Differential response to corticosteroid therapy of MRI findings and clinical manifestations in spinal cord sarcoidosis

Spinal cord sarcoidosis is a rare disorder whose natural history and therap eutic outcome are not fully known. We examined four patients with spinal co rd sarcoidosis both clinically and radiologically, particularly in relation to corticosteroid treatment. The initial manifestation was cervical myelop athy in three and uveitis in one. All four patients progressed slowly until corticosteroid therapy was initiated. The cervical spine was involved in a ll patients. Magnetic resonance imaging (MRI) who showed spinal cord swelli ng with T2-weighted high intensity and linear leptomeningeal and patchy or diffuse intramedullary enhancement with gadolinium diethylene triamine-pent aacetic acid. With corticosteroid therapy, dramatic improvement was seen on MRI, including disappearance or marked reduction of swelling and enhanceme nt. Plasma levels of angiotensin-converting enzyme (ACE) were also markedly improved. In contrast, the clinical symptoms were little improved in one p atient, unchanged in two, and rather worsened in one patient. Recurrence wa s seen on MRI at the maintenance dose in all four patients, without any dra matic change in clinical manifestation. MRI findings and plasma ACE are wel l correlated with active lesion of the spinal cord sarcoidosis, providing a useful marker for recurrence, but do not parallel the clinical manifestati ons.