Cerebral Microvascular Erdheim-Chester Disease: A Perivascular Hematopoietic Vasculopathy

Michael Moussouttas; Shanu Roemer; Dennis W. DIckson

doi:10.1159/000516803

Cerebral Microvascular Erdheim-Chester Disease: A Perivascular Hematopoietic Vasculopathy

Michael Moussouttas, Shanu Roemer, Dennis W. DIckson

Neuroscience

Research output: Contribution to journal › Article › peer-review

Abstract

Erdheim-Chester disease (ECD) is a rare and elusive hematopoietic malignancy that may involve the nervous system in various ways. Cerebrovascular ECD involves the perivascular infiltration and compromise of any cervicocranial vessel by transformed proliferating histiocytes. Presented is the novel case of a patient with pathologically proven perivascular microangiopathy, manifesting in multifaceted fashion with ischemia, hemorrhage, mass lesions, and edema.

Original language	English (US)
Pages (from-to)	746-751
Number of pages	6
Journal	Cerebrovascular Diseases
Volume	50
Issue number	6
DOIs	https://doi.org/10.1159/000516803
State	Published - Dec 1 2021

Keywords

Autopsy
Cerebral vasculature
Erdheim-Chester disease
Hemorrhage
Infarction
Microvascular infiltrates
Perivascular microangiopathy

ASJC Scopus subject areas

Neurology
Clinical Neurology
Cardiology and Cardiovascular Medicine

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10.1159/000516803

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title = "Cerebral Microvascular Erdheim-Chester Disease: A Perivascular Hematopoietic Vasculopathy",

abstract = "Erdheim-Chester disease (ECD) is a rare and elusive hematopoietic malignancy that may involve the nervous system in various ways. Cerebrovascular ECD involves the perivascular infiltration and compromise of any cervicocranial vessel by transformed proliferating histiocytes. Presented is the novel case of a patient with pathologically proven perivascular microangiopathy, manifesting in multifaceted fashion with ischemia, hemorrhage, mass lesions, and edema.",

keywords = "Autopsy, Cerebral vasculature, Erdheim-Chester disease, Hemorrhage, Infarction, Microvascular infiltrates, Perivascular microangiopathy",

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AU - DIckson, Dennis W.

PY - 2021/12/1

Y1 - 2021/12/1

N2 - Erdheim-Chester disease (ECD) is a rare and elusive hematopoietic malignancy that may involve the nervous system in various ways. Cerebrovascular ECD involves the perivascular infiltration and compromise of any cervicocranial vessel by transformed proliferating histiocytes. Presented is the novel case of a patient with pathologically proven perivascular microangiopathy, manifesting in multifaceted fashion with ischemia, hemorrhage, mass lesions, and edema.

AB - Erdheim-Chester disease (ECD) is a rare and elusive hematopoietic malignancy that may involve the nervous system in various ways. Cerebrovascular ECD involves the perivascular infiltration and compromise of any cervicocranial vessel by transformed proliferating histiocytes. Presented is the novel case of a patient with pathologically proven perivascular microangiopathy, manifesting in multifaceted fashion with ischemia, hemorrhage, mass lesions, and edema.

KW - Autopsy

KW - Cerebral vasculature

KW - Erdheim-Chester disease

KW - Hemorrhage

KW - Infarction

KW - Microvascular infiltrates

KW - Perivascular microangiopathy

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Cerebral Microvascular Erdheim-Chester Disease: A Perivascular Hematopoietic Vasculopathy

Abstract

Keywords

ASJC Scopus subject areas

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