NEUROSARCOIDOSIS - A PERSONAL PERSPECTIVE BASED ON THE STUDY OF 37 PATIENTS

Clinically apparent involvement of the nervous system occurs in a rela tively small number of patients with sarcoidosis. The diagnosis of neu rosarcoidosis is often difficult and particularly so in patients who l ark either pulmonary or systemic manifestations of sarcoidosis. Furthe rmore, clinical features of neurosarcoidosis are extremely variable. I n this series of 37 patients, seen during the last 30 years, cranial n erve palsies occurred in 52%, polyneuritis or polyneuropathy, in 24%, meningeal involvement in 24%, muscle disease in 8%, and Guillain-Barre syndrome in 5% of the patients. Other presentations included seizures , brain mass, pituitary/hypothalamic, pothalamic syndrome, and memory loss associated with confusion. The chest radiograph was abnormal in 8 of every 10 patients with neurosarcoidosis. In 18 (85%) of 21 patient s, gallium uptake was consistent with the diagnosis of active sarcoido sis. Serum angiotensin-converting enzyme levels were raised in about h alf of the patients. Cerebrospinal fluid features, including lymphocyt e pleocytosis, raised protein levels, and decreased glucose concentrat ion, were of little help. MRI with gadolinium enhancement was the most sensitive diagnostic tool, particularly in patients with meningeal in volvement. The ultimate arbiter of the diagnosis of neurosarcoidosis, the presence of noncaseating granulomas in the involved tissue, was no t always available. Although corticosteroids are the mainstay of thera py, in this series, 12 patients received chloroquine or hydroxychloroq uine. Prognosis of chronic neurosarcoidosis is poor. Six (18%) of 37 p atients died of complications related to sarcoidosis.