cord stresses from postural loads and their neurologic effects. J Manip Physiol Ther 22:399–410

    CAS  Google Scholar 

  13. Heiss JD, Patronas N, DeVroom HL, Shawker T, Ennis R, Kammerer W, Eidsath A, Talbot T, Morris J, Eskioglu E, Oldfield EH (1999) Elucidating the pathophysiology of syringomyelia. J Neurosurg 91:553–562

    CAS  PubMed  Google Scholar 

  14. Ikata T, Masaki K, Kashwaguchi S (1988) Clinical and experimental studies on permeability of tracers in normal spinal cord and syringomyelia. Spine 13:737–741

    CAS  PubMed  Google Scholar 

  15. Jarzem PF, Quance DR, Doyle DJ, Begin LR, Kostuik JP (1992) Spinal cord tissue pressure during spinal cord distraction in dogs. Spine 17[Suppl 8]:227–234

    Google Scholar 

  16. Josephson A, Greitz D, Klason T, Olson L, Spenger C (2001) A spinal thecal sac constriction model supports the theory that induced pressure gradients in the cord cause edema and cyst formation. Neurosurgery 48:636–646

    Article  CAS  PubMed  Google Scholar 

  17. Kitahara Y, Iida H, Tachibana S (1995) Effect of spinal cord stretching due to head flexion on intramedullary pressure. Neurol Med-Chir (Tokyo) 35:285–288

    CAS  Google Scholar 

  18. Kiwitt JCW, Lanksch WR, Fritsch H, Luis E, Stork W, Roosen N, Schirmer M, Bock W, Marguth F (1988) Magnetic resonance tomography of solid spinal cord tumors with extensive secondary syringomyelia. Adv Neurosurg 16:211–215

    Google Scholar 

  19. Klekamp J (2002) The pathophysiology of syringomyelia. Historical overview and current concept. Acta Neurochir (Wien) 144:649–664

    Article  CAS  Google Scholar 

  20. Klekamp J, Volkel K, Bartels CJ, Samii M (2001) Disturbances of cerebrospinal fluid flow attributable to arachnoid scarring cause interstitial edema of the cat spinal cord. Neurosurgery 48:174–186

    Article  CAS  PubMed  Google Scholar 

  21. Levy EI, Heiss JD, Kent MS, Riedel CJ, Oldfield EH (2000) Spinal cord swelling preceding syrinx development. Case report. J Neurosurg (Spine 1) 92:93–97

    CAS  Google Scholar 

  22. Levy LM (1999) MR imaging of cerebrospinal fluid flow and spinal cord motion in neurologic disorders of the spine. Magn Reson Imaging Clin N Am 7:573–587

    CAS  PubMed  Google Scholar 

  23. Levy MR, Di Chiro G (1990) MR phase imaging and cerebrospinal fluid flow in the head and spine. Neuroradiology 32:399–406

    CAS  PubMed  Google Scholar 

  24. Meves SH, Postert T, Przuntek H, Buttner T (2000) Acute brainstem symptoms associated with cervical syringomyelia. Eur Neurol 43:47–49

    Article  CAS  PubMed  Google Scholar 

  25. Milhorat TH, Bolognese PA, Black KS, Woldenberg RF (2003) Acute syringomyelia: case report. Neurosurgery 53:1220–1222

    Article  PubMed  Google Scholar 

  26. Milhorat TH, Capocelli AL Jr, Anzil AP, Kotzen RM, Milhorat RH (1995) Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg 82:802–812

    CAS  PubMed  Google Scholar 

  27. Milhorat TH, Capocelli AL Jr, Kotzen RM, Bolognese P, Heger IM, Cottrell JE (1997) Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension. Neurosurgery 41:1102–1110

    Google Scholar 

  28. Oldfield EH (2001) Syringomyelia. J Neurosurg (Spine 1) 95:153–155

    CAS  Google Scholar 

  29. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3–15

    CAS  PubMed  Google Scholar 

  30. Quencer RM, Post MJ, Hinks RS (1990) Cine MRI in the evaluation of normal and abnormal CSF flow: intracranial and intraspinal studies. Neuroradiology 32:371–391

    CAS  PubMed  Google Scholar 

  31. Rennels ML, Gregory TF, Blaumanis OR, Fujimoto K, Grady PA (1985) Evidence for a “paravascular” fluid circulation in the mammalian central nervous system, provided by the rapid distribution of tracer protein throughout the brain from the subarachnoid space. Brain Res 326:47–63

    Article  CAS  PubMed  Google Scholar 

  32. Samii M, Klekamp J (1994) Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery 35:865–873

    CAS  PubMed  Google Scholar 

  33. Schlesinger EB, Antunes JL, Michelsen WJ, Louis KM (1981) Hydromyelia: clinical presentation and comparison of modalities of treatment. Neurosurgery 9:356–365

    CAS  PubMed  Google Scholar 

  34. Shiojiri T, Tsuchiya K, Watahiki S (1993) Sudden exacerbation in atypical syringomyelia with a unilateral superficial sensory disorders as the principal manifestation. No To Shinkei 45:1075–1078

    CAS  PubMed  Google Scholar 

  35. Stoodley MA, Jones NR, Yang L, Brown CJ (2000) Mechanisms underlying the formation and enlargement of noncommunicating syringomyelia. Neurosurg Focus 8(3): Article 2

    Google Scholar 

  36. Tachibana S, Iida H, Yada K (1992) Significance of positive Queckenstedt test in patients with syringomyelia associated with Arnold-Chiari malformations. J Neurosurg 76:67–71

    CAS  PubMed  Google Scholar 

  37. Tachibana S, Kitahara Y, Iida H, Yada K (1994) Spinal cord intramedullary pressure. A possible factor in syrinx growth. Spine 19:2174–2178

    CAS  PubMed  Google Scholar 

  38. Williams B (1986) Progress in syringomyelia. Neurol Res 8:130–145

    CAS  PubMed  Google Scholar 

  39. Williams B (1993) Syringobulbia: a surgical review. Acta Neurochir (Wien) 123:190–194

    CAS  Google Scholar 

  40. Yuan Q, Dougherty L, Margulies SS (1998) In vivo human cervical spinal cord deformation and displacement in flexion. Spine 23:1677–1683

    Article  CAS  PubMed  Google Scholar 

  41. Zager EL, Ojemann RG, Poletti CE (1990) Acute presentations of syringomyelia. Report of three cases. J Neurosurg 72:133–138

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Fatebenefratelli Hospital, Corso di Porta Nuova 23, 20121, Milan, Italy

    Vincenzo G. Amato, Roberto Assietti & Mario Morosi

  2. Via Saldini 38, 20133, Milan, Italy

    Vincenzo G. Amato

  3. Neurosurgical Clinic, University of Pavia, Pavia, Italy

    Cesare Arienta

Authors
  1. Vincenzo G. Amato
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Roberto Assietti
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mario Morosi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Cesare Arienta
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Vincenzo G. Amato.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Amato, V.G., Assietti, R., Morosi, M. et al. Acute brainstem dissection of syringomyelia associated with cervical intramedullary neurinoma. Neurosurg Rev 28, 163–167 (2005). https://doi.org/10.1007/s10143-004-0362-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10143-004-0362-5

Keywords

Search

Navigation