Neurosarcoidosis: Phenotypes, Approach to Diagnosis and Treatment

Gosselin, Jeanne; Roy-Hewitson, Chantal; Bullis, Sean S. M.; DeWitt, John; Soares, Bruno P.; Dasari, Sidarth; Nevares, Alana

doi:10.1007/s11926-022-01089-z

Cited by 9 publications

(16 citation statements)

References 57 publications

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“…However, the 18 F-FDG PET/CT is unable to "see" the eye; therefore, to obtain a complete phenotype, an ophthalmologic examination is mandatory (71)(72)(73)(74)(75)(76)(77). Neurological and endocrinology evaluations are also indispensable for thoroughness to detect neurosarcoidosis, smallfiber neuropathy, and abnormal calcium metabolism (78)(79)(80)(81). Until further well-designed multicenter studies are performed to confirm the abovementioned findings as universal patterns of disease behavior, we do not consider this study an ideal destination for sarcoidosis and its assessment but rather treat it as just another journey ahead.…”

Section: F-fdg Pet/ct Scan Phenotypingmentioning

confidence: 99%

From Karl Wurm and Guy Scadding's staging to 18F-FDG PET/CT scan phenotyping and far beyond: perspective in the evading history of phenotyping in sarcoidosis

et al. 2023

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Sarcoidosis is an inflammatory granulomatous disease of unknown etiology involving any organ or tissue along with any combination of active sites, even the most silent ones clinically. The unpredictable nature of the sites involved in sarcoidosis dictates the highly variable natural history of the disease and the necessity to cluster cases at diagnosis based on clinical and/or imaging common characteristics in an attempt to classify patients based on their more homogeneous phenotypes, possibly with similar clinical behavior, prognosis, outcome, and therefore with therapeutic requirements. In the course of the disease's history, this attempt relates to the availability of a means of detection of the sites involved, from the Karl Wurm and Guy Scadding's chest x-ray staging through the ACCESS, the WASOG Sarcoidosis Organ Assessment Instruments, and the GenPhenReSa study to the 18F-FDG PET/CT scan phenotyping and far beyond to new technologies and/or the current “omics.” The hybrid molecular imaging of the 18F-FDG PET/CT scan, by unveiling the glucose metabolism of inflammatory cells, can identify high sensitivity inflammatory active granulomas, the hallmark of sarcoidosis—even in clinically and physiologically silent sites—and, as recently shown, is successful in identifying an unexpected ordered stratification into four phenotypes: (I) hilar–mediastinal nodal, (II) lungs and hilar–mediastinal nodal, (III) an extended nodal supraclavicular, thoracic, abdominal, inguinal, and (IV) all the above in addition to systemic organs and tissues, which is therefore the ideal phenotyping instrument. During the “omics era,” studies could provide significant, distinct, and exclusive insights into sarcoidosis phenotypes linking clinical, laboratory, imaging, and histologic characteristics with molecular signatures. In this context, the personalization of treatment for sarcoidosis patients might have reached its goal.

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Section: F-fdg Pet/ct Scan Phenotypingmentioning

confidence: 99%

From Karl Wurm and Guy Scadding's staging to 18F-FDG PET/CT scan phenotyping and far beyond: perspective in the evading history of phenotyping in sarcoidosis

et al. 2023

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“…The finding of bilateral adenopathy of the hilus with right paratracheal involvement on the chest X-ray makes sarcoidosis very likely. However, in patients with suspected neurosarcoidosis, a careful evaluation of systemic manifestations and neurological examination is required, since the diagnosis of neurosarcoidosis is not based on neurologic features alone [1][2][3][4].…”

Section: Diagnosis Of Neurosarcoidosismentioning

confidence: 99%

“…Sarcoidosis occurs worldwide and affects all races and age groups [1]. The clinical manifestation, natural course and prognosis of sarcoidosis vary widely [1][2][3][4].…”

Section: Introductionmentioning

confidence: 99%

“…The lymphatic system is almost always affected. At the onset of the disease, the lungs are affected in 95% of cases, and multisystem disease is present in about half of cases [1][2][3][4][5]. Neurological symptoms represent the first defining manifestation of sarcoidosis in almost 50% of cases [6,7].…”

Section: Introductionmentioning

confidence: 99%

“…Neurological symptoms represent the first defining manifestation of sarcoidosis in almost 50% of cases [6,7]. Neurosarcoidosis occurs in 5-10% of patients with sarcoidosis, and these rates are not influenced by race or gender [3][4][5]. The clinical manifestations of neurosarcoidosis are also heterogeneous, as granulomas can affect any part of the brain.…”

Section: Introductionmentioning

confidence: 99%

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Neurosarcoidosis: The Presentation, Diagnosis and Treatment Review of Two Cases

Basheer,

Waked,

Jeries

et al. 2023

Life

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Sarcoidosis is a chronic granulomatous disease of unknown cause characterized by the presence of non-caseating granulomas. The disease can affect any organ including the nervous system. Neurosarcoidosis occurs in about 5% patients with sarcoidosis. The clinical presentation of neurosarcoidosis is varied, and it can involve the brain, spinal cord and peripheral nervous system, separately or in different combinations. The diagnosis of neurosarcoidosis is challenging, as biopsies from the nervous system are not readily available. Anti-TNFα agents are becoming one of the cornerstone treatments for neurosarcoidosis. In this case-based review, we discuss two cases of neurosarcoidosis with different clinical presentations. The first patient presented with confusion, while the second presented with walking difficulty and neurogenic bladder. Both patients were treated with methylprednisolone pulse therapy with rapid, but non-complete, improvement. Therefore, infliximab was initiated in both cases with subsequent improvement in the clinical manifestations and imaging findings, emphasizing the effectiveness and safety of infliximab in cases of severe neurosarcoidosis. In conclusion, the goal of neurosarcoidosis management is to prevent organ system damage and minimize the toxic cumulative adverse effects of glucocorticoid use. In this case-based review we discuss the various presentations, the diagnosis and the treatment of neurosarcoidosis.

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