Idiopathic Hypertrophic Spinal Pachymeningitis

Abstract

Hypertrophic pachymeningitis (HP) is a rare presentation with duramater thickening and fibrosis which can result in cranial or spinal compressive disease. Most cases of spinal HP require surgical management. We present an uncommon case of idiopathic hypertrophic spinal pachymeningitis (IHSP) in a 40-year-old male who showed complete improvement to steroids without any further relapses. The patient presented with bilateral upper limb weakness with magnetic resonance imaging (MRI) spine showing diffuse dural thickening of the entire spine with cervical cord compression. He had an extensive workup for underlying etiology and worsening symptoms until he was diagnosed with IHSP. Later, he was started on high-dose steroids with good response and no relapse after 2 years. A descriptive analysis of IHSP cases since 2009 including ours showed that it usually occurs after 50s with female preponderance. Weakness and sensory loss are the most common complaints with 50% patients showing clinical signs of myelopathy like hyperreflexia, clonus, Babinski sign and sensory level. Cerebrospinal fluid (CSF) and inflammatory markers like erythrocytic sedimentation rate (ESR) and C-reactive protein (CRP) can be used to assess disease progression and prognosis. Surgical removal of HP followed by steroids is the best line of management while steroids alone can be tried in cases where clinical signs of myelopathy are absent.

Keywords: Hypertrophic pachymeningitis; IHSP; Idiopathic; Spinal cord compression; Steroids.

Figure 1

MRI of the brain. (a) T1-weighted MRI of the brain sagittal section post-contrast that reveals dural thickening of the posterior fossa indicated by the red arrow. (b) T1-weighted MRI of the brain axial section post-contrast that reveals a right cerebellopontine angle mass revealed by the blue arrow and dural thickening of the posterior fossa indicated by the red arrow. (c) T2-weighted MRI of the brain axial section that reveals a right cerebellopontine angle mass revealed by the blue arrow. MRI: magnetic resonance imaging.

Figure 2

MRI cervical spine. (a, b) T1-weighted MRI spine sagittal section without and with contrast showing cervical spine dural thickening with narrowing of cord respectively. (c, d) T1-weighted MRI spine without and with contrast axial section showing narrowing of cord respectively. MRI: magnetic resonance imaging.

Figure 3

MRI cervical spine. Follow-up T1-weighted MRI with contrast showing decreased meningeal enhancement and narrowing. (a) Sagittal view. (b) Axial view. MRI: magnetic resonance imaging.