Isolated Spinal Cord Neurosarcoidosis Diagnosed by Cord Biopsy and Thalidomide Trial

Ahn, Suk‐Won; Kim, Kyoung‐Tae; Youn, Young Chul; Kwon, Ohsang; Kim, Young-Baeg

doi:10.3346/jkms.2011.26.1.154

Cited by 13 publications

(17 citation statements)

References 13 publications

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“…6,7 A neurosarcoidose raramente se apresenta unicamente mimetizando um tumor cerebral, como um meningioma. [8][9][10] Exames complementares, como dosagem de enzima de conversão da angiotensina, fosfatase alcalina sérica, cálcio sérico e liquórico, auxiliam no diagnóstico, porém são inespecíficos. O líquor apresenta padrão de pleocitose mononuclear, elevação de proteínas e a presença de bandas oligoclonais.…”

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“…3 Lesões com aumento da intensidade em T2 na junção da substância cinzenta com a branca são altamente sugestivas do seu diagnóstico, principalmente quando associadas com o realce das meninges e com a lesão de hipotálamo. 7,8,10 Apenas uma minoria necessita de confirmação histológica com granulomas não caseosos no tecido do sistema nervoso afetado. 4,9 O diagnóstico é de exclusão, porém um diagnóstico provável é definido como evidência de inflamação do sistema nervoso na RM ou no líquor com proteína e celularidade elevada, índices G de imunoglobina ou presença de bandas oligoclonais em combinação com evidência de sarcoidose sistêmica com confirmação histológica.…”

Section: Discussionunclassified

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Sarcoidosis Mimicking Skull Base Meningioma

Chaves

Mattozo

Maeda

et al. 2018

Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery

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ResumoSarcoidose é uma doença sistêmica caracterizada por inflamação granulomatosa em que o envolvimento pulmonar e linfático é comum. Apresentamos um relato de caso raro de envolvimento do sistema nervoso central com acometimento ocular mimetizando meningioma da bainha do nervo óptico. Relatamos uma paciente de 79 anos, do sexo feminino, com diminuição visual progressiva com evolução de 4 anos. A ressonância magnética do crânio com gadolínio, através de intenso realce homogêneo pelo meio de contraste, evidenciou uma lesão expansiva no nervo óptico direito, na altura do canal óptico. A paciente foi submetida à abordagem neurocirúrgica com biópsia de lesão, que evidenciou sarcoidose do sistema nervoso central. Devido à raridade do acometimento do sistema nervoso central, o diagnóstico dessa patologia pode ser, infelizmente, postergado. O presente artigo tem como objetivo elucidar a patologia como diagnóstico diferencial dos tumores retro-orbitários. Keywords ► sarcoidosis ► ocular sarcoidosis AbstractSarcoidosis is a systemic disease characterized by granulomatous inflammation. Pulmonary and lymphatic granulomatous involvement are common. We present a rare case report of involvement of the central nervous system with ocular involvement mimicking optic nerve sheath meningioma. We report a 79-year-old female patient with progressive visual impairment with an evolution of 4 years. The magnetic resonance imaging of the cranium with gadolinium and intense homogeneous contrast enhancement revealed an expansive lesion in the right optic nerve, at the height of the optic canal. The patient was submitted to the neurosurgical approach with lesion biopsy, which showed sarcoidosis of the central nervous system. Due to the rarity of central nervous system involvement, the diagnosis of this pathology may unfortunately be postponed. This article aims to elucidate this pathology as a differential diagnosis of retro-orbital tumors.

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Section: Discussionunclassified

Sarcoidosis Mimicking Skull Base Meningioma

Chaves

Mattozo

Maeda

et al. 2018

Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery

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“…[6] Nervous system involvement has been reported in 5% to 15% of patients and can involve any part of the nervous system, ranging from brain parenchyma, cranial nerves, to peripheral nerves. [7–9] …”

Section: Discussionmentioning

confidence: 99%

Isolated neurosarcoidosis mimicking multifocal meningiomas

Wang

et al. 2016

Medicine

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Introduction:Neurosarcoidosis accounts for approximately 5% of the sarcoidosis, which develops exclusively in the nervous system and is always difficult to diagnose. We describe a rare case of isolated neurosarcoidosis mimicking as multifocal meningiomas. A 27-year-old male was admitted to our hospital with a history of unconsciousness and convulsion 1 month ago, which was suspected as a seizure. The results showed no abnormalities in complete blood count; serum electrolytes; erythrocyte sedimentation rate and ultrasonography of the liver, pancreas, spleen, kidney and parotid gland, and so on. Chest radiograph and electroencephalogram were also normal. Serum-angiotensin-converting enzyme slightly increased. Normal opening pressure was shown in cerebrospinal fluid sampling, which includes 8/μL white blood cells, 0.93 g/L protein, and 3.03 mmol/L glucose. Enhanced magnetic resonance imaging revealed multifocal enhancement lesions, including left sphenoid wing region, left temporal and bilateral occipitoparietal region, which were suspected as multiple “meningioma”. A left frontotemporal craniotomy was further performed. Both necrotizing and non-necrotizing granulomas were revealed in the pathological specimen, most of which were associated with multinucleated giant cells and macrophages. We could also see the fibrosis and inflammatory reaction in the sample composed of lymphocytes, histiocytes, and plasma cells. Histopathological examination showed that the cells were positive for human CD68 (KP1), CD68 (PGM1), and CD163; however, they were negative for the AF, epithelial membrane antigen, and glial fibrillary acidic protein. Tuberculosis-deoxyribonucleic acid test and special stains for acid-fast bacilli and fungi were negative. The diagnosis was finally made as isolated neurosarcoidosis. Then the patient was treated with additional corticosteroid therapy. Serial imaging examination 4 months later revealed that the lesions extremely decreased.Conclusion:The diagnosis of isolated central nervous system sarcoidosis was still difficult because of limitations of available diagnostic tests. So neurosarcoidosis in the clinical work should never be neglected.

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“…[ 4 5 6 7 8 9 10 ] This however is not always true and sarcoidosis albeit rarely; can manifest as true isolated spinal cord involvement in less than 0.5% cases. [ 9 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 ] The rarity of this presentation with nondiagnostic imaging features, clinical presentation, and absence of systemic involvement often makes it challenging to diagnose this condition leading to delay in diagnosis and often inappropriate management with concomitant morbidity. [ 23 ] Blood and cerebrospinal fluid (CSF) markers are mostly adjunct and have very low sensitivity and specificity for NS confounding the diagnosis.…”

Section: Introductionmentioning

confidence: 99%

“…[ 31 32 ] Though these cases initially presented with features of spinal cord involvement, workup revealed presence of extraneural involvement,[ 31 32 33 ] as against very few cases described in the literature wherein true isolated spinal cord NS was described and mandated biopsy. [ 2 9 11 12 14 15 16 17 18 20 21 22 23 24 25 26 27 31 33 34 35 36 37 38 ] We encountered a case of isolated cervical intramedullary NS which presented as cervical myelopathy and could be diagnosed only after performance of spinal cord biopsy meeting the definite Zagilek's criteria. The clinical picture with nuances of diagnosis of this condition from the present case is discussed with comprehensive review of the literature and characteristic MRI features of spinal NS which might be useful for diagnosis/workup.…”

Section: Introductionmentioning

confidence: 99%

Isolated spinal neurosarcoidosis: An enigmatic intramedullary spinal cord pathology-case report and review of the literature

Kasliwal

Harbhajanka

Nag

et al. 2013

J Craniovert Jun Spine

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Isolated spinal cord neurosarcoidosis (NS) in the absence of systemic disease or intracranial involvement is exceptionally rare. Adjunctive laboratory tests though useful may not be reliable and the absence of any pathognomonic radiological features makes the diagnosis difficult. As spinal cord NS may be a presenting feature of systemic sarcoidosis which may be occult on routine workup, 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) may be of value in unraveling this systemic involvement avoiding biopsying the spinal cord. A case of truly isolated NS is described with review of literature on this enigmatic pathology. Long segment intramedullary signal changes with focal parenchymal along with dural/meningeal enhancement in the absence of significant cervical stenosis in a young patient of northern European or African-American decent is very suggestive of NS and although may be presumably treated with steroids; there should be a low threshold for spinal cord biopsy especially in the absence of response to steroids to confirm isolated spinal cord NS in a patient with clinical neurological deterioration.

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Isolated Spinal Cord Neurosarcoidosis Diagnosed by Cord Biopsy and Thalidomide Trial

Cited by 13 publications

References 13 publications

Sarcoidosis Mimicking Skull Base Meningioma

Sarcoidosis Mimicking Skull Base Meningioma

Isolated neurosarcoidosis mimicking multifocal meningiomas

Isolated spinal neurosarcoidosis: An enigmatic intramedullary spinal cord pathology-case report and review of the literature

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