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Remitting/relapsing idiopathic hypertrophic spinal pachymeningitidis: comprehensive imaging work-up and MR monitoring
Emmanuel Claus1, Matthieu Rutgers, Christian Sindic
1Department of Radiology and Medical Imaging, Cliniques Universitaires Saint-Luc, Université catholique de Louvain, Avenue Hippocrate 10, 1200 Brussels, Belgium.
Abstract:
We present the initial MR and PET work-up in a diabetic 31-year-old female patient presenting with spinal cord compression because of abnormal meningeal tissue and highlight the close correlation between the clinical course and the monitoring of the lesions on MR images. The combination of non-specific inflammatory signs at histopathological examination and of the negative results of a comprehensive bacteriological and serological testing led to the diagnosis of idiopathic hypertrophic spinal pachymeningitidis. The abnormal tissue showed mild glucose uptake at whole-body FDG-PET examination. Repeated MR follow-up examinations during corticoid treatment only demonstrated partial shrinkage of the abnormal tissue. Symptomatic relapse and re-increase in lesion size on MR images occurred concomitantly after an 11-month symptom-free remitting interval under tapering corticoid treatment.
Insights
Idiopathic hypertrophic spinal pachymeningitis can cause spinal cord compression. This case highlights the correlation between clinical symptoms and MRI monitoring during treatment for this rare inflammatory condition.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Spinal cord compression can result from various pathologies, including inflammatory conditions affecting the meninges.
- Idiopathic hypertrophic spinal pachymeningitis is a rare, non-neoplastic, non-infectious inflammatory condition characterized by thickening of the dura mater.
Observation:
- A 31-year-old diabetic female presented with spinal cord compression due to abnormal meningeal tissue.
- Initial Magnetic Resonance (MR) and Positron Emission Tomography (PET) imaging revealed mild glucose uptake in the abnormal tissue.
- Histopathology showed non-specific inflammatory signs, with negative bacteriological and serological tests.
Findings:
- The patient was diagnosed with idiopathic hypertrophic spinal pachymeningitis.
- MR imaging demonstrated a close correlation with the patient's clinical course.
- Corticosteroid treatment led to partial shrinkage of lesions, but relapse occurred upon tapering therapy.
Implications:
- This case highlights the utility of MR and PET imaging in diagnosing and monitoring IHSP.
- The findings underscore the chronic and relapsing nature of IHSP, even with treatment.
- Further research into optimal management strategies for IHSP is warranted.
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