Neurosarcoidosis: Clinical manifestations, diagnosis and treatment

Nozaki, Kenkichi; Judson, Marc A.

doi:10.1016/j.lpm.2011.12.017

Cited by 107 publications

(126 citation statements)

References 86 publications

(225 reference statements)

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“…Our findings from a retrospective neurosarcoidosis series confirm that the clinical features of headache in patients with this disorder may have different presentation [2,4,13]. Our cases were clinically similar to Tolosa-Hunt syndrome or to tension-type headache.…”

Section: Discussionsupporting

confidence: 79%

“…Neurological complications in sarcoidosis are rare, highly variable. The appropriate diagnostic investigations include magnetic resonance imaging (MRI) of the brain and spinal cord, cerebrospinal fluid studies, brain and meningeal biopsy if feasible, chest radiography, angiotensin-converting enzyme level in the serum or in the cerebrospinal fluid (CSF) [1,2]. Kveim test [2] and fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET) [3,4] can be performed in particular cases.…”

Section: Introductionmentioning

confidence: 99%

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Headache as presenting symptom of neurosarcoidosis

et al. 2013

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Headache may occur in neurosarcoidosis and diagnostic criteria are given in the International Classification of Headache Disorders (ICHD-II). We present a case series of patients suffering from neurosarcoidosis in whom headache was the presenting symptom. The aim of the present study was to analyze the possible clinical presentations and the corresponding cerebral lesions in a retrospective chart review of patients suffering from neurosarcoidosis in whom headache was the presenting symptom. Medical records and data of six patients were analyzed. The possible diagnoses of headache forms included in ICDH-II, and in particular the correspondence with the criteria for ''headache attributed to neurosarcoidosis'', as well as neuroimaging findings were evaluated in each patient. The ICHD-II criteria were fulfilled in all the six patients. As for as clinical presentation, in three patients (50 %), headache had the clinical characteristics of TolosaHunt syndrome, with evidence of a lesion into the cavernous sinus. In the remaining three cases headache was the only neurological symptom found in association with systemic features of sarcoidosis, and had the clinical features of tension-type headache. Our findings confirm that the clinical features of headache in patients with this disorder may have different presentations, which depend on neuropathologic involvement. Thus, a detailed neuroimaging study and CSF evaluation are needed to confirm diagnosis, particularly in patients with no sign of systemic sarcoidosis or in those in whom head pain may mimic a primary headache syndrome.

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Section: Discussionsupporting

confidence: 79%

Section: Introductionmentioning

confidence: 99%

Headache as presenting symptom of neurosarcoidosis

et al. 2013

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“…There is general consensus that corticosteroids are the first-line treatment for neurosarcoidosis (9,26,113,114). If symptoms are severe, a short course of pulsed intravenous steroid treatment (such as 1 g methylprednisolone/d for 3-5 days) is usually applied.…”

Section: Treatmentmentioning

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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“…Typical CSF finding include elevated protein levels and a lymphocytosis, but normal results do not exclude neurosarcoidosis. The value of CSF ACE is controversial, but an elevated level can provide supportive evidence for the diagnosis [6]. Nerve conduction studies and electromyography should be performed in patients with suspected peripheral neuropathy; and MRI has become useful to demonstrate enlarged segments of roots or nerve [12,13].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Neurosarcoidosis is relatively rare and may involve cranial nerves, brain parenchyma, leptomeninges, dura, and peripheral nerves [3]. Neurological symptoms are observed in 5-13% of sarcoidosis, but are often attributable to another cause [4][5][6]. Cranial neuropathies, particularly facial palsy, and optic neuritis, are the most common manifestations of neurosarcoidosis whereas peripheral nervous system involvement is less common and its prevalence varies depending on diagnosis criteria [4][5][6].…”

mentioning

confidence: 99%

Sarcoid neuropathy

Vital

2014

Peripheral Nerve Disorders

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Neurosarcoidosis: Clinical manifestations, diagnosis and treatment

Cited by 107 publications

References 86 publications

Headache as presenting symptom of neurosarcoidosis

Headache as presenting symptom of neurosarcoidosis

Challenges in the diagnosis and treatment of neurosarcoidosis

Sarcoid neuropathy

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