Hypertrophic spinal pachymeningitis associated with heavy-chain disease

Case report

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✓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.



  • 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.


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