Full recovery after acute hemorrhagic leukoencephalitis (Hurst's disease)

Klein, Christopher J.; Wijdicks, Eelco F. M.; Earnest, Franklin

doi:10.1007/s004150070060

Cited by 33 publications

(22 citation statements)

References 6 publications

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“…Some of these areas exhibit gadolinium-enhancement on T1-weighted images 16 . However, in some case reports of AHLE in the literature, MRI demonstrates a more diffuse compromise of the brain, affecting not only the white matter, but also extensive cortical areas like our patient 17 . The patient's initial CSF examination failed to demonstrate any abnormality.…”

Section: Discussionmentioning

confidence: 47%

“…The more severe and more acute clinical symptomatology discriminates AHLE from ADEM 8,24 . In addition, MRI may establish the diagnosis in the appropriate clinical setting 17 . Of note is the fact that until recently AHLE was misdiagnosed as a viral encephalitis 17 .…”

Section: Fig 2 Axial T2-weighted Mr Image Demonstrates Areas Of T2-hmentioning

confidence: 99%

“…In addition, MRI may establish the diagnosis in the appropriate clinical setting 17 . Of note is the fact that until recently AHLE was misdiagnosed as a viral encephalitis 17 . AHLE is a rare demyelinating disease first described by Hurst 25 in 1941.…”

Section: Fig 2 Axial T2-weighted Mr Image Demonstrates Areas Of T2-hmentioning

confidence: 99%

“…Occasionally patients may be discharged with no neurological sequelae 5,26 . Recently two case reports 17,24 demonstrated that treatment with high-dose intravenous corticosteroid (e.g. methilprednisolone 1 g/day for 3 days 24 ) was followed by full recovery.…”

Section: Fig 2 Axial T2-weighted Mr Image Demonstrates Areas Of T2-hmentioning

confidence: 99%

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Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report

Martins

Teixeira-Jr

Lana-Peixoto

2004

Arq. Neuro-Psiquiatr.

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Acute hemorrhagic leukoencephalitis (AHLE) is a more severe form of acute disseminated encephalomyelities (ADEM) characterized by a fulminant clinical course and the presence of hemorrhagic necrosis of the white matter. We report the case of a 57-year-old woman who developed delirium following a respiratory infection. Magnetic resonance imaging of the brain disclosed signal abnormalities in the frontal and temporal lobes, usually found in herpes simplex encephalitis (HSE). Gram stain, India ink and acid-fast bacilli staining were all negative in CSF as was a polymerase chain reaction (PCR) for herpes simplex virus. A diagnosis of AHLE was made and the patient was treated with IV methylprednisolone 1g/day for 5 days. Despite treatment, the patient developed several neurological sequelae compatible with the severity of her illness.

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Section: Discussionmentioning

confidence: 47%

Section: Fig 2 Axial T2-weighted Mr Image Demonstrates Areas Of T2-hmentioning

confidence: 99%

Section: Fig 2 Axial T2-weighted Mr Image Demonstrates Areas Of T2-hmentioning

confidence: 99%

Section: Fig 2 Axial T2-weighted Mr Image Demonstrates Areas Of T2-hmentioning

confidence: 99%

See 2 more Smart Citations

Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report

Martins

Teixeira-Jr

Lana-Peixoto

2004

Arq. Neuro-Psiquiatr.

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“…MRI reveals multiple, asymmetric, and large signal abnormalities in the CNS [71]. Acute hemorrhagic leukoencephalitis [AHL] of Weston Hurst is an aggressive and hemorrhagic variant of ADEM usually triggered by a respiratory infection [72,73].…”

Section: Acute Disseminated Encephalomyelitis (Adem)mentioning

confidence: 99%

Management of acute viral encephalitis in Brazil

Domingues

Teixeira

2009

Braz J Infect Dis

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Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation

Pinto

Taipa

Moreira

et al. 2011

Magnetic Resonance Imaging

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Acute hemorrhagic leukoencephalitis (AHLE) is a rare and fulminant demyelinating disease considered to be the most severe form of acute disseminated encephalomyelitis (ADEM). A 70-year-old man was admitted to our emergency department (ED) after 1 week of unspecific abdominal symptoms and moderate fever in the first 3 days. Within the ED he developed a rapid onset coma and flaccid tetraparesis. Cerebrospinal fluid (CSF) analysis showed mild polymorphonuclear pleocytosis and magnetic resonance imaging (MRI) revealed supratentorial focal white matter lesions and diffuse involvement of the medulla and spinal cord. A presumptive diagnosis of ADEM was made and the patient was treated with corticosteroids followed by intravenous immunoglobulin. His neurological state did not improve and the MRI on day 8 after admission showed an increased number of lesions, mainly in the brainstem, with hemorrhagic foci. The patient died the following day and pathological features confirmed the diagnosis of AHLE. This is a unique presentation of a rare disease with detailed MRI characteristics and pathological confirmation. Although this condition is usually fatal, early recognition and aggressive therapeutic management can facilitate survival.

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Full recovery after acute hemorrhagic leukoencephalitis (Hurst's disease)

Cited by 33 publications

References 6 publications

Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report

Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report

Management of acute viral encephalitis in Brazil

Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation

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