Neurosarcoidosis

Stern, Barney J.; Griffin, D. E.; Luke, Robert A.; Krumholz, Allan; Johns, Carol J.

doi:10.1212/wnl.37.5.878

Cited by 52 publications

(27 citation statements)

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“…While activated CD4+ T- cells are the hallmark of the sarcoid granuloma immunopathogenesis and the CD4/CD8 T-cell ratio typically increased in bronchoalveolar lavage of sarcoidosis patients, the diagnostic utility of this test for neurosarcoidosis remains uncertain. In a previous case series of 8 patients, it was increased only in three and normal in five [14,15,16]. Sarcoid-induced neuropathy is rare, but may be a presenting symptom of neurosarcoidosis [3,17,18,19].…”

Section: Discussionmentioning

confidence: 99%

Clinically Isolated Neurosarcoidosis: A Recommended Diagnostic Path

Wegener¹,

Linnebank²,

Martin³

et al. 2014

Eur Neurol

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The involvement of the central nervous system in sarcoidosis can manifest with a variety of neurological symptoms, most of them nonspecific. We identified 13 patients with neurosarcoidosis diagnosed at our clinic. Six of 13 patients presented with clinically isolated neurosarcoidosis (CINS) without signs or symptoms of systemic disease. CINS patients were not different with respect to age, as well as imaging and spinal fluid findings, or disease course. However, we found spinal cord involvement in neurosarcoidosis patients much more common than previously described (in 8 out of 13 patients). Spinal cord affection was associated with older age at diagnosis and a less favorable response to therapy. Based on our findings, we propose a diagnostic path for neurosarcoidosis, including spinal magnetic resonance imaging (MRI) as a mandatory and early step during diagnostic workup. i 2014 S. Karger AG, Basel

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Section: Discussionmentioning

confidence: 99%

Clinically Isolated Neurosarcoidosis: A Recommended Diagnostic Path

Wegener¹,

Linnebank²,

Martin³

et al. 2014

Eur Neurol

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“…The CD4/CD8 T-cell ratio is typically increased in bronchoalveolar lavage of sarcoidosis patients (74). In neurosarcoidosis, the diagnostic utility of this test is uncertain (75,76).…”

Section: Laboratory Testsmentioning

confidence: 99%

Challenges in the diagnosis and treatment of neurosarcoidosis

et al. 2015

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The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. Clinical and imaging findings are often non-specific since they can be found in a large number of neurological disorders. Histopathology can also be confounding if not performed by an expert pathologist and not placed in an appropriate clinical context. In this review, we discuss clinical features, laboratory findings, imaging, and histology of neurosarcoidosis, and we report current evidence regarding drug therapy. We conclude that a correct diagnostic approach should include a multidisciplinary evaluation involving clinicians, radiologists, and pathologists and that future studies should evaluate the genetic signature of neurosarcoidosis as they could be helpful in the assessment of this uncommon disease. With head-to-head comparisons of medical treatment for neurosarcoidosis still lacking due to the rarity of the disease and an increasing number of immunomodulating therapies at hand, novel therapeutic approaches are to be expected within the next few years. Key messages Neurosarcoidosis is a rare disorder that affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies, revealing that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. A multidisciplinary evaluation is useful to achieve a correct diagnosis because clinical and imaging findings are often non-specific. Corticosteroids are the first-line treatment for neurosarcoidosis, followed by steroid-sparing immune-modulating agents if prednisone therapy is insufficient. Dear Author, Please check these proofs carefully. It is the responsibility of the corresponding author to check against the original manuscript and approve or amend these proofs. A second proof is not normally provided. Informa Healthcare cannot be held responsible for uncorrected errors, even if introduced during the composition process. The journal reserves the right to charge for excessive author alterations, or for changes requested after the proofing stage has concluded.The following queries have arisen during the editing of your manuscript and are marked in the margins of the proofs. Unless advised otherwise, submit all corrections using the CATS online correction form. Once you have added all your corrections, please ensure you press the ''Submit All Corrections'' button.Please review the table of contributors below and confirm that the first and last names are structured correctly and that the authors are listed in the correct order of contribution. The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percenta...

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“…While deep cerebral sarcoid lesions and distal cranial neuropathies often do not show CSF immunocytologic abnormalities, granuloma formation near subarachnoid spaces would have enhanced CSF T4 lymphocyte subpopulation activity. In one series, 3 only three of eight neurosarcoid patients showed an elevated CSF T4:T8 ratio, yet the ratio was increased in two of three patients with mass lesions. Intramedullary spinal sarcoidosis may be especially prone to elevations in the CSF T4:T8 ratio due to granulomas abutting CSF.…”

Section: Com/scmentioning

confidence: 90%

“…2 Small studies suggest an elevated ratio of CSF CD4:CD8 þ (T helper:T suppressor or T4:T8) lymphocytes in neurosarcoidosis. 3 We present a patient with sarcoid myelopathy whose clinical and radiographic diagnosis and treatment response correlated with the CSF immune profile.…”

Section: Introductionmentioning

confidence: 97%