Clinical characterization and outcomes of 85 patients with neurosarcoidosis

Abstract

To analyze the frequency and clinical phenotype of neurosarcoidosis (NS) in one of the largest nationwide cohorts of patients with sarcoidosis reported from southern Europe. NS was evaluated according to the Diagnostic Criteria for Central Nervous System and Peripheral Nervous System Sarcoidosis recently proposed by Stern et al. Pathologic confirmation of granulomatous disease was used to subclassify NS into definite (confirmation in neurological tissue), probable (confirmation in extraneurological tissue) and possible (no histopathological confirmation of the disease). Of the 1532 patients included in the cohort, 85 (5.5%) fulfilled the Stern criteria for NS (49 women, mean age at diagnosis of NS of 47.6 years, 91% White). These patients developed 103 neurological conditions involving the brain (38%), cranial nerves (36%), the meninges (3%), the spinal cord (10%) and the peripheral nerves (14%); no patient had concomitant central and peripheral nerve involvements. In 59 (69%) patients, neurological involvement preceded or was present at the time of diagnosis of the disease. According to the classification proposed by Stern et al., 11 (13%) were classified as a definite NS, 61 (72%) as a probable NS and the remaining 13 (15%) as a possible NS. In comparison with the systemic phenotype of patients without NS, patients with CNS involvement presented a lower frequency of thoracic involvement (82% vs 93%, q = 0.018), a higher frequency of ocular (27% vs 10%, q < 0.001) and salivary gland (15% vs 4%, q = 0.002) WASOG involvements. In contrast, patients with PNS involvement showed a higher frequency of liver involvement (36% vs 12%, p = 0.02) in comparison with patients without NS. Neurosarcoidosis was identified in 5.5% of patients. CNS involvement prevails significantly over PNS involvement, and both conditions do not overlap in any patient. The systemic phenotype associated to each involvement was clearly differentiated, and can be helpful not only in the early identification of neurological involvement, but also in the systemic evaluation of patients diagnosed with neurosarcoidosis.

High-resolution ultrasound of peripheral neuropathies in rheumatological patients: An overview of clinical applications and imaging findings

“…The two clinical entities differ for anatomo-pathologic features and clinical presentation. The GN occurs in 1% of SA patients and usually manifest as distal, symmetric or asymmetric axonal sensory motor polyneuropathy ( 123 ). Less commonly, CIDP pattern or motor multifocal pattern with electromyographic findings of demyelination or conduction blocks are reported ( 124 ).…”

Multiorgan sarcoidosis as a diabetes insipidus mask

“…Sarcoidosis (also known as Besnier–Boeck–Schaumann disease, BBS disease) is an inflammatory disease characterized by noncaseating granulomas in the affected organs ( 1 , 2 , 3 ). The estimated incidence of BBS disease ranges between 1 and 40 cases per 100 000 individuals ( 4 ). Many studies have assessed the immunopathogenesis of sarcoidosis, but the exact stimulus that induces the disease remains elusive.…”

“…The formation of sarcoid granulomas involves macrophages, epithelioid cells, multinucleated giant cells, and T and B lymphocytes ( 1 , 2 , 3 , 7 ). BBS disease is usually diagnosed in young and middle-aged adults ( 1 , 3 , 4 , 8 ). An initial assessment of all patients with suspected sarcoidosis should include a careful examination of all potentially affected organs and systems ( 6 ).…”

“…An initial assessment of all patients with suspected sarcoidosis should include a careful examination of all potentially affected organs and systems ( 6 ). The lungs are involved in approximately 90% of patients ( 2 , 4 , 6 ). The extrapulmonary manifestations of sarcoidosis are estimated in 30% of cases ( 2 ).…”

“…Other commonly impaired organs are the lymph nodes, skin, eyes, liver, spleen, and heart ( 1 , 6 , 8 ). Neurosarcoidosis (NS) occurs in 3–20% of patients with systemic sarcoidosis ( 4 , 5 , 8 , 9 ) and may involve the cranial nerves, meninges, brain parenchyma (especially the hypothalamic region), spinal cord, peripheral nerves, and muscles ( 3 , 4 , 10 ). The endocrine signs of sarcoidosis include diabetes insipidus (DI), hypothalamic hypopituitarism, amenorrhea–galactorrhea, hypogonadotropic hypogonadism, secondary hypothyroidism, and diffuse goiter or a solitary thyroid nodule ( 5 , 8 , 10 ).…”