Hypertrophic spinal pachymeningitis associated with heavy-chain disease

Case report

Restricted access

✓The authors present a case of concurrent hypertrophic spinal pachymeningitis (HSP) and heavy-chain disease (HCD). The patient was a 71-year-old woman presenting with leg paralysis and fever. Laminectomy was performed for thoracic HSP, and chemotherapy was administered for HCD. Because the dura mater was not surgically opened, marked hypertrophy remained postoperatively. Once the HCD was controlled by chemotherapy, the fever subsided and the hypertrophy was resolved. No previous reports have supported a link between the two diseases. Idiopathic HSP may be secondary to rare conditions such as HCD, and systemic examinations should be performed to the fullest extent possible to investigate the possibility of some underlying disease.

Abbreviations used in this paper:HCD = heavy-chain disease; HSP = hypertrophic spinal pachymeningitis; MR = magnetic resonance.

Article Information

Address correspondence to: Kazuki Yunokawa, M.D., Department of Orthopedic Surgery, Matsuyama City Hospital, 2-6-5 Otemachi, Matsuyama City, Ehime 790–0067, Japan. email: yunoka@xk9.so-net.ne.jp.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    A: Gadolinium-enhanced MR imaging study revealing enhancement of the thickened dura mater. B: Axial view demonstrating ring-shaped enhancement of the dura mater at T11–12.

  • View in gallery

    Photomicrographs showing sections of excised tissue. A: Infiltration of inflammatory cells, mostly plasmacytes and lymphocytes, forming a lymphoid follicle. B: Diffuse infiltration of plasmacytes. H & E, original magnification ×40 (A) and × 100 (B).

  • View in gallery

    A: Gadolinium-enhanced MR imaging study obtained 3 days postoperatively revealing enhancement of the thickened dura mater. B: The ring-shaped enhancement of the dura mater remains.

  • View in gallery

    A: Gadolinium-enhanced MR image obtained 2 months postoperatively. B: Axial view, in which the ring-shaped enhancement of the dura mater is no longer evident.

References

  • 1

    Ashkenazi EConstantini SPappo OGomori MAverbuch-Heller LUmansky F: Hypertrophic spinal pachymeningitis: report of two cases and review of the literature. Neurosurgery 28:7307321991

  • 2

    Bernat JLSadowsky CHVincent FMNordgren REMargolis G: Sclerosing spinal pachymeningitis. A complication of intra-thecal administration of Depo-Medrol for multiple sclerosis. J Neurol Neurosurg Psychiatry 39:112411281976

  • 3

    Bucy PCFreeman LW: Hypertrophic spinal pachymeningitis with special reference to appropriate surgical treatment. J Neurosurg 9:5645781952

  • 4

    Charcot JMJoffroy A: Deux cas d'atrophie musculaire progressive avec lesions de la substance grise et des faisceaux anterolateraux de la moelle epiniere. Arch Physiol Norm Pathol 2:3543676296497447691869

  • 5

    Cogné MAucouturier PBrizard ADreyfus BDuarte FPreud'homme JL: Complete variable region deletion in a mu heavy chain disease protein (ROUL). Correlation with light chain secretion. Leuk Res 17:5275321993

  • 6

    Cogné MSilvain CKhamlichi AAPreud'homme JL: Structurally abnormal immunoglobulins in human immunoproliferative disorders. Blood 79:218121951992

  • 7

    Digman KEPartington CRGraves VB: MR imaging of spinal pachymeningitis. J Comput Assist Tomogr 14:9889901990

  • 8

    Dumont ASClark AWSevick RJMyles ST: Idiopathic hypertrophic pachymeningitis: a report of two patients and review of the literature. Can J Neurol Sci 27:3333402000

  • 9

    Fermand JPBrouet JCDanon FSeligmann M: Gamma heavy chain “disease”: heterogeneity of the clinicopathologic features. Report of 16 cases and review of the literature. Medicine (Baltimore) 68:3213351989

  • 10

    Forte FAPrelli FYount WJJerry LMKochwa SFranklin EC: Heavy chain disease of the gamma (gamma M) type: report of the first case. Blood 36:1371441970

  • 11

    Franklin ECLowenstein JBigelow BMeltzer M: Heavy chain disease—a new disorder of serum gamma-globulins: report of the first case. Am J Med 37:3323501964

  • 12

    Gribble LD: Syphilitic spinal pachymeningitis. S Afr Med J 46:132613281972

  • 13

    Guidetti BLa Torre E: Hypertrophic spinal pachymeningitis. J Neurosurg 26:4965031967

  • 14

    Gutmann LHable K: Rheumatoid pachymeningitis. Neurology 13:9019051963

  • 15

    Haas IG: BiP—a heat shock protein involved in immunoglobulin chain assembly. Curr Top Microbiol Immunol 167:71821991

  • 16

    Huang HHaq N: Spinal leptomeningeal sarcoidosis. Neuroradiology 29:1001987

  • 17

    Kanamori MMatsui HTerahata NTsuji H: Hypertrophic spinal pachymeningitis. A case report. Spine 22:178717901997

  • 18

    Kurz RMohssenipour ITwerdy K: [Meningeal inflammatory diseases causing transverse lesions in childhood (arachnoiditis spinalis chronica and pachymeningitis spinalis hypertrophicans). Diagnosis and treatment.]. Padiatr Padol 12:3653761977. (Ger)

  • 19

    Melnick JAviel SArgon Y: The endoplasmic reticulum stress protein GRP94, in addition to BiP, associates with unassembled immunoglobulin chains. J Biol Chem 267:21303213061992

  • 20

    Mikawa YWatanabe RHino YHirano K: Hypertrophic spinal pachymeningitis. Spine 19:6206251994

  • 21

    Oonishi TIshiko TArai MKase MMatsushita M: Pachymeningitis cervicalis hypertrophica. Acta Pathol Jpn 32:1631711982

  • 22

    Paulson GWMeagher JNBurkhart J: Spinal pachymeningitis secondary to mucopolysaccharidosis. Case report. J Neurosurg 41:6186211974

  • 23

    Rosenfeld JVKaye AHDavis SGonzales M: Pachymeningitis cervicalis hypertrophica. Case report. J Neurosurg 66:1371391987

  • 24

    Selby R: Pachymeningitis secondary to Allescheria boydii. Case report. J Neurosurg 36:2252271972

  • 25

    Seligmann MDanon FHurez DMihaesco EPreud'homme JL: Alpha-chain disease: a new immunoglobulin abnormality. Science 162:139613971968

  • 26

    Sridhar KVasudevan MC: Idiopathic chronic hypertrophic pachymeningitis causing thoracic cord compression. Br J Neurosurg 18:5155172004

  • 27

    Vilpo JAIrjala KViljanen MKKlemi PKouvonen IRönnemaa T: delta-Heavy chain disease. A study of a case. Clin Immunol Immunopathol 17:5845941980

  • 28

    Voller BVass KWanschitz JMachold KAsenbaum SHoberstorfer M: Hypertrophic chronic pachymeningitis as a localized immune process in the craniocervical region. Neurology 56:1071092001

  • 29

    Wahner-Roedler DLKyle RA: Heavy chain diseases. Best Pract Res Clin Haematol 18:7297462005

  • 30

    Yakushiji YKurohara KToda SAbe MKuroda Y: [A case of hypertrophic spinal pachymeningitis associated with MPO-ANCA.]. Rinsho Shinkeigaku 42:8738772002. (Jpn)

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 61 61 3
Full Text Views 64 64 0
PDF Downloads 77 77 0
EPUB Downloads 0 0 0

PubMed

Google Scholar