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Intramedullary spinal sarcoidosis: clinical and magnetic resonance imaging characteristics
S S Junger1, B J Stern, S R Levine
1North Central Neurology, Denville, NJ 07834.
Abstract:
We present a retrospective series of the clinical and MRI findings in 16 patients with intramedullary spinal sarcoidosis (ISS), including 12 patients studied with gadolinium-DTPA. The spectrum of MRI findings includes leptomeningeal enhancement, fusiform spinal cord enlargement, focal or diffuse intramedullary disease, and spinal cord atrophy. We present a classification of ISS correlating the clinical progression and the temporal sequence of MRI abnormalities. Improvement occurred in five of 12 patients (42%) treated with immunosuppressive therapy.
Insights
Intramedullary spinal sarcoidosis (ISS) presents diverse MRI findings, including cord enlargement and enhancement. Immunosuppressive therapy showed improvement in 42% of patients with this rare spinal cord condition.
Area of Science:
- Neurology
- Radiology
- Immunology
Background:
- Sarcoidosis is a multisystem inflammatory disease.
- Intramedullary spinal sarcoidosis (ISS) is a rare manifestation affecting the spinal cord parenchyma.
- Clinical and imaging features of ISS can be diverse and challenging to diagnose.
Purpose of the Study:
- To describe the clinical and magnetic resonance imaging (MRI) findings in patients with ISS.
- To present a classification system for ISS based on clinical progression and MRI abnormalities.
- To evaluate the efficacy of immunosuppressive therapy in patients with ISS.
Main Methods:
- Retrospective case series of 16 patients diagnosed with ISS.
- Review of clinical data and MRI studies, including 12 patients who received gadolinium-DTPA contrast.
- Correlation of clinical course with temporal MRI findings.
Main Results:
- MRI findings varied, including leptomeningeal enhancement, spinal cord enlargement, focal/diffuse intramedullary lesions, and atrophy.
- A classification system was developed correlating clinical progression with MRI changes.
- Five out of 12 (42%) patients treated with immunosuppressive therapy demonstrated clinical improvement.
Conclusions:
- ISS exhibits a spectrum of MRI abnormalities that can be classified based on disease progression.
- Immunosuppressive therapy may lead to clinical improvement in a significant proportion of patients with ISS.
- Further research is warranted to elucidate the pathophysiology and optimize treatment strategies for ISS.