Movement Disorders (revue)

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Erdheim‐chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome

Identifieur interne : 005014 ( Main/Exploration ); précédent : 005013; suivant : 005015

Erdheim‐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]

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RBID : ISTEX:9643B58895A68F3B10716F2DF03BA40F7A934AE3

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English descriptors

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.

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DOI: 10.1002/mds.870130335


Affiliations:


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Le document en format XML

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<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>
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<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>
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<term>Granuloma</term>
<term>Histiocytosis, Langerhans-Cell</term>
<term>Lipomatosis</term>
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<term>Cerebellar Ataxia</term>
<term>Cerebellar Diseases</term>
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<term>Cerebellum</term>
<term>Granuloma</term>
<term>Histiocytosis, Langerhans-Cell</term>
<term>Lipomatosis</term>
<term>Medulla Oblongata</term>
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<term>Ataxie</term>
<term>Diabète insipide</term>
<term>Etude cas</term>
<term>Granulomatose osseuse Chester Erdheim</term>
<term>Homme</term>
<term>Imagerie RMN</term>
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<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>
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<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>
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