PURPOSE: Neurosarcoidosis is a rare manifestation of sarcoidosis, is difficult to diagnose and manage, and is a significant cause of morbidity and mortality. As such, racial differences in neurosarcoidosis are not clearly defined.
METHODS: Records were retrospectively reviewed from 2 hospital settings in Indianapolis. Wishard Memorial Hospital records were obtained after a search for appropriate ICD-9 codes. Records of patients treated at the Indiana University sarcoid referral clinic were also reviewed. Scoring systems were modified to record neurologic involvement and response to treatment.
RESULTS: 39 patients had neurosarcoidosis. Neurosarcoidosis was the sole manifestation in 7.7%. Peripheral neuropathy was the most common manifestation. Cranial nerve neuropathy was seen in 46% of patients. MRI of the brain correlated with clinical findings in most cases. CSF analysis showed increased protein and lymphocytosis in eleven of seventeen patients. CSF ACE levels were elevated in 4 of 8 patients. Nervous system biopsies were infrequently performed. Patients receiving combination therapy had a good response to treatment in 53% of cases while prednisone monotherapy led to a good clinical response in 36% of patients. Cranial nerve involvement had a good response to treatment (70.7%). However, only 10% of patients with CN 8 lesions improved. African Americans had significantly more CNS involvement than caucasians (75% vs 45%, p = 0.05), with similar treatment response.
CONCLUSION: Neurosarcoidosis is difficult to diagnose and treat effectively. Objective testing may not be conclusive, and patients with peripheral neuropathy are likely underreported. Neurosarcoid patients respond best to combination therapy, but may have a poor response despite maximizing therapy. African Americans may be particularly prone to CNS disease.
CLINICAL IMPLICATIONS: We should be vigilant in seeking neurologic symptoms in sarcoid patients as neurosarcoidosis can be overlooked and is an indication for early combination therapy.
DISCLOSURE: Khalil Diab, None.