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Acute Hemorrhagic Leukoencephalitis (Hurst Syndrome)

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A fulminant and often fatal demyelinating disease of the brain which primarily affects young adults and children. Clinical features include the rapid onset of weakness, SEIZURES, and COMA. It may follow a viral illness or MYCOPLASMA PNEUMONIAE infections but in most instances there is no precipitating event. Pathologic examination reveals marked perivascular demyelination and necrosis of white matter with microhemorrhages. (Adams et al., Principles of Neurology, 6th ed, pp924-5)

Also Known As:

Hurst Syndrome; Syndrome, Hurst; Leukoencephalitis, Acute Hemorrhagic; Encephalomyelitis, Acute Necrotizing Hemorrhagic; Encephalomyelitis, Hemorrhagic Necrotizing; Encephalomyelitis, Necrotizing Hemorrhagic; Hemorrhagic Encephalomyelitis, Necrotizing; Hemorrhagic Necrotizing Encephalomyelitis; Hurst's Disease; Leukoencephalitis Acuta Hemorrhagica; Leukoencephalitis, Subacute Hemorrhagic; Necrotizing Encephalomyelitis, Hemorrhagic; Acute Hemorrhagic Leukoencephalitides; Acute Necrotizing Encephalitides; Acute Necrotizing Encephalitis; Disease, Hurst; Disease, Hurst's; Encephalitides, Acute Necrotizing; Encephalomyelitides, Hemorrhagic Necrotizing; Hemorrhagic Encephalomyelitides, Necrotizing; Hemorrhagic Leukoencephalitides, Acute; Hemorrhagic Leukoencephalitides, Subacute; Hemorrhagic Leukoencephalitis, Acute; Hemorrhagic Leukoencephalitis, Subacute; Hursts Disease; Leukoencephalitides, Acute Hemorrhagic; Leukoencephalitides, Subacute Hemorrhagic; Necrotizing Encephalitides, Acute; Necrotizing Encephalitis, Acute; Necrotizing Encephalomyelitides, Hemorrhagic; Necrotizing Hemorrhagic Encephalomyelitides; Subacute Hemorrhagic Leukoencephalitides; Subacute Hemorrhagic Leukoencephalitis; Encephalitis, Acute Necrotizing; Hurst Disease; Necrotizing Hemorrhagic Encephalomyelitis

Networked: 14 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Acute Hemorrhagic Leukoencephalitis (Hurst Syndrome)
2.	Malformations of Cortical Development
3.	Lissencephaly
4.	Herpes Simplex Encephalitis (Herpes Encephalitis)
5.	Neurocutaneous Syndromes (Phakomatoses)

Experts

1.	Takanashi, Jun-ichi: 1 article (05/2014)
2.	Putnam, Christopher D: 1 article (01/2013)
3.	Gandhi, Chhavi: 1 article (01/2013)
4.	Newbury, Robert O: 1 article (01/2013)
5.	Giclas, Patricia C: 1 article (01/2013)
6.	Aceves, Seema S: 1 article (01/2013)
7.	Hoffman, Hal M: 1 article (01/2013)
8.	Broderick, Lori: 1 article (01/2013)
9.	Peterson, Karin S: 1 article (01/2013)
10.	Sheets, Robert M: 1 article (01/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Acute Hemorrhagic Leukoencephalitis:

1.	AntigensIBA 01/01/1979 - "Intracranial inoculation of suckling hamsters produces an acute necrotizing encephalitis with large amounts of infectious virus and virus antigen in the brain. " 08/16/2002 - "Acute hemorrhagic leukoencephalitis (AHL), or Hurst disease is a rare, usually fatal, disease, probably due to an autoimmune cross reaction against myelin antigens present in the central nervous system, and which forms a spectrum of post infeccious demyelinating diseases with acute disseminated encephalomyelitis. "
2.	Complement Factor I (C3b Inactivator)IBA 01/01/2013 - "Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis."
3.	N-Methylaspartate (NMDA)IBA 05/01/2014 - "In this article, I reviewed radiological findings associated with seizures (reduced diffusion and swelling of hippocampus and cortex, and a reversible splenial lesion), and lesions causing epilepsy and seizures, such as congenital abnormality of the brain (holoprosencephaly, hemimegalencephaly, lissencephaly, heterotopia, polymicrogyria, schizencephaly, and focal cortical dysplasia), neurocutaneous syndromes (tuberous sclerosis and Sturge-Weber syndrome), vascular disorders (moyamoya disease/syndrome and cavernous angioma), and encephalitis/encephalopathy (herpes encephalitis, anti-NMDA receptor encephalitis, acute necrotizing encephalopathy of childhood, and acute encephalopathy with biphasic seizures and late reduced diffusion)."
4.	SteroidsIBA 11/01/2014 - "We report a case of an unusual presentation of acute hemorrhagic leukoencephalitis with autoimmune myopathy and a complete recovery with steroids and plasmapheresis. "
5.	ParaffinIBA 01/01/1988 - "These results demonstrate that immunocytochemistry and ISH are techniques of comparable sensitivity (72%) for detection of HSV in paraffin sections of acute necrotizing encephalitis brains; their combined use enhanced sensitivity, in our hands, to 84%. "
6.	OxidoreductasesIBA 05/01/2011 - "Severe 5-MTHF depletion (n = 8; range, 0.6-13 nmol/L) was detected in severe MTHF reductase deficiency, Kearns-Sayre syndrome, biotin-responsive striatal necrosis, acute necrotizing encephalitis of Hurst, and FOLR1 defect. "
7.	Methylprednisolone FDA LinkGeneric 10/01/2001 - "Successful recovery after high-dose intravenous methylprednisolone in acute hemorrhagic leukoencephalitis."
8.	Biotin (Vitamin H)FDA Link 05/01/2011 - "Severe 5-MTHF depletion (n = 8; range, 0.6-13 nmol/L) was detected in severe MTHF reductase deficiency, Kearns-Sayre syndrome, biotin-responsive striatal necrosis, acute necrotizing encephalitis of Hurst, and FOLR1 defect. "
9.	AntibodiesIBA 01/01/1975 - "By analogy with the simple and the hyperacute forms of EAE, the myelinotoxicity may result from sensitized lymphocytes alone in the perivenous encephalomyelitis, from an association of circulating antibodies and lymphocytes in Hurst's disease. "
10.	Lennox-Gastaut type Epileptic encephalopathyIBA 05/01/2014 - "In this article, I reviewed radiological findings associated with seizures (reduced diffusion and swelling of hippocampus and cortex, and a reversible splenial lesion), and lesions causing epilepsy and seizures, such as congenital abnormality of the brain (holoprosencephaly, hemimegalencephaly, lissencephaly, heterotopia, polymicrogyria, schizencephaly, and focal cortical dysplasia), neurocutaneous syndromes (tuberous sclerosis and Sturge-Weber syndrome), vascular disorders (moyamoya disease/syndrome and cavernous angioma), and encephalitis/encephalopathy (herpes encephalitis, anti-NMDA receptor encephalitis, acute necrotizing encephalopathy of childhood, and acute encephalopathy with biphasic seizures and late reduced diffusion)."

Therapies and Procedures

1.	Surgical Decompression 07/01/1963 - "ACUTE HEMORRHAGIC LEUKOENCEPHALITIS; A CLINICAL AND ELECTRON-MICROSCOPIC REPORT OF 2 PATIENTS TREATED WITH SURGICAL DECOMPRESSION."
2.	Intensive Care (Surgical Intensive Care) 09/01/1992 - "Intensive care management of acute hemorrhagic leukoencephalitis with favorable neurologic outcome."
3.	Plasmapheresis 11/01/2014 - "We report a case of an unusual presentation of acute hemorrhagic leukoencephalitis with autoimmune myopathy and a complete recovery with steroids and plasmapheresis. "
4.	Plasma Exchange 06/01/2007 - "Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature."
5.	Curettage 04/01/1972 - "Acute necrotizing encephalitis complicating bifrontal craniotomy and pituitary curettage. "