Skip to main content

Advertisement

SpringerLink
Log in

An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

This study determined the statewide incidence and prevalence of acute disseminated encephalomyelitis (ADEM) and examined the course of three pediatric patients treated for tumefactive demyelination (TD) at the Blair E. Batson Children’s Hospital.

Methods

Analyses of ICD-9-CM code hospital records and clinical database were conducted.

Results

From 2001 through 2007 the incidence in pediatric patients under 20 years was 0.4/100,000/year, with a prevalence of 8.6/100,000 during 2008. Three patients presented with TD. Case 1 had a 3-week history of ataxia and diplopia; case 2 presented with a sudden onset of coma, while the third child had a 4-month history of increasing lethargy and clumsiness in all extremities. Cerebrospinal fluid examinations were nondiagnostic. MRI examinations revealed asymmetric T2/fluid-attenuated inversion recovery hyperintensity within the pons (case 1), a large heterogenously enhancing temporal lobe mass, with extensive edema (case 2), and multiple small brain lesions with occasional ring enhancement (case 3). In case 1, intralesional MR spectroscopy demonstrated changes consistent with ADEM. Case 2 required intracranial monitoring, and medical treatment to control elevated ICP. Cases 2 and 3 underwent cortical biopsies that revealed ADEM. All three patients improved with corticosteroid therapy. At a minimum of 15 months follow-up, cases 1 and 2 showed resolution of deficits and MRI lesions, while the third patient demonstrated additional MRI lesions and increasing paraparesis.

Conclusions

These cases demonstrate that appropriate neuroradiological evaluation, treatment of acutely elevated ICP, and brain biopsy can play critical roles in the management of children with undiagnosed ADEM and TD.

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
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Krupp LB, Banwell B, Tenembaum S (2007) Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology 68(16 Suppl 2):S7–S12

    Article  PubMed  Google Scholar 

  2. Tenembaum S, Chitnis T, Ness J, Hahn JS (2007) Acute disseminated encephalomyelitis. Neurology 68(16 Suppl 2):S23–S36

    Article  PubMed  Google Scholar 

  3. Marchioni E, Tavazzi E, Minoli L, Del BS, Ferrante P, Piccolo G, Bastianello S, Ravaglia S (2008) Acute disseminated encephalomyelitis. Neurol Sci 29(Suppl 2):S286–S288

    Article  PubMed  Google Scholar 

  4. Leake JA, Albani S, Kao AS, Senac MO, Billman GF, Nespeca MP, Paulino AD, Quintela ER, Sawyer MH, Bradley JS (2004) Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J 23(8):756–764

    Article  PubMed  Google Scholar 

  5. Murthy SN, Faden HS, Cohen ME, Bakshi R (2002) Acute disseminated encephalomyelitis in children. Pediatrics 110(2 Pt 1):e21

    Article  PubMed  Google Scholar 

  6. Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG (2000) Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 123(Pt 12):2407–2422

    Article  PubMed  Google Scholar 

  7. Tenembaum S, Chamoles N, Fejerman N (2002) Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology 59(8):1224–1231

    PubMed  Google Scholar 

  8. Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ (2001) Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 56(10):1308–1312

    CAS  PubMed  Google Scholar 

  9. Khurana DS, Melvin JJ, Kothare SV, Valencia I, Hardison HH, Yum S, Faerber EN, Legido A (2005) Acute disseminated encephalomyelitis in children: discordant neurologic and neuroimaging abnormalities and response to plasmapheresis. Pediatrics 116(2):431–436

    Article  PubMed  Google Scholar 

  10. Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B (2001) Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients. Neurology 56(10):1313–1318

    CAS  PubMed  Google Scholar 

  11. Menge T, Hemmer B, Nessler S, Wiendl H, Neuhaus O, Hartung HP, Kieseier BC, Stuve O (2005) Acute disseminated encephalomyelitis: an update. Arch Neurol 62(11):1673–1680

    Article  PubMed  Google Scholar 

  12. Cuadrado MJ, Khamashta MA, Ballesteros A, Godfrey T, Simon MJ, Hughes GR (2000) Can neurologic manifestations of Hughes (antiphospholipid) syndrome be distinguished from multiple sclerosis? Analysis of 27 patients and review of the literature. Medicine (Baltimore) 79(1):57–68

    Article  CAS  Google Scholar 

  13. Paran D, Chapman J, Korczyn AD, Elkayam O, Hilkevich O, Groozman GB, Levartovsky D, Litinsky I, Caspi D, Segev Y, Drory VE (2006) Evoked potential studies in the antiphospholipid syndrome: differential diagnosis from multiple sclerosis. Ann Rheum Dis 65(4):525–528

    Article  CAS  PubMed  Google Scholar 

  14. Singh S, Alexander M, Korah IP (1999) Acute disseminated encephalomyelitis: MR imaging features. AJR Am J Roentgenol 173(4):1101–1107

    CAS  PubMed  Google Scholar 

  15. Khong PL, Ho HK, Cheng PW, Wong VC, Goh W, Chan FL (2002) Childhood acute disseminated encephalomyelitis: the role of brain and spinal cord MRI. Pediatr Radiol 32(1):59–66

    Article  PubMed  Google Scholar 

  16. Tateishi K, Takeda K, Mannen T (2002) Acute disseminated encephalomyelitis confined to brainstem. J Neuroimaging 12(1):67–68

    Article  CAS  PubMed  Google Scholar 

  17. Miller DH, Scaravilli F, Thomas DC, Harvey P, Hirsch NP (1993) Acute disseminated encephalomyelitis presenting as a solitary brainstem mass. J Neurol Neurosurg Psychiatry 56(8):920–922

    Article  CAS  PubMed  Google Scholar 

  18. Firat AK, Karakas HM, Yakinci C, Altinok T, Alkan A, Bicak U (2004) An unusual case of acute disseminated encephalomyelitis confined to brainstem. Magn Reson Imaging 22(9):1329–1332

    Article  PubMed  Google Scholar 

  19. Balasubramanya KS, Kovoor JM, Jayakumar PN, Ravishankar S, Kamble RB, Panicker J, Nagaraja D (2007) Diffusion-weighted imaging and proton MR spectroscopy in the characterization of acute disseminated encephalomyelitis. Neuroradiology 49(2):177–183

    Article  CAS  PubMed  Google Scholar 

  20. Bizzi A, Ulug AM, Crawford TO, Passe T, Bugiani M, Bryan RN, Barker PB (2001) Quantitative proton MR spectroscopic imaging in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol 22(6):1125–1130

    CAS  PubMed  Google Scholar 

  21. Sekula RF Jr, Marchan EM, Baghai P, Jannetta PJ, Quigley MR (2006) Central brain herniation secondary to fulminant acute disseminated encephalomyelitis: implications for neurosurgical management. Case report. J Neurosurg 105(3):472–474

    Article  PubMed  Google Scholar 

  22. Refai D, Lee MC, Goldenberg FD, Frank JI (2005) Decompressive hemicraniectomy for acute disseminated encephalomyelitis: case report. Neurosurgery 56(4):E872

    Article  PubMed  Google Scholar 

  23. Mitha AP, Scott JN, George D, Hanson A, MacRae ME, Bell RB (2007) Tumefactive demyelinating lesions. Can J Neurol Sci 34(3):362–364

    CAS  PubMed  Google Scholar 

  24. McAdam LC, Blaser SI, Banwell BL (2002) Pediatric tumefactive demyelination: case series and review of the literature. Pediatr Neurol 26(1):18–25

    Article  PubMed  Google Scholar 

  25. Riva D, Chiapparini L, Pollo B, Balestrini MR, Massimino M, Milani N (2008) A case of pediatric tumefactive demyelinating lesion misdiagnosed and treated as glioblastoma. J Child Neurol 23(8):944–947

    Article  PubMed  Google Scholar 

  26. Tan HM, Chan LL, Chuah KL, Goh NS, Tang KK (2004) Monophasic, solitary tumefactive demyelinating lesion: neuroimaging features and neuropathological diagnosis. Br J Radiol 77(914):153–156

    Article  CAS  PubMed  Google Scholar 

  27. Yapici Z, Eraksoy M (2002) Bilateral demyelinating tumefactive lesions in three children with hemiparesis. J Child Neurol 17(9):655–660

    Article  PubMed  Google Scholar 

  28. Lim KE, Hsu YY, Hsu WC, Chan CY (2003) Multiple complete ring-shaped enhanced MRI lesions in acute disseminated encephalomyelitis. Clin Imaging 27(4):281–284

    Article  PubMed  Google Scholar 

  29. van der Meyden CH, de Villiers JF, Middlecote BD, Terblanche J (1994) Gadolinium ring enhancement and mass effect in acute disseminated encephalomyelitis. Neuroradiology 36(3):221–223

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors wish to thank Ms. Laura Day for her secretarial assistance.

Disclaimer

The authors declare they have no conflicts of interest.

Author information

Authors and Affiliations

  1. Department of Neurosurgery, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS, 39216-4505, USA

    Matthew VanLandingham & William Hanigan

  2. Department of Neurology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS, 39216-4505, USA

    Vetta Vedanarayanan

  3. Department of Pathology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS, 39216-4505, USA

    Jonathan Fratkin

Authors
  1. Matthew VanLandingham
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. William Hanigan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Vetta Vedanarayanan
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jonathan Fratkin
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to William Hanigan.

Rights and permissions

Reprints and permissions

About this article

Cite this article

VanLandingham, M., Hanigan, W., Vedanarayanan, V. et al. An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children. Childs Nerv Syst 26, 655–661 (2010). https://doi.org/10.1007/s00381-009-1045-x

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-009-1045-x

Keywords

Search

Navigation