Overview of neurosarcoidosis: recent advances

Hebel, Renata; Dubaniewicz-Wybieralska, Mirosława; Dubaniewicz, Anna

doi:10.1007/s00415-014-7482-9

Cited by 93 publications

(85 citation statements)

References 54 publications

(124 reference statements)

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“…In this regard, there is increasing evidence of a specific genetic predisposition to a selective sarcoid involvement of the nervous system in some patients (26)(27)(28). Caucasians present more often with manifestations deriving from peripheral nervous system involvement than do African-Americans, and patients with small fiber neuropathy have a high frequency of HLA-DQB1*0602 antigen and non-HLA polymorphic gene occurrence (26).…”

Section: Etiology Of Sarcoidosis and Selective Involvement Of The Nermentioning

confidence: 99%

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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: Etiology Of Sarcoidosis and Selective Involvement Of The Nermentioning

confidence: 99%

“…Caucasians present more often with manifestations deriving from peripheral nervous system involvement than do African-Americans, and patients with small fiber neuropathy have a high frequency of HLA-DQB1*0602 antigen and non-HLA polymorphic gene occurrence (26).…”

Section: Etiology Of Sarcoidosis and Selective Involvement Of The Nermentioning

confidence: 99%

Challenges in the diagnosis and treatment of neurosarcoidosis

et al. 2015

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“…Akciğer tutulumu olmaksızın sadece sinir sisteminde sarkoidoz görülmesi %1 oranında söz konusudur. [76] Buna karşın, nö-ro-sarkoidoz olgularının yaklaşık %50'sinde ön planda nö-rolojik tutulum görülmektedir. [72] Sarkoidoz tanısı genellikle histopatolojik doğrulamaya dayanmaktadır; bunun yapıla-madığı durumlarda tanı güçlükleri ile karşılaşılabilmektedir.…”

Section: Sarkoidozunclassified

“…Hastalık seyri içinde obstrüktif hidrosefali, poliradikülopati ve farklı tiplerde polinöropati veya miyopati de gelişebilir. Epileptik nöbetler görece seyrektir; hastaların yaklaşık %15'inde epileptik nöbetler görülmektedir ve bunun daha kötü prognozla ilişkili olduğu düşünülmektedir; [72,76] çocuklarda epileptik nöbetler biraz daha sık görülür. [72] Nöro-sarkoidoz hastalığı tedavisinde yüksek doz iv.…”

Section: Sarkoidozunclassified

Autoimmune Diseases Associated with Epilepsy

Kürtüncü¹,

Altunrende²,

Akman‐Demir³

2015

Epilepsi

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“…It is characterized by granulomatous inflammation with unclear etiology. Several theories have been postulated in literature, with evidence pointing towards an infectious etiology, genetic factors, environmental triggers, as well as hormonal dysregulation [1]. Anecdotal data suggests that certain infections like Mycobacterium tuberculosis, Propionibacteria acne, Mycoplasma pneumoniae have been mentioned in the literature [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

Neurosarcoidosis Flare Following Urinary Tract Infection with Extended Spectrum Beta Lactamase Producing Escherichia coli

Sinha¹

2017

MOJCR

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Neurosarcoidosis affects 5%-10% of Sarcoidosis patients. The etiology of a sarcoidosis flare has been unknown. Several factors have been implicated in the pathogenesis of an acute flare, including genetic, environmental and infectious agents. We report the case of a 52-year-old female with known neurosarcoidosis who presented with altered sensorium on two occasions. On both episodes she had leptomeningeal enhancements on imaging consistent with a neurosarcoidosis flare, presumably secondary to Extended Spectrum Beta Lactamase producing (ESBL) Escherichia Coli in the urine.

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Overview of neurosarcoidosis: recent advances

Cited by 93 publications

References 54 publications

Challenges in the diagnosis and treatment of neurosarcoidosis

Challenges in the diagnosis and treatment of neurosarcoidosis

Autoimmune Diseases Associated with Epilepsy

Neurosarcoidosis Flare Following Urinary Tract Infection with Extended Spectrum Beta Lactamase Producing Escherichia coli

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