Neurosarcoidosis

Cohort studies of large series of patients with sarcoidosis over a long period of time are scarce. The aim of this study is to report a 40-year clinical experience of a large series of patients at Bellvitge University Hospital, a tertiary university hospital in Barcelona, Spain. Diagnosis of sarcoidosis required histological confirmation except in certain specific situations. All patients underwent a prospective study protocol. Clinical assessment and follow-up of patients were performed by a multidisciplinary team.From 1976 to 2015, 640 patients were diagnosed with sarcoidosis, 438 of them (68.4%) were female (sex ratio F/M 2:1). The mean age at diagnosis was 43.3 ± 13.8 years (range, 14–86 years), and 613 patients (95.8%) were Caucasian. At diagnosis, 584 patients (91.2%) showed intrathoracic involvement at chest radiograph, and most of the patients had normal pulmonary function. Erythema nodosum (39.8%) and specific cutaneous lesions (20.8%) were the most frequent extrapulmonary manifestations, but there was a wide range of organ involvement. A total of 492 patients (76.8%) had positive histology. Follow-up was carried out in 587 patients (91.7%), over a mean of 112.4 ± 98.3 months (range, 6.4–475 months). Corticosteroid treatment was administered in 255 patients (43.4%), and steroid-sparing agents in 49 patients (7.7%). Outcomes were as follows: 111 patients (18.9%) showed active disease at the time of closing this study, 250 (42.6%) presented spontaneous remission, 61 (10.4%) had remission under treatment, and 165 (28.1%) evolved to chronic sarcoidosis; among them, 115 (19.6%) with mild disease and 50 (8.5%) with moderate to severe organ damage. A multivariate analysis showed that at diagnosis, age more than 40 years, the presence of pulmonary involvement on chest radiograph, splenic involvement, and the need of treatment, was associated with chronic sarcoidosis, whereas Löfgren syndrome and mediastinal lymphadenopathy on chest radiograph were indicators of good outcome.Sarcoidosis is a multisystem disease with protean clinical-radiographic manifestations. Although almost half of patients follow a spontaneous resolution or under treatment, a significant number of them may have several degrees of organ damage. This study emphasizes the value of a multidisciplinary approach and long-term follow-up by specialized teams in sarcoidosis.

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“…Peripheral neuropathy is uncommon and small fiber neuropathy causing disabling pain is being increasingly recognized. [54] …”

Section: Discussionmentioning

confidence: 99%

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis

et al. 2017

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“…Diagnosis of neurosarcoidosis can be challenging, as this condition may mimic other inflammatory, infective or neoplastic disorders. The individual presented here was diagnosed with probable spinal neurosarcoidosis using the modified Zajicek criteria on the basis of characteristic signs of central nervous system (CNS) inflammation on magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) analysis, coupled with positive histology from a systemic lesion, and a positive high‐resolution CT chest and 18 F‐FDG PET scan.…”

Section: Introductionmentioning

confidence: 99%

Application of the modified Zajicek criteria to diagnose probable spinal cord neurosarcoidosis

Owen

Jabeen

Bhattacharjee

2018

Clinical Case Reports

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“…Histopathological biopsy of the organ affected by the disease process identifies the characteristic non‐caseating granulomata (in about 80%) following exclusion of other causes of granuloma formation, primarily tuberculosis . Neurosarcoidosis is known as “the great mimicker” because of its vast range of MRI appearances, which include a tumor‐like mass, periventricular white matter lesions, and focal or diffuse leptomeningeal disease . Therefore, the evidence of neural and systemic sarcoidosis is critical for the right diagnosis of neurosarcoidosis.…”

Section: Discussionmentioning

confidence: 99%

Propionibacterium acnes‐associated neurosarcoidosis: A case report with review of the literature

et al. 2017

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A 56-year-old woman with a 3-year history of hydrocephalus and ventriculo-peritoneal shunt placement, presented with worsening altered level of consciousness for 2 days. Imaging studies showed severe ventriculomegaly involving the lateral and third ventricles with multiple septated cysts noted in the lateral ventricles predominantly near the frontal horns. Histopathologic examination of the excised brain lesion revealed choroid plexus tissue and adjacent cerebral parenchyma with several non-caseating granulomas. Granulomatous inflammation was also identified in mediastinal lymph nodes. By using specific monoclonal antibodies, Propionibacterium acnes (P. acnes) were detected in non-caseating granulomas of both the brain and mediastinal lymph nodes. No acid-fast bacilli or fungal elements were present. To the best of our knowledge, this is the first demonstration of P. acnes in sarcoid granulomas of cerebral tissue, and it reinforces the possible link between P. acnes and sarcoidosis.

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Neurosarcoidosis

Cited by 52 publications

References 43 publications

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis

Application of the modified Zajicek criteria to diagnose probable spinal cord neurosarcoidosis

Propionibacterium acnes‐associated neurosarcoidosis: A case report with review of the literature

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