J Korean Neurosurg Soc.  2014 Aug;56(2):162-165. 10.3340/jkns.2014.56.2.162.

Idiopathic Hypertrophic Spinal Pachymeningitis with an Osteolytic Lesion

• Jee TK
• Lee SH
• Kim ES
• Eoh W

• KMID: 1956522
• DOI: http://doi.org/10.3340/jkns.2014.56.2.162

Abstract

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic, progressive, inflammatory disorder characterized by marked fibrosis of the spinal dura mater with unknown etiology. According to the location of the lesion, it might induce neurologic deficits by compression of spinal cord and nerve root. A 58-year old female with a 3-year history of progressive weakness in both lower extremities was referred to our institute. Spinal computed tomography (CT) scan showed an osteolytic lesion involving base of the C6 spinous process with adjacent epidural mass. Magnetic resonance imaging (MRI) revealed an epidural mass involving dorsal aspect of cervical spinal canal from C5 to C7 level, with low signal intensity on T1 and T2 weighted images and non-enhancement on T1 weighted-enhanced images. We decided to undertake surgical exploration. At the operation field, there was yellow colored, thickened fibrous tissue over the dura mater. The lesion was removed totally, and decompression of spinal cord was achieved. Symptoms improved partially after the operation. Histopathologically, fibrotic pachymeninges with scanty inflammatory cells was revealed, which was compatible with diagnosis of idiopathic hypertrophic pachymeningitis. Six months after operation, motor power grade of both lower extremities was normal on physical examination. However, the patient still complained of mild weakness in the right lower extremity. Although the nature of IHSP is generally indolent, decompressive surgery should be considered for the patient with definite or progressive neurologic symptoms in order to prevent further deterioration. In addition, IHSP can present as an osteolytic lesion. Differential diagnosis with neoplastic disease, including giant cell tumor, is important.

Keyword

Idiopathic hypertrophic spinal pachymeningitis; Osteolytic lesion; Mimicking giant cell tumor; Decompressive surgery; Differential diagnosis

MeSH Terms

Decompression
Diagnosis
Diagnosis, Differential
Dura Mater
Female
Fibrosis
Giant Cell Tumors
Humans
Lower Extremity
Magnetic Resonance Imaging
Meningitis*
Neurologic Manifestations
Physical Examination
Spinal Canal
Spinal Cord

Figure

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  Fig. 1 Computed tomography scan revealed an osteolytic lesion involving base of the C6 spinous process.

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  Fig. 2 Magnetic resonance imaging revealed an epidural mass involving the dorsal aspect of cervical spinal canal from C5 to C7 level, with low signal intensity on T2 weighted image (A) and non-enhancement on T1 weighted-enhanced image (B).

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  Fig. 3 Photomicrograph of a section of the excised dura mater showed fibrotic pachymeninges with no significant inflammation, which was more apparent at the higher magnification view (Hematoxylin and eosin staining).

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