Abstract
Objective
Chronic involvement of the nervous system is relatively rare in sarcoidosis. We describe 7 cases that fulfil Zajicek's criteria for neurosarcoidosis (NS) and propose some modifications to such criteria.
Materials and methods
The patients were admitted for various neurological syndromes: 2 cases presented with chronic lymphocytic meningitis, 4 with spinal cord symptoms, one case was initially confused with multiple sclerosis. Serological tests, immunological screening, cerebrospinal fluid (CSF) analysis, bacteriological and viral testing were performed in all patients. Spinal and cerebral MRI, gallium scan, bronchoscopy with biopsy and bronchoalveolar–lavage fluid analysis, high–resolution computed tomography (HRCT) of the chest, biopsy of the lungs, skin, mediastinal lymph–node and meninges, were useful in diagnosing NS.
Results and discussion
Laboratory tests showed serum inflammatory abnormalities, but were negative for infectious diseases, while CSF showed inflammatory signs in all patients. MRI revealed meningeal enhancement or hypertrophic pachymeningeal lesions in 4 patients, white matter abnormalities and mass lesions in 2 patients, and a spinal mass lesion in 1 patient. Gallium scan, HRCT, bronchoscopy were positive in most cases. Patients were treated with steroid and immunosuppressive therapy, with improvement in six cases. One patient died from infectious complications.
Conclusion
A definite diagnosis of NS requires demonstration of non–caseating granulomas affecting nervous tissues. In most cases, histological evidence of systemic disease (probable NS) is sufficient in the presence of compatible alterations in the CNS. In our patients the bronchoalveolarlavage fluid analysis, gallium scan, and chest HRCT were important for diagnosis,while serum ACE was always normal and chest radiographs were not suggestive of sarcoidosis.
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References
Agostini, et al. (1998) Cells and molecules involved in the development of sarcoid granuloma. J Clin Immunol 18:184–192
Agbogu, et al. (1995) Therapeutic considerations in patients with refractory neurosarcoidosis. Arch Neurol 52:875–879
Chapelon–Abric, et al. (1991) Neurosarcoidoses. Ann Med Intern 142:601–608
Costabel U (1992) Sensitivity and specificity of BAL findings in sarcoidosis. Sarcoidosis 9(Suppl 1):211–214
Ferreby, et al. (2000) Manifestations cliniques et approche therapeutique de la neurosarcoidose: 40 cas. Rev Neurol (Paris) 156:965–975
Ferreby, et al. (2001) Long–term followup of neurosarcoidosis. Neurology 57:927–929
Howard Jaster J, et al. (1997) Solitary spinal cord sarcoidosis without other manifestations of systemic sarcoidosis. Clinical Imaging 21:17–22
Hunninghake, et al. (1999) ATS/ERS/WASOG Statement on Sarcoidosis. Sarcoidosis vasculitis and diffuse lung diseases 16:149–173
Levivier M, et al. (1991) Sarcoidosis presenting as an isolated intramedullary tumor. Neurosurgery 29:271–276
Lower, et al. (1997) Diagnosis and management of neurological sarcoidosis. Arch Intern Med 155:395–420
Kelly, et al. (1988) MR demonstration of spinal cord sarcoidosis: report of a case. AJNR 9:197–199
Manà J, et al. (1994) Clinical factors predicting persistence of activity in sarcoidosis: a multivariate analysis of 193 cases. Respiration 61:219–225
Mayock, et al. (1963) Manifestations of sarcoidosis – analysis of 145 patients, with a review of nine series selected from the literature. Am J Med 35:67–89
Moller DR (1999) Cells and cytokines involved in the pathogenesis of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 16:24–31
Nowak, et al. (2001) Neurosarcoidosis: a review of its intracranial manifestation. J Neurol 248:363–372
Paramothayan, et al. (2002) Corticosteroid therapy in pulmonary sarcoidosis. JAMA 287:1301–1307
Stern, et al. (1985) Sarcoidosis and its neurological manifestations. Arch Neurol 42:909–917
Stern, et al. (1987) Neurosarcoidosis: cerebrospinal fluid lymphocyte subpopulations. Neurology 37:878–881
Sulavik SB, et al. (1990) Recognition of distinctive patterns of gallium–67 distribution in sarcoidosis. J Nucl Med 31:1909–1914
Takada K, et al. (1993) The clinical course and prognosis of patients with severe, moderate or mild sarcoidosis. J Clin Epidemiol 46:359–366
Vrethem, et al. (1998) CD4 and CD8 lymphocyte subsets in cerebrospinal fluid and peripheral blood from patients with multiple sclerosis, meningitis and normal controls. Acta Neurol Scand 97:215–220
Zajicek, et al. (1999) Central nervous system sarcoidosis – diagnosis and management. Q J Med 92:103–117
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Marangoni, S., Argentiero, V. & Tavolato, B. Neurosarcoidosis. J Neurol 253, 488–495 (2006). https://doi.org/10.1007/s00415-005-0043-5
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DOI: https://doi.org/10.1007/s00415-005-0043-5