Extramedullary Spinal Neurosarcoidosis: Report of Two Cases

Case Reports Patient 1. An 80 year-old woman was admitted to our department for a sudden onset of a sensorimotor defi cit in her right upper and lower limbs; she also complained of neck pain radiating into her right shoulder. The patient presented a sarcoidosis of the lung with hylar and mediastinal lymphadenopathy, and anterior uveitis, diagnosed in 1994. Sarcoid pathology of noncaseating granuloma with giant cells was detected in the lung biopsy specimen. She was treated with oral steroids for 1 year; for the last 10 years there had been no signs of disease. Neurological examination revealed right-sided hemiplegia and severe hypesthesia over the left side. The plantar response was extensor on the right side. Computed tomography of the brain and cervical spine was normal; magnetic resonance imaging (MRI) of the cervical spinal cord revealed a mass that was extramedullary in the right lateral aspect of the spinal canal at the level C 2 –C 3 and compressing the spinal cord ( fi g. 1 a–c). Surprisingly the plasma level of angiotensin-converting enzyme (ACE) Dear Sir, Sarcoidosis is a chronic systemic disorder of unknown etiology characterized in affected organs by an accumulation of epithelioid granulomas without caseation or staining for infectious agents and derangement of the normal tissue architecture [1] . These granulomas often incorporate multinucleated giant cells and lymphocytes. In the past decade, there has been signifi cant progress in our understanding of the immunopathogenesis of the disease, but the etiology of this enigmatic condition still eludes us. Clinical neurological involvement occurs in approximately 5% of patients. However, autopsy results suggest that subclinical involvement may be present in up to 25% of patients. Neurosarcoidosis is a great mimicker and an uncommon presentation of sarcoidosis; it is therefore a diagnostic challenge, especially when there is no prior history of systemic sarcoidosis [2–8] . A typical imaging feature is thickening and enhancement of the basilar leptomeninges of the brain. Other imaging fi ndings, such as enhancing or nonenhancing parenchymal lesions, dural and bone lesions may occur in the head and spine. Spinal sarcoidosis is a rare condition, whose natural history and therapeutic outcome are still not fully known, and extramedullary extradural mass formation is even rarer. We describe 2 additional cases Received: June 23, 2005 Accepted: October 12, 2005 Published online: January 6, 2006