Effective Response of Methotrexate for Recurrent Idiopathic Hypertrophic Spinal Pachymeningitis

Abstract

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic progressive and diffuse inflammatory fibrosis of the spinal dura mater. Though treatment of IHSP is surgical decompression with steroid therapy, treatment for recurrent IHSP is controversial. Our patient was diagnosed with IHSP based on magnetic resonance imaging (MRI) and underwent laminectomy for decompression following steroid pulse therapy. Despite maintenance of steroid therapy, the patient experienced 3 recurrences. As an alternative immunosuppressant medication, methotrexate was introduced with low-dose steroid. Fortunately, the symptom was resolved, and a decrease of dura thickening was revealed on MRI. We present the case and suggest that methotrexate might be an effective treatment modality for recurrent IHSP.

Idiopathic hypertrophic spinal pachymeningitis with compressive myelopathy: a case report

“…The stage-based symptoms of IHSP were first reported by Charcot [11]. The first stage primarily shows progressive radicular symptoms, while the second stage shows muscle weakness and atrophy, and the third stage shows paralysis of the lower extremities and bladder and progressive anal dysfunction [12]. The etiology of IHSP is unclear.…”

Idiopathic Hypertrophic Spinal Pachymeningitis with Spinal Cord Lesion: A Case Report

“… 2 5 10) In one case report, the author suggested methotrexate therapy for recurrent IHSP that was refractory to steroid administration, which produced a good outcome without complications. 13) Many reports have suggested positive effects of postoperative immunosuppressive treatment, including methotrexate, cyclophosphamide, azathioprine, and radiotherapy. 1 2 5) The treatment of our patient involved surgical excision of the lesion, decompressive laminectomy, and postoperative steroid therapy.…”

Recurrent ominous spinal mass

“…1 Other authors have recommend methotrexate as medical first line treatment. 2 Another case report, describing a case of recurrent idiopathic hypertrophic spinal pachymeningitis refractory to first line steroid treatment, describes the successful use of Rituximab as second-line treatment. 3 Moreover, strategies combining these three medical treatment approaches are also possible.…”

“…It is typically encountered intracranially rather than spinally and can have a progressive and recurrent course as encountered in our patient. [1][2][3] The aetiology of this disorder is multifactorial, including infectious diseases (tuberculosis, lues, neurocysticercosis), rheumatic diseases (sarcoidosis), metabolic diseases, as well as vasculitis, autoimmune disorders, intrathecal injections or -as in our case -idiopathic. [4][5][6][7] While acute management of hypertrophic pachymeningitis consists of immediate decompression in conjunction with steroid medication and treatment of the underlying disease, the management of chronic and recurrent disease is controversially debated in the current literature.…”