Difficulties in the differentiation of chronic inflammatory diseases of the central nervous system - value of cerebrospinal fluid analysis and immunological abnormalities in the diagnosis

Reske, D.; Petereit, Hela-Felicitas; Heiss, Wolf‐Dieter

doi:10.1111/j.1600-0404.2005.00414.x

Cited by 58 publications

(56 citation statements)

References 61 publications

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“…This is in line with observations from other groups: neither serum ACE nor calcium or ESR is useful to establish the diagnosis of neurosarcoidosis (69,70). Cerebrospinal fluid findings indicative of a chronic inflammatory disease have a much higher diagnostic yield in neurosarcoidosis (71). An important part of the initial CSF examination is to rule out infectious CNS disease and malignancy.…”

Section: Laboratory Testssupporting

confidence: 64%

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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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Section: Laboratory Testssupporting

confidence: 64%

“…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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“…Apart from the relatively low sensitivity, the detection of OCB seemed to have a low specificity, mainly when a blind method of analysis was used 7,8 . OCB have been described in other inflammatory diseases, their frequency of detection ranging from 25 to 80% 5,7,19 , as well as in noninflammatory diseases of the CNS (in up to 17% of patients 15 ). However, there is no consensus about this low specificity of OCB in MS. 9,20,21 .…”

Section: Resultsmentioning

confidence: 99%

“…sions, characterization of the local immune response and differential diagnosis with other neurological diseases 2,5,6 .…”

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confidence: 99%

Study of oligoclonal bands restricted to the cerebrospinal fluid in multiple sclerosis patients in the city of São Paulo

Gama

Machado

Livramento

et al. 2009

Arq. Neuro-Psiquiatr.

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-The frequency of oligoclonal bands (OCB) restricted to the cerebrospinal fluid (CSF) from patients with multiple sclerosis (MS) varies widely in different populations. The objective of this study was to determine the frequency of these OCB in a group of MS patients in the city of São Paulo. Techniques used to detect OCB consisted of isoelectric focusing followed by immunoblotting. Oligoclonal bands were found in 49 (54.4%) out of 90 patients with clinically definite MS; in (31.2%) of the 16 patients with clinically isolated syndrome; in 7 (17.9%) of 39 patients with inflammatory disorders of the central nervous system (IDCNS), and in none of the individuals with no neurological condition (control group). The specificity of the method was 100% when compared to the control group and 82.1% when compared to the IDCNS group. These results suggest that the frequency of CSF OCB is much lower in Brazilian MS patients from São Paulo city than that reported in MS series from Caucasian populations.KEY WORDS: multiple sclerosis, cerebrospinal fluid, oligoclonal bands, Brazilian population. estudo de bandas oligoclonais restritas ao líquido cefalorraquidiano em pacientes com esclerose múltipla na cidade de São paulo resumo -A frequência da detecção de bandas oligoclonais (BOC) restritas ao líquido cerebrorraquidiano (LCR) em pacientes com esclerose múltipla (EM) varia amplamente em diferentes populações. O objetivo deste estudo foi determinar a frequência destas BOC em pacientes com EM em amostra de população da cidade de São Paulo. A técnica utilizada para a detecção das BOC foi a focalização isoelétrica, seguida do immunoblotting. BOC foram detectadas: em 49 (54,4%) de 90 pacientes com EM clinicamente definida; em 5 (31,2%) de 16 pacientes com síndrome clínica isolada; em 7 (17,9%) de 30 pacientes com doenças inflamatórias do sistema nervoso central (DISNC); e em nenhum indivíduo sem doença neurológica. A especificidade do método foi 100% quando comparada ao grupo controle e 82,1% quando comparada ao grupo de DISNC. Estes resultados sugerem que a freqüência de BOC no LCR é mais baixa em pacientes da cidade de São Paulo portadores de EM do que aquelas descritas em populações caucasianas. PALAVRAS-CHAVE: esclerose múltipla, líquido cefalorraquidiano, bandas oligoclonais, população brasileira.

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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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Difficulties in the differentiation of chronic inflammatory diseases of the central nervous system - value of cerebrospinal fluid analysis and immunological abnormalities in the diagnosis

Abstract: Here we compare typical clinical and CSF findings in MS, neurosarcoidosis, neurolupus, neuro-Behçet and nervous system involving SS with special emphasis on those findings allowing differentiation of the respective diseases by reviewing the literature.

Cited by 58 publications

References 61 publications

Challenges in the diagnosis and treatment of neurosarcoidosis

Challenges in the diagnosis and treatment of neurosarcoidosis

Study of oligoclonal bands restricted to the cerebrospinal fluid in multiple sclerosis patients in the city of São Paulo

Clinically Isolated Neurosarcoidosis: A Recommended Diagnostic Path

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