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Neurosarcoidosis

Identifieur interne : 001157 ( Istex/Corpus ); précédent : 001156; suivant : 001158

Neurosarcoidosis

Auteurs : S. Marangoni ; V. Argentiero ; B. Tavolato

Source :

RBID : ISTEX:58EED0CB97350EA1A47B683F52DEA473E4825858

English descriptors

Abstract

Abstract: Objective: Chronic involvement of the nervous system is relatively rare in sarcoidosis. We describe 7 cases that fulfil Zajicek's criteria for neurosarcoidosis (NS) and propose some modifications to such criteria. Materials and methods: The patients were admitted for various neurological syndromes: 2 cases presented with chronic lymphocytic meningitis, 4 with spinal cord symptoms, one case was initially confused with multiple sclerosis. Serological tests, immunological screening, cerebrospinal fluid (CSF) analysis, bacteriological and viral testing were performed in all patients. Spinal and cerebral MRI, gallium scan, bronchoscopy with biopsy and bronchoalveolar–lavage fluid analysis, high–resolution computed tomography (HRCT) of the chest, biopsy of the lungs, skin, mediastinal lymph–node and meninges, were useful in diagnosing NS. Results and discussion: Laboratory tests showed serum inflammatory abnormalities, but were negative for infectious diseases, while CSF showed inflammatory signs in all patients. MRI revealed meningeal enhancement or hypertrophic pachymeningeal lesions in 4 patients, white matter abnormalities and mass lesions in 2 patients, and a spinal mass lesion in 1 patient. Gallium scan, HRCT, bronchoscopy were positive in most cases. Patients were treated with steroid and immunosuppressive therapy, with improvement in six cases. One patient died from infectious complications. Conclusion: A definite diagnosis of NS requires demonstration of non–caseating granulomas affecting nervous tissues. In most cases, histological evidence of systemic disease (probable NS) is sufficient in the presence of compatible alterations in the CNS. In our patients the bronchoalveolarlavage fluid analysis, gallium scan, and chest HRCT were important for diagnosis,while serum ACE was always normal and chest radiographs were not suggestive of sarcoidosis.

Url:
DOI: 10.1007/s00415-005-0043-5

Links to Exploration step

ISTEX:58EED0CB97350EA1A47B683F52DEA473E4825858

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</Heading>
<Para>The patients were admitted for various neurological syndromes: 2 cases presented with chronic lymphocytic meningitis, 4 with spinal cord symptoms, one case was initially confused with multiple sclerosis. Serological tests, immunological screening, cerebrospinal fluid (CSF) analysis, bacteriological and viral testing were performed in all patients. Spinal and cerebral MRI, gallium scan, bronchoscopy with biopsy and bronchoalveolar–lavage fluid analysis, high–resolution computed tomography (HRCT) of the chest, biopsy of the lungs, skin, mediastinal lymph–node and meninges, were useful in diagnosing NS.</Para>
</AbstractSection>
<AbstractSection ID="AbsSec3">
<Heading>
<Emphasis Type="Italic">Results and discussion</Emphasis>
</Heading>
<Para>Laboratory tests showed serum inflammatory abnormalities, but were negative for infectious diseases, while CSF showed inflammatory signs in all patients. MRI revealed meningeal enhancement or hypertrophic pachymeningeal lesions in 4 patients, white matter abnormalities and mass lesions in 2 patients, and a spinal mass lesion in 1 patient. Gallium scan, HRCT, bronchoscopy were positive in most cases. Patients were treated with steroid and immunosuppressive therapy, with improvement in six cases. One patient died from infectious complications.</Para>
</AbstractSection>
<AbstractSection ID="AbsSec4">
<Heading>
<Emphasis Type="Italic">Conclusion</Emphasis>
</Heading>
<Para>A definite diagnosis of NS requires demonstration of non–caseating granulomas affecting nervous tissues. In most cases, histological evidence of systemic disease (probable NS) is sufficient in the presence of compatible alterations in the CNS. In our patients the bronchoalveolarlavage fluid analysis, gallium scan, and chest HRCT were important for diagnosis,while serum ACE was always normal and chest radiographs were not suggestive of sarcoidosis.</Para>
</AbstractSection>
</Abstract>
<KeywordGroup Language="En">
<Heading>Key words</Heading>
<Keyword>neurosarcoidosis</Keyword>
<Keyword>CNS diseases</Keyword>
<Keyword>inflammatory</Keyword>
</KeywordGroup>
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<title>Neurosarcoidosis</title>
<subTitle>Clinical description of 7 cases with a proposal for a new diagnostic strategy</subTitle>
</titleInfo>
<titleInfo type="alternative" contentType="CDATA">
<title>Neurosarcoidosis</title>
</titleInfo>
<name type="personal">
<namePart type="termsOfAddress">Dr. ssa</namePart>
<namePart type="given">S.</namePart>
<namePart type="family">Marangoni</namePart>
<affiliation>Clinica Neurologica II, Ospedale S.Antonio, Via Facciolati 71, 35127, Padova, Italy</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="termsOfAddress">Dr. ssa</namePart>
<namePart type="given">V.</namePart>
<namePart type="family">Argentiero</namePart>
<affiliation>Clinica Neurologica II, Ospedale S.Antonio, Via Facciolati 71, 35127, Padova, Italy</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal" displayLabel="corresp">
<namePart type="termsOfAddress">Prof.</namePart>
<namePart type="given">B.</namePart>
<namePart type="family">Tavolato</namePart>
<affiliation>Clinica Neurologica II, Ospedale S.Antonio, Via Facciolati 71, 35127, Padova, Italy</affiliation>
<affiliation>E-mail: bruno.tavolato@unipd.it</affiliation>
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<abstract lang="en">Abstract: Objective: Chronic involvement of the nervous system is relatively rare in sarcoidosis. We describe 7 cases that fulfil Zajicek's criteria for neurosarcoidosis (NS) and propose some modifications to such criteria. Materials and methods: The patients were admitted for various neurological syndromes: 2 cases presented with chronic lymphocytic meningitis, 4 with spinal cord symptoms, one case was initially confused with multiple sclerosis. Serological tests, immunological screening, cerebrospinal fluid (CSF) analysis, bacteriological and viral testing were performed in all patients. Spinal and cerebral MRI, gallium scan, bronchoscopy with biopsy and bronchoalveolar–lavage fluid analysis, high–resolution computed tomography (HRCT) of the chest, biopsy of the lungs, skin, mediastinal lymph–node and meninges, were useful in diagnosing NS. Results and discussion: Laboratory tests showed serum inflammatory abnormalities, but were negative for infectious diseases, while CSF showed inflammatory signs in all patients. MRI revealed meningeal enhancement or hypertrophic pachymeningeal lesions in 4 patients, white matter abnormalities and mass lesions in 2 patients, and a spinal mass lesion in 1 patient. Gallium scan, HRCT, bronchoscopy were positive in most cases. Patients were treated with steroid and immunosuppressive therapy, with improvement in six cases. One patient died from infectious complications. Conclusion: A definite diagnosis of NS requires demonstration of non–caseating granulomas affecting nervous tissues. In most cases, histological evidence of systemic disease (probable NS) is sufficient in the presence of compatible alterations in the CNS. In our patients the bronchoalveolarlavage fluid analysis, gallium scan, and chest HRCT were important for diagnosis,while serum ACE was always normal and chest radiographs were not suggestive of sarcoidosis.</abstract>
<note>ORIGINAL COMMUNICATION</note>
<subject lang="en">
<genre>Key words</genre>
<topic>neurosarcoidosis</topic>
<topic>CNS diseases</topic>
<topic>inflammatory</topic>
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<title>Journal of Neurology</title>
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<dateIssued encoding="w3cdtf">2006-04-01</dateIssued>
<copyrightDate encoding="w3cdtf">2006</copyrightDate>
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<genre>Medicine & Public Health</genre>
<topic>Neurosciences</topic>
<topic>Neuroradiology</topic>
<topic>Neurology</topic>
</subject>
<identifier type="ISSN">0340-5354</identifier>
<identifier type="eISSN">1432-1459</identifier>
<identifier type="JournalID">415</identifier>
<identifier type="IssueArticleCount">28</identifier>
<identifier type="VolumeIssueCount">12</identifier>
<part>
<date>2006</date>
<detail type="volume">
<number>253</number>
<caption>vol.</caption>
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<detail type="issue">
<number>4</number>
<caption>no.</caption>
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<start>488</start>
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