Erdheim‐chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome
Identifieur interne : 005014 ( Main/Exploration ); précédent : 005013; suivant : 005015Erdheim‐chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome
Auteurs : Virgilio Gerald H. Evidente [États-Unis] ; Charles H. Adler [États-Unis] ; Caterina Giannini [États-Unis] ; Christopher R. Conley [États-Unis] ; Joseph E. Parisi [États-Unis] ; Geoffrey P. Fletcher [États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 1998-05.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
- Aged, Ataxia, Brain stem, Case study, Cerebellar Ataxia (diagnosis), Cerebellar Ataxia (pathology), Cerebellar Diseases (diagnosis), Cerebellar Diseases (pathology), Cerebellar Nuclei (pathology), Cerebellum (pathology), Chester-Erdheim disease, Diabetes insipidus, Diagnosis, Differential, Erdheim‐Chester, Follow-Up Studies, Granuloma (diagnosis), Granuloma (pathology), Histiocytosis, Histiocytosis, Langerhans-Cell (diagnosis), Histiocytosis, Langerhans-Cell (pathology), Human, Humans, Inaugural sign, Lipogranulomatosis, Lipomatosis (diagnosis), Lipomatosis (pathology), Magnetic Resonance Imaging, Male, Medulla Oblongata (pathology), Nuclear magnetic resonance imaging, Pons (pathology).
- MESH :
- diagnosis : Cerebellar Ataxia, Cerebellar Diseases, Granuloma, Histiocytosis, Langerhans-Cell, Lipomatosis.
- pathology : Cerebellar Ataxia, Cerebellar Diseases, Cerebellar Nuclei, Cerebellum, Granuloma, Histiocytosis, Langerhans-Cell, Lipomatosis, Medulla Oblongata, Pons.
- Aged, Diagnosis, Differential, Follow-Up Studies, Humans, Magnetic Resonance Imaging, Male.
Abstract
We report a rare case of Erdheim‐Chester disease (ECD) presenting as a progressive cerebellar syndrome and diabetes insipidus. On magnetic resonance imaging, a 7‐mm extraaxial, enhancing mass was seen enveloping the right vertebral artery and was confirmed at autopsy to represent an adventitial xanthoma with lipid‐laden, foamy histiocytes. The cerebellar syndrome most likely resulted from extensive histiocytic infiltration of the pons, particularly the basis pontis and middle cerebellar peduncles.
Url:
DOI: 10.1002/mds.870130335
Affiliations:
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Le document en format XML
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<term>Ataxia</term>
<term>Brain stem</term>
<term>Case study</term>
<term>Cerebellar Ataxia (diagnosis)</term>
<term>Cerebellar Ataxia (pathology)</term>
<term>Cerebellar Diseases (diagnosis)</term>
<term>Cerebellar Diseases (pathology)</term>
<term>Cerebellar Nuclei (pathology)</term>
<term>Cerebellum (pathology)</term>
<term>Chester-Erdheim disease</term>
<term>Diabetes insipidus</term>
<term>Diagnosis, Differential</term>
<term>Erdheim‐Chester</term>
<term>Follow-Up Studies</term>
<term>Granuloma (diagnosis)</term>
<term>Granuloma (pathology)</term>
<term>Histiocytosis</term>
<term>Histiocytosis, Langerhans-Cell (diagnosis)</term>
<term>Histiocytosis, Langerhans-Cell (pathology)</term>
<term>Human</term>
<term>Humans</term>
<term>Inaugural sign</term>
<term>Lipogranulomatosis</term>
<term>Lipomatosis (diagnosis)</term>
<term>Lipomatosis (pathology)</term>
<term>Magnetic Resonance Imaging</term>
<term>Male</term>
<term>Medulla Oblongata (pathology)</term>
<term>Nuclear magnetic resonance imaging</term>
<term>Pons (pathology)</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Cerebellar Ataxia</term>
<term>Cerebellar Diseases</term>
<term>Granuloma</term>
<term>Histiocytosis, Langerhans-Cell</term>
<term>Lipomatosis</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Cerebellar Ataxia</term>
<term>Cerebellar Diseases</term>
<term>Cerebellar Nuclei</term>
<term>Cerebellum</term>
<term>Granuloma</term>
<term>Histiocytosis, Langerhans-Cell</term>
<term>Lipomatosis</term>
<term>Medulla Oblongata</term>
<term>Pons</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Aged</term>
<term>Diagnosis, Differential</term>
<term>Follow-Up Studies</term>
<term>Humans</term>
<term>Magnetic Resonance Imaging</term>
<term>Male</term>
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<keywords scheme="Pascal" xml:lang="fr"><term>Ataxie</term>
<term>Diabète insipide</term>
<term>Etude cas</term>
<term>Granulomatose osseuse Chester Erdheim</term>
<term>Homme</term>
<term>Imagerie RMN</term>
<term>Mâle</term>
<term>Signe inaugural</term>
<term>Tronc cérébral</term>
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<front><div type="abstract" xml:lang="en">We report a rare case of Erdheim‐Chester disease (ECD) presenting as a progressive cerebellar syndrome and diabetes insipidus. On magnetic resonance imaging, a 7‐mm extraaxial, enhancing mass was seen enveloping the right vertebral artery and was confirmed at autopsy to represent an adventitial xanthoma with lipid‐laden, foamy histiocytes. The cerebellar syndrome most likely resulted from extensive histiocytic infiltration of the pons, particularly the basis pontis and middle cerebellar peduncles.</div>
</front>
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<affiliations><list><country><li>États-Unis</li>
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<region><li>Arizona</li>
<li>Minnesota</li>
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<tree><country name="États-Unis"><region name="Arizona"><name sortKey="Evidente, Virgilio Gerald H" sort="Evidente, Virgilio Gerald H" uniqKey="Evidente V" first="Virgilio Gerald H." last="Evidente">Virgilio Gerald H. Evidente</name>
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<name sortKey="Adler, Charles H" sort="Adler, Charles H" uniqKey="Adler C" first="Charles H." last="Adler">Charles H. Adler</name>
<name sortKey="Conley, Christopher R" sort="Conley, Christopher R" uniqKey="Conley C" first="Christopher R." last="Conley">Christopher R. Conley</name>
<name sortKey="Fletcher, Geoffrey P" sort="Fletcher, Geoffrey P" uniqKey="Fletcher G" first="Geoffrey P." last="Fletcher">Geoffrey P. Fletcher</name>
<name sortKey="Giannini, Caterina" sort="Giannini, Caterina" uniqKey="Giannini C" first="Caterina" last="Giannini">Caterina Giannini</name>
<name sortKey="Parisi, Joseph E" sort="Parisi, Joseph E" uniqKey="Parisi J" first="Joseph E." last="Parisi">Joseph E. Parisi</name>
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