Objective:

To characterize the cerebrospinal fluid (CSF) profile in neurosarcoidosis and its variability across disease phenotypes. 

Background:

While CSF profiles in neurosarcoidosis typically include a lymphocytic pleocytosis, elevated protein, and hypoglycorrhachia, it is not known if the location of inflammation influences the probability of an inflammatory profile.   

Design/Methods:

In this single institution, retrospective study, the CSF profiles of adult patients with definite, probable, or possible neurosarcoidosis were analyzed.  

Results:

35 patients (median age of onset 46 years; female 21/35; Black 27/35) were included, most of whom (31/35) had systemic sarcoidosis. Disease was most commonly localized to the cranial leptomeninges (24/35), cranial nerves (17/35), hemispheric parenchyma (12/35), spinal leptomeninges (9/35), and cranial pachymeninges (8/35). Most (26/35) had at least one routine abnormality in the CSF: pleocytosis in 20/28 (range 0-100 cells/mm3; lymphocytic in 15/20), elevated protein in 24/34 (range 16-275 mg/dL), hypoglycorrhachia in 12/34 (range 15-114 mg/dL), elevated ACE level in 4/11, and oligoclonal bands in 4/14. Phenotypes with direct CSF contact usually exhibited a pleocytosis: spinal leptomeningitis (7/7), cranial neuropathies (13/15), cranial lepto- (15/18) and pachymeningitis (5/7), and cauda equina disease (4/5). While a pleocytosis was uniform in brain parenchymal disease (hemispheric 8/9, brainstem 5/5, and cerebellar 2/2), it was less common in spinal parenchymal disease (cervical 2/5, thoracic 1/3). A pleocytosis was most commonly present with inflammation in both the cerebral and spinal compartments (8/10), rather than in isolation (cranial 12/21, spinal 0/4). Similarly, hypoglycorrhachia was most frequently present with inflammation in both compartments (8/12) compared to in isolation (cranial 4/17, spinal 0/6).  

Conclusions:

Inflammatory CSF profiles are more likely in phenotypes directly exposed to the CSF. Despite the lumbar location of CSF sampling, CSF inflammation was more commonly present in those with widespread inflammation and cranial phenotypes rather than ones isolated to the spinal compartment.