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Posterior reversible encephalopathy syndrome in neuromyelitis optica spectrum disorders

Neurology, 2009-02, Vol.72 (8), p.712-717 [Peer Reviewed Journal]

2009 INIST-CNRS ;ISSN: 0028-3878 ;EISSN: 1526-632X ;DOI: 10.1212/01.wnl.0000343001.36493.ae ;PMID: 19237699 ;CODEN: NEURAI

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Title:
Posterior reversible encephalopathy syndrome in neuromyelitis optica spectrum disorders
Author: MAGANA, S. M ; MATIELLO, M ; PITTOCK, S. J ; MCKEON, A ; LENNON, V. A ; RABINSTEIN, A. A ; SHUSTER, E ; KANTARCI, O. H ; LUCCHINETTI, C. F ; WEINSHENKER, B. G
Subjects: Adolescent ; Adult ; Antibodies - blood ; Aquaporin 4 - immunology ; Autoimmunity ; Biological and medical sciences ; Body Water - metabolism ; Brain - metabolism ; Brain - pathology ; Brain Edema - diagnosis ; Brain Edema - epidemiology ; Brain Edema - etiology ; Brain Edema - immunology ; Brain Edema - psychology ; Cerebrovascular Disorders - complications ; Child ; Comorbidity ; Confusion - etiology ; Consciousness Disorders - etiology ; Diffusion Magnetic Resonance Imaging ; Dose-Response Relationship, Drug ; Female ; Gadolinium ; Humans ; Immunoglobulins, Intravenous - adverse effects ; Malformations of the nervous system ; Medical sciences ; Methylprednisolone - administration & dosage ; Methylprednisolone - adverse effects ; Middle Aged ; Neurology ; Neuromyelitis Optica - complications ; Neuromyelitis Optica - immunology ; Remission, Spontaneous ; Syndrome ; Time Factors ; Young Adult
Is Part Of: Neurology, 2009-02, Vol.72 (8), p.712-717
Description: Posterior reversible encephalopathy syndrome (PRES) is characterized by vasogenic subcortical edema without infarction. It has been associated with hypertensive crises and with immunosuppressive medications but not with neuromyelitis optica (NMO). We reviewed the clinical and neuroimaging features of five NMO-immunoglobulin G (IgG) seropositive white women who experienced an episode of PRES and had a coexisting NMO spectrum disorder (NMOSD). We also tested for the aquaporin-4 (AQP4) water channel autoantibody (NMO-IgG) in 14 patients from an independently ascertained cohort of individuals with PRES. All five patients developed abrupt confusion and depressed consciousness consistent with PRES. The encephalopathy resolved completely within 7 days. Comorbid conditions or interventions recognized to be associated with PRES included orthostatic hypotension with supine hypertension, plasma exchange, IV immunoglobulin treatment, and high-dose IV methylprednisolone. Brain MRI studies revealed bilateral T2-weighted (T2W) hyperintense signal abnormalities, primarily in frontal, parieto-occipital, and cerebellar regions. Three patients had highly symmetric lesions and three had gadolinium-enhancing lesions. Follow-up neuroimaging revealed partial or complete disappearance of T2W hyperintensity or gadolinium-enhancing lesions in all five patients. Patients with PRES without NMOSD were uniformly NMO-IgG seronegative. Brain lesions in some patients with neuromyelitis optica spectrum disorder (NMOSD) may be accompanied by vasogenic edema and manifest as posterior reversible encephalopathy syndrome (PRES). Water flux impairment due to aquaporin-4 autoimmunity may predispose to PRES in patients with NMOSD who experience blood pressure fluctuations or who are treated with therapies that can cause rapid fluid shifts.
Publisher: Hagerstown, MD: Lippincott Williams & Wilkins
Language: English
Identifier: ISSN: 0028-3878
EISSN: 1526-632X
DOI: 10.1212/01.wnl.0000343001.36493.ae
PMID: 19237699
CODEN: NEURAI
Source: MEDLINE
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Posterior reversible encephalopathy syndrome in neuromyelitis optica spectrum disorders

2009 INIST-CNRS ;ISSN: 0028-3878 ;EISSN: 1526-632X ;DOI: 10.1212/01.wnl.0000343001.36493.ae ;PMID: 19237699 ;CODEN: NEURAI

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