Neurosarcoidosis: clinical presentations and course in 50 patients

Oksanen, Virpi

doi:10.1111/j.1600-0404.1986.tb03277.x

Cited by 200 publications

(83 citation statements)

References 26 publications

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“…Main findings are central loss or blurred vision, retrobulbar pain, optic nerve atrophy, and papilledema resulting from local granuloma formation (8,37,38). The VII nerve paresis is usually evident unilaterally and less frequently affects both sides of the face (26,35,39). Rarely, the involvement of the VII nerve is associated with other specific symptoms such as Heerfordt's syndrome, a clinical variety characterized by facial nerve palsy, fever, swelling of the parotid glands, and uveitis (40).…”

Section: Central Nervous System (Cns) Sarcoidosismentioning

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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Section: Central Nervous System (Cns) Sarcoidosismentioning

confidence: 99%

Challenges in the diagnosis and treatment of neurosarcoidosis

et al. 2015

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“…The diagnostic value of chest radiography in suspected systemic sarcoidosis is undisputable: abnormalities are seen in 90%, but of course, a normal chest X-ray does not exclude generalized sarcoidosis 30 . HDCTT is useful in diagnosing pulmonary sarcoidosis and also assists in targeting transbronchial biopsy.…”

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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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“…Progressive motor dysfunction may even mimic anterior horn syndrome 10 . Involvement of basal ganglia may lead to movement disorders, with rare reports linking sarcoidosis to Parkinsonism and generalized chorea 11 . Stroke, subarachnoid hemorrhage, or intraparenchymal hemorrhage are quite rare in NS and may result from blood vessels involvement by sarcoid granulomas 9,11 .…”

Section: Central Nervous Systemmentioning

confidence: 99%

“…Involvement of basal ganglia may lead to movement disorders, with rare reports linking sarcoidosis to Parkinsonism and generalized chorea 11 . Stroke, subarachnoid hemorrhage, or intraparenchymal hemorrhage are quite rare in NS and may result from blood vessels involvement by sarcoid granulomas 9,11 . Most NS patients with increased intracranial pressure present mass lesions, meningitis, or venous sinus thrombosis.…”

Section: Central Nervous Systemmentioning

confidence: 99%

Neurosarcoidosis: guidance for the general neurologist

Dutra

Braga‐Neto

Oliveira

et al. 2012

Arq. Neuro-Psiquiatr.

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Sarcoidosis is an inflammatory multisystem disorder of unknown etiology with a worldwide distribution that can affect lungs, lymphatic system, skin, liver, eyes, and nervous system 1 . We owe the first recorded and illustrated description of sarcoidosis to Jonathan Hutchinson in 1878, when he reported a patient with a dermatologic disease, which comprised purplish, symmetric, and non-tender skin plaques, initially considered a manifestation of gout 2 . The term sarcoidosis comes from histological studies performed by Cesar Boeck, in 1899, who described pathological findings of skin lesions that resembled those of sarcoma, which he called 'sarkoid ' or 'sarcoma-like' 3 . Involvement of the nervous system was first recognized, in 1905, by Winkler, and, in 1909, Heerfordt described three patients in the context of facial nerve palsies, uveitis, parotid enlargement, and fever 1,3 . Neurosarcoidosis (NS) may involve any part of the nervous system with acute and chronic courses 1,4 . Cranial nerves, hypothalamus, and pituitary gland are the most commonly regions involved, but meninges, parenchyma, brainstem, and spinal cord may also be affected 4 . Clinical differentiation from other neurological diseases may be difficult when an isolated involvement of the nervous system is present 3,4 . In this review, we highlighted its clinical forms, pathogenesis, and treatment guidelines for NS, as guidance for general neurologists. neUROLOGiCaL ManiFesTaTiOns OF saRCOidOsisNeurologic manifestations are found in 5 to 20% of cases and symptoms may be mild or even severe, requiring a more aggressive intervention. About one-half of patients with NS can develop neurologic manifestations before systemic sarcoidosis is evident absTRaCT Neurosarcoidosis (NS) more commonly occurs in the setting of systemic disease. The diagnosis is based on a clinical history suggestive of NS, presence of noncaseating granulomas, and supportive evidence of sarcoid pathology, laboratory, and imaging studies. NS could involve any part of the nervous system and often demands high doses of steroids for symptom control. It presents low response to isolated steroids administration and frequently requires immunosuppressive agents. In NS, lymphocytes are polarized toward an excessive Th1 response, leading to overproduction of TNF-alpha and INF-gama, as well as lL-2 and IL-15. Infliximab, a chimeric monoclonal antibody that neutralizes the biological activity of TNF-alpha, is a new option in the NS treatment. We revised pathophysiology, clinical manifestations, diagnostic work up, and treatment of NS as guidance for the general neurologist.Key words: sarcoidosis, neurosarcoidosis, methotrexate, azathioprine, cyclophosphamide, infliximab. ResUMOA neurosarcoidose (NS) ocorre frequentemente no contexto de doença sistêmica. O diagnóstico é baseado na história clínica sugestiva de NS, presença de granulomas não-caseosos e achados anatomopatológicos, laboratoriais e radiológicos de sarcoidose. A NS causa manifestações neurológicas variadas, que apresentam, em ge...

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Neurosarcoidosis: clinical presentations and course in 50 patients

Cited by 200 publications

References 26 publications

Challenges in the diagnosis and treatment of neurosarcoidosis

Challenges in the diagnosis and treatment of neurosarcoidosis

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

Neurosarcoidosis: guidance for the general neurologist

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