Leptomeningeal and intraventricular myelomatosis manifesting an aggressive form of communicating hydrocephalus

Leptomeningeal myelomatosis (LMM) is a fatal complication that occurs in

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Veröffentlicht in:Neuropathology 2021-06, Vol.41 (3), p.243-249
Hauptverfasser: Miki, Yasuo, Kamata, Kosuke, Akemoto, Yui, Tsushima, Fumiyasu, Sakuraba, Hirotake, Yamagata, Kazufumi, Kurose, Akira, Fukuda, Shinsaku, Wakabayashi, Koichi
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container_issue 3
container_start_page 243
container_title Neuropathology
container_volume 41
creator Miki, Yasuo
Kamata, Kosuke
Akemoto, Yui
Tsushima, Fumiyasu
Sakuraba, Hirotake
Yamagata, Kazufumi
Kurose, Akira
Fukuda, Shinsaku
Wakabayashi, Koichi
description Leptomeningeal myelomatosis (LMM) is a fatal complication that occurs in
doi_str_mv 10.1111/neup.12728
format Article
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Many patients with LMM present with neurologic symptoms referable to cranial neuropathies, while the manifestation of communicating hydrocephalus has been underrecognized. A Japanese man with Bence Jones protein‐κ multiple myeloma developed fever and headache at age 54 years. He then became somnolent and went into a coma. Neuroimaging analyses identified rapidly progressive communicating hydrocephalus due to meningitis. He died 83 days after the onset of headache without any response to treatment at age 55 years. No symptoms or signs associated with cranial nerves were found during the course of illness. Postmortem examination revealed hydrocephalus and diffuse infiltration of myeloma cells into the subarachnoid space of the cerebrum, cerebellum, and brainstem. In addition, the interstitial tissue of the choroid plexuses was filled with myeloma cells. These myeloma cells were positive for CD156 and light chain κ. The Ki‐67 labeling index in myeloma cells of the central nervous system (CNS) was 30–40%. Histopathological examination further revealed many myeloma cells on the surface of the lateral, third and fourth ventricles and at the area postrema of the medulla oblongata. Patients with LMM can develop an aggressive form of communicating hydrocephalus. Given that cerebrospinal fluid, produced by epithelial cells in the choroid plexuses of the ventricles, passes into the subarachnoid space through the third and fourth ventricles, myeloma cells may invade the CNS through the choroid plexuses.</description><identifier>ISSN: 0919-6544</identifier><identifier>EISSN: 1440-1789</identifier><identifier>DOI: 10.1111/neup.12728</identifier><identifier>PMID: 33973283</identifier><language>eng</language><publisher>Melbourne: John Wiley &amp; Sons Australia, Ltd</publisher><subject>Area postrema ; Brain stem ; Central nervous system ; Cerebellum ; Cerebrospinal fluid ; Cerebrum ; Clinical Neurology ; CNS invasion ; CNS relapse ; communicating hydrocephalus ; Cranial nerves ; Epithelial cells ; Fever ; Headache ; Hydrocephalus ; leptomeningeal myelomatosis ; Life Sciences &amp; Biomedicine ; Medulla oblongata ; Meninges ; Meningitis ; Multiple myeloma ; Neuroimaging ; Neuropathy ; Neurosciences ; Neurosciences &amp; Neurology ; Pathology ; Science &amp; Technology ; Skull ; Subarachnoid space</subject><ispartof>Neuropathology, 2021-06, Vol.41 (3), p.243-249</ispartof><rights>2021 Japanese Society of Neuropathology</rights><rights>2021 Japanese Society of Neuropathology.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>true</woscitedreferencessubscribed><woscitedreferencescount>2</woscitedreferencescount><woscitedreferencesoriginalsourcerecordid>wos000648927400001</woscitedreferencesoriginalsourcerecordid><citedby>FETCH-LOGICAL-c3818-c03bc395b9ed26a76ba1ffee1b8b98283a0d6d9f2afe03c52b4b6371602908d13</citedby><cites>FETCH-LOGICAL-c3818-c03bc395b9ed26a76ba1ffee1b8b98283a0d6d9f2afe03c52b4b6371602908d13</cites><orcidid>0000-0001-9375-9590 ; 0000-0002-3756-7209</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fneup.12728$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fneup.12728$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>315,781,785,1418,27929,27930,39263,45579,45580</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/33973283$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Miki, Yasuo</creatorcontrib><creatorcontrib>Kamata, Kosuke</creatorcontrib><creatorcontrib>Akemoto, Yui</creatorcontrib><creatorcontrib>Tsushima, Fumiyasu</creatorcontrib><creatorcontrib>Sakuraba, Hirotake</creatorcontrib><creatorcontrib>Yamagata, Kazufumi</creatorcontrib><creatorcontrib>Kurose, Akira</creatorcontrib><creatorcontrib>Fukuda, Shinsaku</creatorcontrib><creatorcontrib>Wakabayashi, Koichi</creatorcontrib><title>Leptomeningeal and intraventricular myelomatosis manifesting an aggressive form of communicating hydrocephalus</title><title>Neuropathology</title><addtitle>NEUROPATHOLOGY</addtitle><addtitle>Neuropathology</addtitle><description>Leptomeningeal myelomatosis (LMM) is a fatal complication that occurs in &lt; 1% of patients with multiple myeloma. Many patients with LMM present with neurologic symptoms referable to cranial neuropathies, while the manifestation of communicating hydrocephalus has been underrecognized. A Japanese man with Bence Jones protein‐κ multiple myeloma developed fever and headache at age 54 years. He then became somnolent and went into a coma. Neuroimaging analyses identified rapidly progressive communicating hydrocephalus due to meningitis. He died 83 days after the onset of headache without any response to treatment at age 55 years. No symptoms or signs associated with cranial nerves were found during the course of illness. Postmortem examination revealed hydrocephalus and diffuse infiltration of myeloma cells into the subarachnoid space of the cerebrum, cerebellum, and brainstem. In addition, the interstitial tissue of the choroid plexuses was filled with myeloma cells. These myeloma cells were positive for CD156 and light chain κ. The Ki‐67 labeling index in myeloma cells of the central nervous system (CNS) was 30–40%. Histopathological examination further revealed many myeloma cells on the surface of the lateral, third and fourth ventricles and at the area postrema of the medulla oblongata. Patients with LMM can develop an aggressive form of communicating hydrocephalus. Given that cerebrospinal fluid, produced by epithelial cells in the choroid plexuses of the ventricles, passes into the subarachnoid space through the third and fourth ventricles, myeloma cells may invade the CNS through the choroid plexuses.</description><subject>Area postrema</subject><subject>Brain stem</subject><subject>Central nervous system</subject><subject>Cerebellum</subject><subject>Cerebrospinal fluid</subject><subject>Cerebrum</subject><subject>Clinical Neurology</subject><subject>CNS invasion</subject><subject>CNS relapse</subject><subject>communicating hydrocephalus</subject><subject>Cranial nerves</subject><subject>Epithelial cells</subject><subject>Fever</subject><subject>Headache</subject><subject>Hydrocephalus</subject><subject>leptomeningeal myelomatosis</subject><subject>Life Sciences &amp; Biomedicine</subject><subject>Medulla oblongata</subject><subject>Meninges</subject><subject>Meningitis</subject><subject>Multiple myeloma</subject><subject>Neuroimaging</subject><subject>Neuropathy</subject><subject>Neurosciences</subject><subject>Neurosciences &amp; Neurology</subject><subject>Pathology</subject><subject>Science &amp; Technology</subject><subject>Skull</subject><subject>Subarachnoid space</subject><issn>0919-6544</issn><issn>1440-1789</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>HGBXW</sourceid><recordid>eNqN0U1v1DAQBmALgehSuPADUCQuVVGKPxLHPqJVC0irlgM9R44z3rqK7WDHRfvv6-0uPXBA-GD78Mzo1QxC7wm-IOV89pDnC0I7Kl6gFWkaXJNOyJdohSWRNW-b5gS9SekeY9JJKl6jE8Zkx6hgK-Q3MC_Bgbd-C2qqlB8r65eoHqDcVudJxcrtYApOLSHZVDnlrYG0lIKiK7XdRkjJPkBlQnRVMJUOzmVvtXoyd7sxBg3znZpyeoteGTUleHd8T9Ht1eXP9bd6c_P1-_rLptZMEFFrzAbNZDtIGClXHR8UMQaADGKQogRXeOSjNFQZwEy3dGgGzjrCMZVYjISdorND3zmGX7mk7Z1NGqZJeQg59bSlLW-Z6Pb041_0PuToS7qiWMspp7Qp6vygdAwpRTD9HK1TcdcT3O-30O-30D9toeAPx5Z5cDA-0z9jL-DTAfyGIZikLXgNzwxjzBshadeUH94nFP-v13Ypcw9-HbJfSik5ltoJdv_I3F9f3v44pH8EHoq1sA</recordid><startdate>202106</startdate><enddate>202106</enddate><creator>Miki, Yasuo</creator><creator>Kamata, Kosuke</creator><creator>Akemoto, Yui</creator><creator>Tsushima, Fumiyasu</creator><creator>Sakuraba, Hirotake</creator><creator>Yamagata, Kazufumi</creator><creator>Kurose, Akira</creator><creator>Fukuda, Shinsaku</creator><creator>Wakabayashi, Koichi</creator><general>John Wiley &amp; Sons Australia, Ltd</general><general>Wiley</general><general>Wiley Subscription Services, Inc</general><scope>BLEPL</scope><scope>DTL</scope><scope>HGBXW</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7TK</scope><scope>K9.</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-9375-9590</orcidid><orcidid>https://orcid.org/0000-0002-3756-7209</orcidid></search><sort><creationdate>202106</creationdate><title>Leptomeningeal and intraventricular myelomatosis manifesting an aggressive form of communicating hydrocephalus</title><author>Miki, Yasuo ; Kamata, Kosuke ; Akemoto, Yui ; Tsushima, Fumiyasu ; Sakuraba, Hirotake ; Yamagata, Kazufumi ; Kurose, Akira ; Fukuda, Shinsaku ; Wakabayashi, Koichi</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3818-c03bc395b9ed26a76ba1ffee1b8b98283a0d6d9f2afe03c52b4b6371602908d13</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Area postrema</topic><topic>Brain stem</topic><topic>Central nervous system</topic><topic>Cerebellum</topic><topic>Cerebrospinal fluid</topic><topic>Cerebrum</topic><topic>Clinical Neurology</topic><topic>CNS invasion</topic><topic>CNS relapse</topic><topic>communicating hydrocephalus</topic><topic>Cranial nerves</topic><topic>Epithelial cells</topic><topic>Fever</topic><topic>Headache</topic><topic>Hydrocephalus</topic><topic>leptomeningeal myelomatosis</topic><topic>Life Sciences &amp; Biomedicine</topic><topic>Medulla oblongata</topic><topic>Meninges</topic><topic>Meningitis</topic><topic>Multiple myeloma</topic><topic>Neuroimaging</topic><topic>Neuropathy</topic><topic>Neurosciences</topic><topic>Neurosciences &amp; Neurology</topic><topic>Pathology</topic><topic>Science &amp; Technology</topic><topic>Skull</topic><topic>Subarachnoid space</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Miki, Yasuo</creatorcontrib><creatorcontrib>Kamata, Kosuke</creatorcontrib><creatorcontrib>Akemoto, Yui</creatorcontrib><creatorcontrib>Tsushima, Fumiyasu</creatorcontrib><creatorcontrib>Sakuraba, Hirotake</creatorcontrib><creatorcontrib>Yamagata, Kazufumi</creatorcontrib><creatorcontrib>Kurose, Akira</creatorcontrib><creatorcontrib>Fukuda, Shinsaku</creatorcontrib><creatorcontrib>Wakabayashi, Koichi</creatorcontrib><collection>Web of Science Core Collection</collection><collection>Science Citation Index Expanded</collection><collection>Web of Science - Science Citation Index Expanded - 2021</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>ProQuest Health &amp; 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Many patients with LMM present with neurologic symptoms referable to cranial neuropathies, while the manifestation of communicating hydrocephalus has been underrecognized. A Japanese man with Bence Jones protein‐κ multiple myeloma developed fever and headache at age 54 years. He then became somnolent and went into a coma. Neuroimaging analyses identified rapidly progressive communicating hydrocephalus due to meningitis. He died 83 days after the onset of headache without any response to treatment at age 55 years. No symptoms or signs associated with cranial nerves were found during the course of illness. Postmortem examination revealed hydrocephalus and diffuse infiltration of myeloma cells into the subarachnoid space of the cerebrum, cerebellum, and brainstem. In addition, the interstitial tissue of the choroid plexuses was filled with myeloma cells. These myeloma cells were positive for CD156 and light chain κ. The Ki‐67 labeling index in myeloma cells of the central nervous system (CNS) was 30–40%. Histopathological examination further revealed many myeloma cells on the surface of the lateral, third and fourth ventricles and at the area postrema of the medulla oblongata. Patients with LMM can develop an aggressive form of communicating hydrocephalus. Given that cerebrospinal fluid, produced by epithelial cells in the choroid plexuses of the ventricles, passes into the subarachnoid space through the third and fourth ventricles, myeloma cells may invade the CNS through the choroid plexuses.</abstract><cop>Melbourne</cop><pub>John Wiley &amp; Sons Australia, Ltd</pub><pmid>33973283</pmid><doi>10.1111/neup.12728</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0001-9375-9590</orcidid><orcidid>https://orcid.org/0000-0002-3756-7209</orcidid></addata></record>
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subjects Area postrema
Brain stem
Central nervous system
Cerebellum
Cerebrospinal fluid
Cerebrum
Clinical Neurology
CNS invasion
CNS relapse
communicating hydrocephalus
Cranial nerves
Epithelial cells
Fever
Headache
Hydrocephalus
leptomeningeal myelomatosis
Life Sciences & Biomedicine
Medulla oblongata
Meninges
Meningitis
Multiple myeloma
Neuroimaging
Neuropathy
Neurosciences
Neurosciences & Neurology
Pathology
Science & Technology
Skull
Subarachnoid space
title Leptomeningeal and intraventricular myelomatosis manifesting an aggressive form of communicating hydrocephalus
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