Fulminant Demyelinating Diseases

Megan R. Rahmlow, Orhun H Kantarci

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

Fulminant demyelinating disease is a heading that covers acute disseminated encephalomyelitis and its variant acute hemorrhagic leukoencephalitis (Hurst disease), severe relapses of multiple sclerosis (MS), variants of MS (tumefactive MS, Marburg variant, Balo concentric sclerosis, myelinoclastic diffuse sclerosis), and neuromyelitis optica-spectrum disorders associated with aquaporin autoimmunity. These categories of inflammatory demyelinating disease often prompt hospital admission and many necessitate intensive care monitoring due to the aggressive nature of the illness and associated neurologic morbidity. In this review, we highlight the discriminating clinical, radiographic, and pathologic features of these disorders. Acute management is often accomplished with use of high-dose intravenous steroids and plasma exchange. Aggressive disease may respond to immunosuppression. Prognosis for recovery varies among the disorders but most patients improve. Factors influencing outcome are also discussed.

Original languageEnglish (US)
Pages (from-to)81-91
Number of pages11
JournalThe Neurohospitalist
Volume3
Issue number2
DOIs
StatePublished - 2013

Fingerprint

Acute Hemorrhagic Leukoencephalitis
Demyelinating Diseases
Diffuse Cerebral Sclerosis of Schilder
Multiple Sclerosis
Acute Disseminated Encephalomyelitis
Neuromyelitis Optica
Aquaporins
Plasma Exchange
Critical Care
Autoimmunity
Immunosuppression
Nervous System
Steroids
Morbidity
Recurrence

Keywords

  • ADEM
  • fulminant MS
  • Marburg
  • neuromyelitis optica
  • tumefactive MS

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Fulminant Demyelinating Diseases. / Rahmlow, Megan R.; Kantarci, Orhun H.

In: The Neurohospitalist, Vol. 3, No. 2, 2013, p. 81-91.

Research output: Contribution to journalArticle

Rahmlow, Megan R. ; Kantarci, Orhun H. / Fulminant Demyelinating Diseases. In: The Neurohospitalist. 2013 ; Vol. 3, No. 2. pp. 81-91.
@article{036dce765787488894ea7baa3fef2333,
title = "Fulminant Demyelinating Diseases",
abstract = "Fulminant demyelinating disease is a heading that covers acute disseminated encephalomyelitis and its variant acute hemorrhagic leukoencephalitis (Hurst disease), severe relapses of multiple sclerosis (MS), variants of MS (tumefactive MS, Marburg variant, Balo concentric sclerosis, myelinoclastic diffuse sclerosis), and neuromyelitis optica-spectrum disorders associated with aquaporin autoimmunity. These categories of inflammatory demyelinating disease often prompt hospital admission and many necessitate intensive care monitoring due to the aggressive nature of the illness and associated neurologic morbidity. In this review, we highlight the discriminating clinical, radiographic, and pathologic features of these disorders. Acute management is often accomplished with use of high-dose intravenous steroids and plasma exchange. Aggressive disease may respond to immunosuppression. Prognosis for recovery varies among the disorders but most patients improve. Factors influencing outcome are also discussed.",
keywords = "ADEM, fulminant MS, Marburg, neuromyelitis optica, tumefactive MS",
author = "Rahmlow, {Megan R.} and Kantarci, {Orhun H}",
year = "2013",
doi = "10.1177/1941874412466873",
language = "English (US)",
volume = "3",
pages = "81--91",
journal = "The Neurohospitalist",
issn = "1941-8744",
publisher = "Sage Publications",
number = "2",

}

TY - JOUR

T1 - Fulminant Demyelinating Diseases

AU - Rahmlow, Megan R.

AU - Kantarci, Orhun H

PY - 2013

Y1 - 2013

N2 - Fulminant demyelinating disease is a heading that covers acute disseminated encephalomyelitis and its variant acute hemorrhagic leukoencephalitis (Hurst disease), severe relapses of multiple sclerosis (MS), variants of MS (tumefactive MS, Marburg variant, Balo concentric sclerosis, myelinoclastic diffuse sclerosis), and neuromyelitis optica-spectrum disorders associated with aquaporin autoimmunity. These categories of inflammatory demyelinating disease often prompt hospital admission and many necessitate intensive care monitoring due to the aggressive nature of the illness and associated neurologic morbidity. In this review, we highlight the discriminating clinical, radiographic, and pathologic features of these disorders. Acute management is often accomplished with use of high-dose intravenous steroids and plasma exchange. Aggressive disease may respond to immunosuppression. Prognosis for recovery varies among the disorders but most patients improve. Factors influencing outcome are also discussed.

AB - Fulminant demyelinating disease is a heading that covers acute disseminated encephalomyelitis and its variant acute hemorrhagic leukoencephalitis (Hurst disease), severe relapses of multiple sclerosis (MS), variants of MS (tumefactive MS, Marburg variant, Balo concentric sclerosis, myelinoclastic diffuse sclerosis), and neuromyelitis optica-spectrum disorders associated with aquaporin autoimmunity. These categories of inflammatory demyelinating disease often prompt hospital admission and many necessitate intensive care monitoring due to the aggressive nature of the illness and associated neurologic morbidity. In this review, we highlight the discriminating clinical, radiographic, and pathologic features of these disorders. Acute management is often accomplished with use of high-dose intravenous steroids and plasma exchange. Aggressive disease may respond to immunosuppression. Prognosis for recovery varies among the disorders but most patients improve. Factors influencing outcome are also discussed.

KW - ADEM

KW - fulminant MS

KW - Marburg

KW - neuromyelitis optica

KW - tumefactive MS

UR - http://www.scopus.com/inward/record.url?scp=84992828894&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84992828894&partnerID=8YFLogxK

U2 - 10.1177/1941874412466873

DO - 10.1177/1941874412466873

M3 - Article

AN - SCOPUS:84992828894

VL - 3

SP - 81

EP - 91

JO - The Neurohospitalist

JF - The Neurohospitalist

SN - 1941-8744

IS - 2

ER -