Acute Hemorrhagic Leukoencephalitis: A Cause of Acute Brainstem Dysfunction

Posey, Kristi; Alpert, Jack N.; Langford, Lauren A.; Yeakley, Joel W.

doi:10.1097/00007611-199408000-00023

Cited by 6 publications

(5 citation statements)

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“…In cases of ADEM, the prognosis is favorable, with 60–80% of cases experiencing complete recovery [ 3 , 4 ]. However, AHLE is associated with rapidly deteriorating focal and diffuse neurological symptoms leading to death within 2–14 days [ 5 - 8 ]. The few patients that survive AHLE usually have significant residual neurological symptoms [ 9 ].…”

Section: Resultsmentioning

confidence: 99%

Acute hemorrhagic demyelination in a murine model of multiple sclerosis

Pirko

Suidan

Rodriguez

et al. 2008

J Neuroinflammation

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Acute hemorrhagic leukoencephalomyelitis (AHLE) is a rare neurological condition characterized by the development of acute hemorrhagic demyelination and high mortality. The pathomechanism of AHLE, as well as potential therapeutic approaches, have remained elusive due to the lack of suitable animal models. We report the first murine model of AHLE using a variation of the Theiler's Murine Encephalitis Virus (TMEV) MS model. During acute TMEV infection, C57BL/6 mice do not normally undergo demyelination. However, when 7 day TMEV infected C57BL/6 mice are intravenously administered the immunodominant CD8 T cell peptide, VP2121–130, animals develop characteristics of human AHLE based on pathologic, MRI and clinical features including microhemorrhages, increased blood-brain barrier permeability, and demyelination. The animals also develop severe disability as assessed using the rotarod assay. This study demonstrates the development of hemorrhagic demyelination in TMEV infected C57BL/6 mice within 24 hours of inducing this condition through intravenous administration of CD8 T cell restricted peptide. This study is also the first demonstration of rapid demyelination in a TMEV resistant non-demyelinating strain without transgenic alterations or pharmacologically induced immunosuppression.

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Section: Resultsmentioning

confidence: 99%

Acute hemorrhagic demyelination in a murine model of multiple sclerosis

Pirko

Suidan

Rodriguez

et al. 2008

J Neuroinflammation

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“…The usual presentation of AHL is a prodromal mild viral infection followed by neurological symptoms, rapid deterioration, and death [Suchowersky et al, 1983;Geerts et al, 1991;Posey et al, 1994]. Patients have survived when treated with large doses of steroids [Huang et al, 1988].…”

Section: Discussionmentioning

confidence: 99%

Acute hemorrhagic leukoencephalitis in male sibs

et al. 2002

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Male siblings developed acute hemorrhagic leukoencephalitis (AHL) at age five months and eight months, respectively. This rare condition has not been reported in individuals less than two years of age or with a recurrence in the family. The diagnosis was based on neuropathologic features after ruling out potentially mimicking conditions. Genetic counseling of early onset AHL should include the possibility of recurrence in siblings.

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“…Furthermore, the specificity and pathological role of GluRε2 NR2B antibodies for NHALE should be investigated. ( Posey et al, 1994). The MRIs of AHLE patients present with edema, white matter lesions, and petechial hemorrhage (Gibbs et al, 2005;Fugate et al, 2010;Pinto et al, 2011).…”

Section: Discussionmentioning

confidence: 99%

“…(Pinto et al, 2011). Usually, the patient's level of consciousness progressively declines, resulting in coma (Posey et al, 1994). AHLE progresses rapidly, causing an accelerated deterioration of the patient.…”

Section: Discussionmentioning

confidence: 99%

“…ADEM typically has an overall favorable prognosis, with 60-80% of patients showing a complete recovery (Dale et al, 2000;Gibbs et al, 2005;Rust, 2000;Tenembaum et al, 2002). AHLE, which is also known as Hurst's disease, has been considered a severe form of ADEM and has a very poor prognosis, usually resulting in death in 2-14 days (Geerts et al, 1991;Hart & Earle 1975;Pinto et al, 2011;Posey et al, 1994;Suchowersky et al, 1983). This disease is characterized by extensive BBB disruption and microhemorrhage formation.…”

Section: Introductionmentioning

confidence: 99%

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The pathogenesis of encephalitis

Owens¹,

Hassan‐Zahraee²,

Babcock³

et al. 2001

NeuroImmune Biology

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He had a postdoctoral training at the Center for Infectious Disease and Vaccine Research at the University of Massachusetts Medical School in the USA. His research interest is on the pathogenesis of neurotropic viruses, especially encephalitic viruses that include Japanese encephalitis virus and tick-borne encephalitis virus. Contents Preface XIII Part Diagnosis and Clinical Symptoms of Encephalitis 1 Chapter Biomarkers of Encephalitis 3 Dafna Bonneh-Barkay Chapter Neuropathologic Diagnosis of Central Nervous System Viral Diseases 19 Kymberly A. Gyure Chapter The Neuropsychiatric Consequences of Childhood Encephalitis: A Review of Cases from Middle-Eastern Countries 39

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Acute Hemorrhagic Leukoencephalitis: A Cause of Acute Brainstem Dysfunction

Cited by 6 publications

References 0 publications

Acute hemorrhagic demyelination in a murine model of multiple sclerosis

Acute hemorrhagic demyelination in a murine model of multiple sclerosis

Acute hemorrhagic leukoencephalitis in male sibs

The pathogenesis of encephalitis

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