Clinical features of hypertrophic pachymeningitis in a center survey

  • Gonçalo Cação
  • Margarida Calejo
  • José Eduardo Alves
  • Pedro Bettencourt Medeiros
  • Nuno Vila-Cha
  • Teresa Mendonça
  • Ricardo Taipa
  • Ana Martins Silva
  • Joana DamásioEmail author
Original Article



Hypertrophic pachymeningitis (HP) is characterized by cranial and/or spinal thickening of the dura mater with or without associated inflammation. Neuroimaging studies reveal dura mater thickening and focal or diffuse contrast enhancement. It is described in association with trauma, infections, tumors, autoimmune/inflammatory diseases, and cerebrospinal fluid hypotension syndrome, with some cases remaining idiopathic.


A retrospective study was conducted with patients’ identification through a key terms search within MRI reports in the period of July 2008 to September 2015. Clinical files, MRI, laboratory, and pathology data were reviewed.


Fifty-three patients were identified and 20 were excluded because they did not meet the inclusion criteria. Of the 33 included, 19 were female, with a mean age at symptoms onset of 51.2 ± 17.6 years. The most common presenting symptoms were headache and cranial nerves palsy, followed by seizures, delirium, lumbar pain, cognitive decline, motor deficit, and language impairment. In 17 patients, a neoplastic etiology was identified; in eight, inflammatory/autoimmune; in six, infectious; and two were classified as idiopathic. Of the eight patients with inflammatory/autoimmune etiology, four had possible IgG4-related disease (IgG4-RD) and the remaining had granulomatosis with polyangiitis, sarcoidosis, rheumatoid arthritis, and Tolosa-Hunt syndrome. Treatment was directed according to the underlying etiology.


In the described series, a female predominance was identified, with symptoms’ onset in the 5th decade. Although headache was the most common symptom, clinical presentation was varied, emphasizing the role of MRI in HP diagnosis. The underlying etiologies were diverse, with only a few cases remaining idiopathic, also reflecting the contribution of the recently described IgG4-RD.


Hypertrophic pachymeningitis Neurological symptoms Magnetic resonance imaging IgG4-related disease 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.


  1. 1.
    Kupersmith MJ, Martin V, Heller G, Shah A, Mitnick HJ (2004) Idiopathic hypertrophic pachymeningitis. Neurology 62(5):686–694CrossRefGoogle Scholar
  2. 2.
    Kim JH, Park Y, Chin DK (2011) Idiopathic hypertrophic spinal pachymeningitis: report of two cases and review of the literature. J Korean Neurosurg Soc 50(4):392–395. CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Mott FW (1909) A case of localized syphilitic pachymeningitis cerebri. Arch Neurol Psychiatr 4:63–69Google Scholar
  4. 4.
    Sylaja PN, Cherian P, Das CK, Radhakrishnan VV, Radhakrishnan K (2002) Idiopathic hypertrophic cranial pachymeningitis. Neurol India 50(1):53–59PubMedGoogle Scholar
  5. 5.
    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(7):732–739. CrossRefPubMedGoogle Scholar
  6. 6.
    Wang YJ, Fuh JL, Lirng JF, Lu SR, Wang SJ (2004) Headache profile in patients with idiopathic hypertrophic cranial pachymeningitis. Headache 44(9):916–923CrossRefGoogle Scholar
  7. 7.
    Honma S, Fukazama T, Hamada K, Hamada T, Tashiro K (1996) MRI changes in spontaneous intracranial hypotension. Rinsho Shinkeigaku 36(7):912–915PubMedGoogle Scholar
  8. 8.
    Joelson E, Ruthrauff B, Ali F, Lindeman N, Sharp FR (2000) Multifocal dural enhancement associated with temporal arteritis. Arch Neurol 57(1):119–122CrossRefGoogle Scholar
  9. 9.
    Muthukumar N, Senthilbabu S, Usharani K (2005) Idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa-Hunt syndrome. J Clin Neurosci 12(5):589–592CrossRefGoogle Scholar
  10. 10.
    Lu LX, Della-Torre E, Stone JH, Clark SW (2014) IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol 71(6):785–793. CrossRefPubMedGoogle Scholar
  11. 11.
    Tan HJ, Raymond A, Phadke PP, Rozman Z (2004) Rheumatoid pachymeningitis. Singap Med J 45(7):337–339Google Scholar
  12. 12.
    Just SA, Knudsen J, Nielsen MK, Junker P (2011) Wegener’s granulomatosis presenting with pachymeningitis: clinical and imaging remission by rituximab. ISRN Rheumatol 608942:1–4. CrossRefGoogle Scholar
  13. 13.
    Ranoux D, Devaux B, Lamy C, Mear JY, Roux FX, Mas JL (1992) Meningeal sarcoidosis, pseudo-meningioma, and pachymeningitis of the convexity. J Neurol Neurosurg Psychiatry 55(4):300–303CrossRefGoogle Scholar
  14. 14.
    Li JY, Lai PH, Lam HC, Lu LY, Cheng HH, Lee JK (1999) Hypertrophic cranial pachymeningitis and lymphocytic hypophysitis in Sjögren syndrome. Neurology 52:420–423CrossRefGoogle Scholar
  15. 15.
    Noel N, Drier A, Wechsler B, et al. (2014) Neurological manifestations of Behçet’s disease. Rev Med Interne 35(2):112–20.
  16. 16.
    Yu WL, Bhatia KSS, Wang K (2012) Hypertrophic Pachymeningitis as the first manifestation of systemic lupus erythematosus. Hong Kong J Radiol 15:119–122Google Scholar
  17. 17.
    Bosman T, Simonin C, Launay D, Caron S, Destée A, Defebvre L (2008) Idiopathic hypertrophic cranial pachymeningitis treated by oral methotrexate: a case report and review of literature. Rheumatol Int 28(7):713–718. CrossRefPubMedGoogle Scholar
  18. 18.
    Hori T, Tsuboi Y, Okubo R, Hirooka M, Yamada T (1999) Crow-Fukase syndrome associated with Castleman disease showing hypertrophic cranial pachymeningitis and bilateral internal carotid artery occlusion. Rinsho Shinkeigaku 39(4):456–460PubMedGoogle Scholar
  19. 19.
    Watanabe M, Ushiyama O, Matsui M, Kakigi R, Kuroda Y (1993) A case of Crow-Fukase syndrome associated with chronic pachymeningitis. Rinsho Shinkeigaku 33(4):422–426PubMedGoogle Scholar
  20. 20.
    Briani C, Fedrigo M, Manara R, Castellani C, Zambello R, Citton V, Campagnolo M, Torre CD, Lucchetta M, Orvieto E, Rotilio A, Marangoni S, Magi S, Pareyson D, Florio I, Pegoraro E, Thiene G, Battistin L, Adami F, Angelini A (2012) Pachymeningeal involvement in POEMS syndrome: MRI and histopathological study. J Neurol Neurosurg Psychiatry 83(1):33–37. CrossRefPubMedGoogle Scholar
  21. 21.
    Massey J (2017) IgG4-related hypertrophic pachymeningitis coexpressing antineutrophil cytoplasmic antibodies. Neurol Neuroimmunol Neuroinflamm 4(3):e341. CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Wallace ZS, Carruthers MN, Khosroshahi A, Carruthers R, Shinagare S, Stemmer-Rachamimov A, Deshpande V, Stone JH (2013) IgG4-related disease and hypertrophic pachymeningitis. Medicine 92:206–216. CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Hahn LD, Fulbright R, Baehring JM (2016) Hypertrophic pachymeningitis. J Neurol Sci 367:278–283. CrossRefPubMedGoogle Scholar
  24. 24.
    Williams T, Marta M, Giovannoni G (2015) IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension. Pract Neurol 0:1–5. CrossRefGoogle Scholar
  25. 25.
    Baptista B, Casian A, Gunawardena H, D’Cruz D, Rice CM (2017) Neurological manifestations of IgG4-related disease. Curr Treat Options Neurol 19(4):14. CrossRefPubMedPubMedCentralGoogle Scholar
  26. 26.
    Deshpande V, Zen Y, Chan JK et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25:1181–1192. CrossRefPubMedGoogle Scholar
  27. 27.
    Makino S, Tanaka Y (2013) A case of hypertrophic pachymeningitis with elevated serum IgG4. J Clin Exp Ophthalmol 4:1. CrossRefGoogle Scholar
  28. 28.
    Thurtell MJ, Keed AB, Yan M, Gottlieb T, Spies JM, Halmagyi GM (2007) Tuberculous cranial pachymeningitis. Neurology 68:298–300CrossRefGoogle Scholar
  29. 29.
    Nadgir DB, Ramdas R, Kulkarni RV, Oak PJ, Shah AB (2003) Cavernous sinus syndrome due to syphilitic pachymeningitis. Neurol India 51(2):289–290PubMedGoogle Scholar

Copyright information

© Fondazione Società Italiana di Neurologia 2018

Authors and Affiliations

  • Gonçalo Cação
    • 1
  • Margarida Calejo
    • 1
  • José Eduardo Alves
    • 2
  • Pedro Bettencourt Medeiros
    • 3
  • Nuno Vila-Cha
    • 1
  • Teresa Mendonça
    • 3
  • Ricardo Taipa
    • 4
  • Ana Martins Silva
    • 1
  • Joana Damásio
    • 1
    Email author
  1. 1.Neurology DepartmentHospital de Santo António, Centro Hospitalar do PortoPortoPortugal
  2. 2.Neuroradiology DepartmentHospital de Santo António, Centro Hospitalar do PortoPortoPortugal
  3. 3.Internal Medicine DepartmentHospital de Santo António, Centro Hospitalar do PortoPortoPortugal
  4. 4.Neuropathology UnitHospital de Santo António, Centro Hospitalar do PortoPortoPortugal

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