Child's Nervous System

, Volume 34, Issue 9, pp 1795–1798 | Cite as

Rapidly progressive fatal idiopathic hypertrophic pachymeningitis with brainstem involvement in a child

  • Kosuke TsuchidaEmail author
  • Shinobu Fukumura
  • Akiyo Yamamoto
  • Yukinori Akiyama
  • Hiroshi Hirano
  • Hiroyuki Tsutsumi
Case Report



Hypertrophic pachymeningitis (HP) is a rare disorder characterized by diffuse thickening of the dura mater with resultant neurologic deficits. HP develops secondary to various conditions or idiopathically usually in adults but rarely in children.

Case report

We describe a 3-year-old female child with idiopathic HP. Her HP involved the entire central nervous system with progression into the brainstem. The lesion responded poorly to pulsed steroids or any immunosuppressants. The brainstem lesion grew rapidly and formed various nodules that ultimately resulted in brain death. This is the first fatal case of HP in a child.


Hypertrophic pachymeningitis Brainstem lesion Brain death Pediatric 


Compliance with ethical standards

Conflict of interest



  1. 1.
    Riku S, Kato S (2003) Idiopathic hypertrophic pachymeningitis. Neuropathology 23:335–344CrossRefPubMedGoogle Scholar
  2. 2.
    Kupersmith MJ, Martin V, Heller G, Shah A, Mitnick HJ (2004) Idiopathic hypertrophic pachymeningitis. Neurology 62:686–694CrossRefPubMedGoogle Scholar
  3. 3.
    Yonekawa T, Murai H, Utsuki S, Matsushita T, Masaki K, Isobe N, Yamasaki R, Yoshida M, Kusunoki S, Sakata K, Fujii K, Kira JI (2014) A nationwide survey of hypertrophic pachymeningitis in Japan. J Neurol Neurosurg Psychiatry 85:732–739CrossRefPubMedGoogle Scholar
  4. 4.
    Dziedzic T, Wojciechowski J, Nowak A, Marchel A (2015) Hypertrophic pachymeningitis. Childs Nerv Syst 31:1025–1031CrossRefPubMedGoogle Scholar
  5. 5.
    Sharma PK, Saikia B, Sharma R, Gagneja V, Khilnani P (2014) Pachymeningitis in a young child responded to antitubercular therapy: a case report. J Child Neurol 29:92–95CrossRefGoogle Scholar
  6. 6.
    Karimi A, Tabatabaei SR, Shiva F, Sayfikar M, Jafari M, Shirvani F, Shiari R (2014) Tuberculous pachymeningitis in a young child with spinal involvement: a case report. Int J Infect Dis 21:419CrossRefGoogle Scholar
  7. 7.
    Asano T, Hayashida M, Ogawa K, Adachi K, Teramoto A, Yamamoto M (1998) Hypertrophic cranial pachymeningitis in a patient with aplastic anemia. Ann Hematol 77:279–282CrossRefPubMedGoogle Scholar
  8. 8.
    van Toorn R, Esser M, Smit D, Andronikou S (2008) Idiopathic hypertrophic cranial pachymeningitis causing progressive polyneuropathies in a child. Eur J Paediatr Neurol Off J Eur Paediatr Neurol Soc 12:144–147CrossRefGoogle Scholar
  9. 9.
    Aburahma SK, Anabtawi AG, Al Rimawi HS, Elheis MA, Mohtaseb AH (2009) Idiopathic hypertrophic pachymeningitis in a child with hydrocephalus. Pediatr Neurol 40:457–460CrossRefPubMedGoogle Scholar
  10. 10.
    Rojana-udomsart A, Pulkes T, Viranuwatti K, Laothamatas J, Phudhichareonrat S, Witoonpanich R (2008) Idiopathic hypertrophic cranial pachymeningitis. J Clin Neurosci 15:465–469CrossRefPubMedGoogle Scholar
  11. 11.
    Van Toorn R, Esser M, Smit D, Andronikou S (2008) Idiopathic hypertrophic cranial pachymeningitis causing progressive polyneuropathies in a child. Eur J Paediatr Neurol 12:144–147CrossRefPubMedGoogle Scholar
  12. 12.
    Lee YC, Chueng YC, Hsu SW, Lui CC (2003) Idiopathic hypertrophic cranial pachymeningitis: case report with 7 years of imaging follow-up. AJNR Am J Neuroradiol 24:119–123PubMedGoogle Scholar
  13. 13.
    Kleiter I, Hans VH, Schuierer G, Marienhagen J, Hau P, Schütz H, Bogdahn U, Steinbrecher A (2004) Intraventricular cytarabine in a case of idiopathic hypertrophic pachymeningitis. J Neurol Neurosurg Psychiatry 75:1346–1348CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Sable SG, Ramadan NM (1991) Meningeal enhancement and low CSF pressure headache. An MRI study. Cephalalgia 11(6):275–276CrossRefPubMedGoogle Scholar

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© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PediatricsSapporo Medical University School of MedicineSapporoJapan
  2. 2.Department of NeurosurgerySapporo Medical University School of MedicineSapporoJapan
  3. 3.Department of Surgical PathologySapporo Medical University School of MedicineSapporoJapan

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