Neurosarcoidosis

Chapelon, C.; Jm, Ziza; Jc, Piette; Levy, Yaron Y.; Raguin, G; Wechsler, B; Mo, Bitker; Blëtry, O.; Laplane, D; Mg, Bousser

doi:10.1097/00005792-199009000-00001

Cited by 277 publications

(17 citation statements)

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“…Four studies included patients with definite or probable neurosarcoidosis according to these criteria, and seven included definite, probable, or possible neurosarcoidosis [8, 9, 25, 27–31, 33, 35, 36]. Eleven studies included biopsy-proven sarcoidosis [6, 11, 14, 16, 18, 20–23, 32, 34]. Five studies included patients with the diagnosis based on either histological evidence of non-caseating granulomas or clinical and radiologic findings consistent with the diagnosis sarcoidosis [13, 15, 17, 19, 24].…”

Section: Resultsmentioning

confidence: 99%

Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

2016

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BackgroundNeurosarcoidosis is a rare variant of sarcoidosis and is only described in small cohort studies. We define clinical features, treatment and outcome of patients with neurosarcoidosis over the last 35 years.MethodsWe performed a systematic review and meta-analysis of studies on neurosarcoidosis published between 1980 and 2016. Studies were included if they reported at least 5 cases. Studies describing one specific neurological presentation were excluded.ResultsWe identified 29 articles describing 1088 patients diagnosed between 1965 and 2015. Neurosarcoidosis occurred in 5% of patients with systemic sarcoidosis. Mean age at presentation was 43 years and neurological symptoms were the first clinical manifestation of sarcoidosis in 52%. The most commonly reported feature of neurosarcoidosis was cranial neuropathy in 55%, with the facial and optic nerve most commonly affected, followed by headache in 32%. Pleiocytosis and elevated CSF protein were found in 58 and 63%. MRI of the brain showed abnormalities in 70%. Chest X-ray, chest CT, or gallium-67-scintigraphy showed findings consistent with sarcoidosis in 60%, 70% and 69%, respectively. First line therapy with corticosteroids was initiated in 434 of 539 patients (81%). Second and third line therapy was started in 27 and 9%. Outcome consisted of complete remission in 27%, incomplete remission in 32%, stable disease in 24%, deterioration in 6% and death in 5%.ConclusionNeurosarcoidosis has a heterogeneous clinical presentation and the diagnosis can be difficult because of low sensitivity of ancillary investigations. New treatments have emerged, but nevertheless one third of patients do not respond to treatment. Prospective cohort studies and RCTs on treatment are urgently needed.Electronic supplementary materialThe online version of this article (doi:10.1186/s12883-016-0741-x) contains supplementary material, which is available to authorized users.

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Section: Resultsmentioning

confidence: 99%

Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

2016

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“…Where patients are symptomatic, the presentation is usually subacute or chronicTypical CSF finds are: pleocytosis (<220 cells/mm 3 ) with a lymphocytic predominance and/or raised protein (<4.3 g/l). Reduced CSF glucose is also reportedConus or cauda equina syndrome [9, 19, 43]This may be of acute or subacute onset. CSF and imaging abnormalities usually confirm a neuro-inflammatory basisCranial neuropathy [2, 6, 8, 18–22, 24]This is the most frequently reported manifestation of NS.…”

Section: Diagnosismentioning

confidence: 99%

Neurosarcoidosis: a clinical approach to diagnosis and management

2016

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Sarcoidosis is a rare but important cause of neurological morbidity, and neurological symptoms often herald the diagnosis. Our understanding of neurosarcoidosis has evolved from early descriptions of a uveoparotid fever to include presentations involving every part of the neural axis. The diagnosis should be suspected in patients with sarcoidosis who develop new neurological symptoms, those presenting with syndromes highly suggestive of neurosarcoidosis, or neuro-inflammatory disease where more common causes have been excluded. Investigation should look for evidence of neuro-inflammation, best achieved by contrast-enhanced brain magnetic resonance imaging and cerebrospinal fluid analysis. Evidence of sarcoidosis outside the nervous system should be sought in search of tissue for biopsy. Skin lesions should be identified and biopsies taken. Chest radiography including high-resolution computed tomography is often informative. In difficult cases, fluorodeoxyglucose positron emission tomography and gallium-67 imaging may identify subclinical disease and a target for biopsy. Symptomatic patients should be treated with corticosteroids, and if clinically indicated other immunosuppressants such as hydroxychloroquine, azathioprine, cyclophosphamide or methotrexate should be added. Anti-tumour necrosis factor alpha therapies may be considered in refractory disease but caution should be exercised as there is evidence to suggest they may unmask disease.

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“…One to five percent of patients die, mainly from respiratory insufficiency, myocardiopathy or CNS lesions. In fact, disability and mortality rates are high in patients with CNS involvement 3,53 . Conventional treatment of pulmonary sarcoidosis is based on oral corticosteroids, but no studies have been published that define optimum dose or duration 54 .…”

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confidence: 99%

Neurosarcoidosis: Two case reports with multiple cranial nerve involvement and review of the literature

Mijajlović¹,

Mirkovic²,

Mihailović-Vučinić³

et al. 2014

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

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aBackground. Involvement of the central nervous system is registered in a relatively small number of patients with sarcoidosis. In this article we present two cases with various neurological symptoms that fulfill criteria for neurosarcoidosis (NS). In addition, we review the literature on NS with special attention to isolated cranial nerve involvement. Methods and Results. First patient: Neurological examination identified multiple cranial neuropathy, moderate rightsided hemiparesis, polyradiculoneuritis of the lower limbs and positive meningeal signs. Laboratory tests showed serum and cerebrospinal fluid (CSF) inflammatory abnormalities, with increased values of the angiotensin-converting enzyme (ACE). CSF analysis also showed presence of 9 oligoclonal IgG bands. Brain and spine magnetic resonance imaging (MRI) revealed diffuse meningopathy, and focal granulomatous lesion in the body of the L5 vertebra. Lung sarcoidosis was confirmed by additional diagnostic procedures. The patient was treated with Methylprednisolone and a tapering course of oral Prednisone, which reduced the pain in the back and legs and improved the strength of the right leg. However, the other neurological deficiencies remained. After confirming lung sarcoidosis, the patient received Methotrexate in addition to Prednisone but during the following 2 years the patient's condition progressively worsened and ended in death. Second patient: Neurological findings showed weakness of the right n. oculomotorius and the right n. trochlearis, as well as the right-side face weakness. We found raised level of the ACE in serum and CSF. Thorax high-definition computed tomography (HDCTT) showed ribbon-like domains of discrete changes in the pulmonary parenchyma. MRI of the brain showed multiple white matter lesions. This patient also received Methylprednisolone followed by Prednisone, and after two months, ocular motility normalized. Conclusion. The diagnosis of NS is always a challenge. For this rerason definitive diagnosis requires the exclusion of other causes of neuropathy. Multiple cranial neuropathies should always arouse suspicion of NS.

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Neurosarcoidosis

Cited by 277 publications

References 0 publications

Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

Neurosarcoidosis: a clinical approach to diagnosis and management

Neurosarcoidosis: Two case reports with multiple cranial nerve involvement and review of the literature

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