Background: Affection of the nervous system is one of the most serious forms of sarcoidosis. The objective of the present work was to evaluate the prevalence and manifestations of this disease, contemporary opportunities of its diagnosis and the importance of early adequate therapy.
Methods and results: Neurosarcoidosis was diagnosed in seven patients (4 men and 3 women) from a total number of 211 patients with sarcoidosis in 1977 to 1993. The mean age of the patients with neurosarcoidosis was 47 years. In six patients affection of the nervous system was the first clinical manifestation of the disease. In four patients it was paresis of the facial nerve, in two instances diabetes insipidus, in two instances affection of the spinal cord with subsequent spastic triparesis or paraparesis of the lower extremities, in one instance the disease was manifested by neuralgia of the trigeminal nerve. The serious character of the disease is apparent from the fact that only in two instances complete regression of symptoms occurred. In three patients regression was partial, in one instance the affection persisted without changes and in one instance it progressed to metabolic breakdown with a fatal outcome. In the differential diagnosis examination of the nervous system by computed tomography, magnetic resonance and possibly perimyelography proved to be helpful. In all instances there was a typical finding in cerebrospinal fluid. Lymphomonocytic pleocytosis was present, a raised protein content, elevated gammaglobulin levels. The diagnosis was assisted by radiograms of the chest and biopsy. The latter was made from intrathoracic or subcutaneous nodes, in one instance from muscle. As auxiliary methods the following were used: tuberculin test, bronchoalveolar lavage, pulmonary gallium scintigraphy, serum level of angiotensin converting enzyme and immunological examination.
Conclusions: The authors emphasize the necessity of early diagnosis and intensive initial treatment with corticoids or antimalaria drugs, which may prevent the development of irreversible changes. Magnetic resonance appears to be the most promising examination method in neurosarcoidosis.