Chest
Volume 112, Issue 1, July 1997, Pages 220-228
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Neurosarcoidosis: A Personal Perspective Based on the Study of 37 Patients

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Clinically apparent involvement of the nervous system occurs in a relatively small number of patients with sarcoidosis. The diagnosis of neurosarcoidosis is often difficult and particularly so in patients who lack either pulmonary or systemic manifestations of sarcoidosis. Furthermore, clinical features of neurosarcoidosis are extremely variable. In this series of 37 patients, seen during the last 30 years, cranial nerve palsies occurred in 52%, polyneuritis or polyneuropathy in 24%, meningeal involvement in 24%, muscle disease in 8%, and Guillain-Barré syndrome in 5% of the patients. Other presentations included seizures, brain mass, pituitary/hypothalamic 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 patients, gallium uptake was consistent with the diagnosis of active sarcoidosis. Serum angiotensin-converting enzyme levels were raised in about half of the patients. Cerebrospinal fluid features, including lymphocyte pleocytosis, raised protein levels, and decreased glucose concentration, were of little help. MRI with gadolinium enhancement was the most sensitive diagnostic tool, particularly in patients with meningeal involvement. The ultimate arbiter of the diagnosis of neurosarcoidosis, the presence of noncaseating granulomas in the involved tissue, was not always available. Although corticosteroids are the mainstay of therapy, in this series, 12 patients received chloroquine or hydroxychloroquine. Prognosis of chronic neurosarcoidosis is poor. Six (18%) of 37 patients died of complications related to sarcoidosis.

Section snippets

Clinical Material

Between January 1965 and December 1995, 37 patients, principally at Los Angeles County-University of Southern California Medical Center and University of Southern California-University Hospital, both in Los Angeles, presented with neurosarcoidosis. These patients had clinical and histologic evidence of multisystem sarcoidosis. In addition to neurologic involvement, there was evidence of intrathoracic involvement in 29 (78%) patients, ocular disease in eight (22%), cutaneous sarcoidosis in five

Discussion

The diagnosis of neurosarcoidosis requires a compatible clinical or radiologic picture of sarcoidosis and histologic confirmation of noncaseating granulomas or a positive Kveim-Stilzbach test. The presence of one without the other may lead to misdiagnosis. Although the chest radiograph is abnormal in >90% of patients with sarcoidosis and is the first tool of diagnostic investigation when sarcoidosis is considered, in this study, the chest radiograph was abnormal in only 78% of the patients. In

Conclusion

Based on the analysis of 37 cases of neurosarcoidosis, the following caveats are appropriately pragmatic and useful.

(1) Any part of the CNS may be affected by sarcoidosis. (2) When the nervous system lesions occur in the absence of other tissue involvement, the diagnostic process is arduous. (3) Serum and CSF ACE levels,67 gallium scanning, CT, MRI, position imaging tomography, visual and auditory evoked potentials, and BAL may all be helpful, but in selected cases in which the possibility of

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    revision accepted December 11.

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