Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

Abstract

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. We report a case of IHSP followed up for 20 years in a 46-year-old man. Expansive laminoplasty was performed in 1991, and this case has been previously reported by a co-author. After 17 years, the patient's gait disturbance returned. Physical examination and imaging confirmed IHSP that had developed into syringomyelia at the T2-L1 conus level. This case was diagnosed as adhesive spinal arachnoiditis due to pachymeningitis caused by syringomyelia. T1-T4 laminectomy, a syringo-subarachnoid shunt (S-S shunt), and L2-L3 laminectomy were performed. The patient again developed dysesthesia and gait disturbance 3 years after the second operation. Most reports of IHSP have limited their focus to short-term follow-up after initial treatment with no long-term results. At present, there are only five reports referring to long-term results of greater than 5 years. All but one case needed additional surgery. To the best of our knowledge, this is the first case in which syringomyelia occurred in a patient with IHSP. It is important to note that syringomyelia may be a cause of symptom recrudescence during long-term follow-up in IHSP patients.

Concomitant idiopathic hypertrophic spinal pachymeningitis and Guillain-Barré syndrome in a patient: coincidence or a triggering mechanism?

“…Early disease recurrence was reported in 8 cases (Table 2). Tsutsui et al 33 reported 5 cases of recurrence, with a follow-up of more than 10 years, stating that the course of the disease follows one of 3 patterns: 1) sustained remission, 2) relapse with corticosteroid resistance, or 3) relapse with corticosteroid dependence. Tosa et al 32 reported that a higher recurrence and poorer prognosis was seen in patients with active inflammatory signs at presentation.…”

Idiopathic Hypertrophic Spinal Pachymeningitis with an Osteolytic Lesion

“…Most reports of IHSP have been limited to short-term follow-up after the initial treatment. According to previous reports, with long term follow-up exceeding 5 years, recurrence was noted in three of five cases 28) . In this regard, repeated follow ups are desirable in patient with IHSP even after successful treatment.…”

Effective Response of Methotrexate for Recurrent Idiopathic Hypertrophic Spinal Pachymeningitis

“…However, the mean follow-up period of reviewed patients was short (1.3 years; range, 1 week-4 years). Recently, Tsutsui et al20) has reported a long-term follow-up of 5 years of IHSP with a high recurrence rate and a review of the literature. Disease progression has been reported to be related to increased cerebro-spinal fluid protein and cells16).…”