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Abstract & Commentary
By Michael Rubin, MD, Professor of Clinical Neurology, Weill Cornell Medical College. Dr. Rubin reports no financial relationships relevant to this field of study.
Synopsis: Spinal cord sarcoidosis is difficult to diagnose and may be confused with multiple sclerosis and neuromyelitis optica. Treatment response is variable.
Source: Cohen-Aubart F, et al. Spinal cord sarcoidosis: Clinical and laboratory profile and outcome of 31 patients in a case-control study. Medicine 2010;89;133-140.
Sarcoidosis, an idiopathic multisystem granulomatous disorder with a prevalence of 1-40/100,000 population, affects the nervous system in 5%–16% of cases, and the spinal cord in less than 1%. Differentiating sarcoid from other causes of myelopathy is challenging and this retrospective, multi-center, case-control study was undertaken to further its understanding by describing the clinical, laboratory, and magnetic resonance imaging (MRI) features of spinal sarcoid, its morbidity, long-term sequelae, and prognosis.
Between 1993 and 2006, 31 patients with spinal cord sarcoidosis were seen by the departments of Neurology, Medicine, and Pulmonology at Pitie-Salpetriere and Avicenne Hospitals in Paris and Bobigny, France, respectively. Inclusionary criteria included myelopathic presentation based on motor, sensory, sexual, and/or sphincteric symptoms and signs below a spinal cord level, spinal cord MRI revealing an intramedullary lesion, noncaseating granulomata on biopsy or elevated lymphocyte levels on bronchioalveolar lavage (BAL), and no other preferred diagnosis. Laboratory studies included chest X-ray, pulmonary function tests, thoracic computed tomography (CT) scans, bronchoscopy, tissue biopsies, histologic analyses, serum angiotensin-converting enzyme (ACE), cerebrospinal fluid (CSF) analysis, and spinal cord and brain MRI. Clinical course was categorized as monophasic, remitting-relapsing (intermittent flares with no progression between flares), or progressive with or without flares. Patients seen between 1999 and 2006 with clinical myelopathy, spinal MRI with an intramedullary abnormality, and laboratory features suggesting a diagnosis other than sarcoid, usually multiple sclerosis or neuromyelitis optica, served as controls (n = 30). Statistical analyses comprised the chi-square, Fisher exact and Mann-Whitney tests, and P values < 0.05 were considered significant.
Among 31 spinal sarcoidosis patients, mean age of onset was 41.6 years, with a mean of 15.1 months between symptom onset and diagnosis. Most (65%) were men, white (61%), with spinal sarcoid the initial presentation in 90%, most often subacute (74%) in onset. Extra-neurologic sarcoid was present in 22, involving the lung and mediastinal lymph nodes (n = 17, 77%), liver (n = 10, 45%), peripheral lymph nodes, eye, or skin (n=2, 9% each), and arthritis, bone or sinuses (n= 1, 5% each). Significant findings in the sarcoid group, compared to controls, included elevated serum C-reactive protein (CRP) (54% vs. 7% controls), lactate dehydrogenase (LDH) (53% vs. 0% controls), and ACE (39% vs. 13% controls), lymphopenia (50% vs. 3% controls), and hypergammaglobulinemia (33% vs. 7% controls), with elevated CSF protein and white blood cell count and low CSF glucose. No difference between patients and controls was found on liver function tests, serum calcium, or CSF ACE level or oligoclonal bands. Lymph node biopsy was most sensitive (8/8), whereas bone marrow and muscle biopsy were least sensitive (0/9). BAL lymphocytosis with CD4/8 >3.5 was present in 15/18. Among 26 patients who underwent spinal MRI, all demonstrated T2-weighted hyperintensity lesions in the thoracic (n = 17) or cervical (n = 15) region, which enhanced in 74%, and were often heterogeneous (31%), and multifocal (27%). Other than T2 extension along multiple vertebral bodies (6 vs. 2 in controls), these findings were not unlike those of controls. Brain MRI was abnormal in 55% of spinal sarcoid patients compared to 23% of controls (P = 0.01). Approximately one-third each had a monophasic, remitting-relapsing, or progressive course with or without flares despite treatments including corticosteroids, cyclosporine, cyclophosphamide, methotrexate, mycophenolate mofetil, and hydroxychloroquine. Spinal sarcoid remains a diagnostic challenge but MRI, CSF and serum CRP and LDH, in addition to biopsy of appropriate tissue, may assist in its accurate diagnosis.
Trans-esophageal, endoscopic, ultrasound-guided, fine-needle aspiration (EUS-FNA) of mediastinal lymph nodes may be a relatively less invasive, yet high yield, method of obtaining tissue for the diagnosis of sarcoid (Endoscopy 2010;42:213-7). Retrospective study was undertaken of 101 consecutive patients with suspected pulmonary sarcoidosis who underwent EUS-FNA of mediastinal lymph nodes with 22-gauge needles. Both cell-block analysis and conventional cytological evaluation were utilized. Over half had previously undergone non-diagnostic bronchoscopy. EUS detected granulomas in 87% by either cytology or cell-block analysis when employed together, and in 6 cytology-negative patients (33%), granulomas were present in the cell-block. Only 1 patient developed mediastinitis. EUS-FNA can reduce the number of mediastinoscopies required for the diagnosis of sarcoidosis.