Backgrounds: Intramedullary neurosarcoidosis may be the first and only manifestation of the disease and may mimic an idiopathic inflammatory demyelinating syndrome both clinically and on neuroimaging results. Methods and Results: Two patients who were seen initially with a relapsing-remitting neurologic course and a cervical intramedullary lesion on magnetic resonance imaging findings are reported. Both proved to have neurosarcoidosis. A computed axial tomographic scan of the chest showed hilar adenopathy, which provided a clue to the diagnosis. Conclusions: Symptoms due to an intramedullary cervical lesion can be the first manifestation of neurosarcoidosis. The clinical course can mimic a demyelinating illness. A high index of suspicion and a search for sarcoidosis at extraneural sites are required for an early diagnosis. Steroid treatment is associated with a favorable outcome.
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