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At least 19 recordsLinked to original sources
PubMed2002

Detection of infectious agents in brain of patients with acute hemorrhagic leukoencephalitis.

Acute hemorrhagic leukoencephalitis (AHL) is a rare and usually fatal disorder characterized clinically by an acute onset of neurologic abnormalities. It may occur in association with a viral illness or vaccination. Radiology and brain biopsy are essential for the diagnosis. The etiology of AHL is unclear. We postulated that viral/bacterial infection might be responsible, directly or through an immune-mediated mechanism, for this acute inflammatory myelinopathy. Fifteen cases of AHL were studied. Infectious agents, including varicella zoster virus (VZV), herpes simplex virus (HSV), human herpes virus-6 (HHV-6), cytomegalovirus, Epstein-Barr virus, and Mycoplasma, were investigated in brain specimens using the polymerase chain reaction (PCR), reverse transcriptase (RT)-PCR, and immunohistochemistry. Using PCR, HSV DNA was found in four cases, VZV DNA in two, and HHV-6 DNA in one. Among the control cases, two were HSV DNA positive. Further investigation to detect HSV RNA and antigens in HSV DNA-positive cases revealed that two cases with AHL were both HSV RNA and antigen positive. AHL is a hyperacute disease, which is considered the most acute form of acute disseminated encephalomyelitis (ADEM). Our findings suggests that a viral infection may be implicated in its pathogenesis, most likely through an indirect mechanism; however, as only a few cases of this rare disease were examined, statistical significance was not achieved. As a number of patients with disorders of the ADEM group may progress to develop multiple sclerosis (MS), we argue that an organism that has produced the former may remain in the brain tissue and be subsequently involved in the production of a self-sustained disorder such as MS.

Acute Disease↗
PubMed1994

Acute hemorrhagic leukoencephalitis: a cause of acute brainstem dysfunction.

A 37-year-old female physician was admitted to the hospital with severe headache, facial and hand paresthesias, dysarthria, and ataxia. Neurologic examination disclosed signs of brain stem dysfunction. There was rapid neurologic deterioration, and she died in 28 hours. Postmortem studies showed the characteristic features of acute hemorrhagic leukoencephalitis.

Acute Disease↗
PubMed1999

Acute hemorrhagic leukoencephalitis in patients with acute myeloid leukemia in hematologic complete remission.

The authors describe the cases of three patients affected by acute myeloid leukemia, in complete remission, who rapidly developed neurologic symptoms leading to death. Neither clinical characteristics, nor radiological or microbiological procedures, allowed an etiological diagnosis of the neurologic syndrome. Post-mortem examination of the brain showed both macroscopic and microscopic findings compatible with acute hemorrhagic leukoencephalitis. The difficulty in distinguishing this entity from other CNS disease-related complications (e.g. leukemia infiltration, drug toxicity, hemorrhages) should not lead to an underestimation of the true incidence of this complication. We believe that with more attention to the possibility of this complication there would probably be both a greater possibility of collecting clinical informations about the real impact of this dramatic disease and a stronger hope of finding the right treatment for it.

Acute Disease↗
PubMed1975

[The neurosurgical forms of Hurst's acute hemorrhagic leukoencephalitis].

Within the group of acute encephalitis, H.A.H.L. is individualized mostly by its post mortem pathology. But several cases of recovery were recently published, the diagnostic being based on biopsy material. In 4 of our 8 cases, a comparison between the biopsy and post mortem examinations was possible : in/the diagnostic of H.A.H.L. was unequivocally confirmed, in 2 H.A.H.L. is nearly certain, in 1 it is doubtful. These considerations do not contradict the necessity of an emergency brain decompression in many instances of acute encephalitic states.

Acute Disease↗
PubMed2002

Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report.

A 15-year-old girl presented with meningeal irritation and bilateral cerebral signs after contracting influenza. A lumbar puncture revealed bloody cerebrospinal fluid and polymorphonuclear predominant pleocytosis with an elevated protein level and normal glucose level. Computed tomography showed a hematoma in the right basal ganglia and lateral ventricles. Symmetrical low density areas were also noted in the bilateral white matter. The preliminary diagnosis was hemorrhagic cerebrovascular disease of unknown cause. However, her neurological condition deteriorated. Magnetic resonance (MR) imaging showed diffuse high intensity signals in the bilateral white matter and small spotty lesions, indicating hemorrhages in various stages. The final diagnosis was acute hemorrhagic leukoencephalitis (AHL). However, high-dose steroid administration and plasmapheresis failed to improve her condition. Hypothermia could not control her intracranial pressure and she died 12 days after admission. The neuroimaging findings indicated the histological characteristics of AHL, but the hematoma formation is rare. AHL is a fulminant form of brain demyelination and can be fatal, so early diagnosis and aggressive treatment are important for successful recovery. Therefore, early investigation by MR imaging is necessary.

Adolescent↗
PubMed2004

Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection.

A 16-year-old girl presented signs of a common cold in combination with a hemolytic crisis. Within 3 days, she developed reduced consciousness and hemiparesis subsequently followed by coma. CT and MRI scans revealed evidence for raised intracranial pressure and an extensive inflammatory process extending from the brain stem up to the thalamus. The patient died within 3 weeks after onset of first symptoms of intracranial pressure despite maximum intensive care. Neuropathological examination revealed disseminated necrotic lesions and perivascular hemorrhages characteristic for acute hemorrhagic leukoencephalitis (Hurst's disease), mainly of the brain stem, diencephalon and cerebellum. Serological results, in situ hybridization and PCR analysis demonstrated an acute Epstein-Barr virus (EBV) infection of the central nervous system. To our knowledge, this is the first reported case of Hurst's disease linked to EBV.

Adolescent↗
PubMed2002

Acute hemorrhagic leukoencephalitis in male sibs.

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.

Female↗
PubMed1997

Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child.

A case of acute hemorrhagic leukoencephalitis (AHLE) in a 6-year-old girl is reported. The presentation was typical for acute hemorrhagic leukoencephalitis, with acute onset of a rapidly progressive neurologic disorder with asymmetric involvement of brain, with polymorphonuclear predominant peripheral leukocytosis and cerebrospinal fluid pleocytosis. Cerebrospinal fluid findings not previously reported included elevation of IgG and the presence of myelin basic protein. Additional previously unreported findings were striking abnormalities on magnetic resonance imaging (MRI) of the brain, in contrast to normal findings on computed tomography (CT). The child was treated with high dose intravenous steroids and made a full recovery, with a parallel disappearance of all of her cerebrospinal fluid abnormalities and almost all of her abnormalities on MRI. Detailed examination of cerebrospinal fluid and MRI of brain should facilitate early diagnosis in other cases of suspected acute hemorrhagic leukoencephalitis and high-dose steroid therapy may lead to improved clinical outcomes.

Anti-Inflammatory Agents↗
PubMed2005

Acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage associated with herpes simplex virus type I.

Acute hemorrhagic leukoencephalitis (AHL) is a rare and usually fatal disorder characterized by an acute onset of neurological abnormalities. It may occur in association with a viral illness or vaccination. Radiology and brain biopsy are essential for the diagnosis. We present a case of AHL mimicking or manifesting as intracerebral hemorrhage associated with herpes simplex virus. A 3-year-old boy was admitted to our hospital with cerebral hemorrhage. The findings of cerebrospinal fluid, MRI and electroencephalogram indicated acute hemorrhagic leukoencephalitis associated with herpes simplex virus involving the left parietal cortex (hemorrhage) and the right temporal lobe (encephalitis). The patient received intravenous dexamethasone (1.5 mg/kg/24 h divided q6 (six times a day) for 5 days, tapered slowly to discontinuation over the next 5 days) and aciclovir (15 mg/kg/every 6 h for 14 days). His clinical condition markedly improved, and after 3 weeks he was discharged from the hospital with moderate right hemiparesis. Mild hemiparesis with normal psychometric testing was determined when he was checked 6 months later. AHL remains misdiagnosed as viral encephalitis because it produces different protean clinical forms. Pediatric patients admitted with cerebral hemorrhage and infarct should be evaluated for presence of hemorrhagic encephalitis-causing agents, especially for herpes simplex. Since, as in our case, treatment with corticosteroid and acyclovir markedly improves conditions of herpes simplex-caused AHL, we suggest that early diagnosis and treatment is of importance for the prognosis.

Acyclovir↗
PubMed2004

Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report.

Acute hemorrhagic leukoencephalitis (AHLE) is a more severe form of acute disseminated encephalomyelitis (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 i.v. methylprednisolone 1g/day for 5 days. Despite treatment, the patient developed several neurological sequelae compatible with the severity of her illness.

Anti-Inflammatory Agents↗
PubMed2002

Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review.

Acute hemorrhagic leukoencephalitis (AHLE) is a rare, fulminant CNS demyelinating condition usually diagnosed at autopsy. We report the clinical, laboratory, radiographic, and pathologic features of the first nonfatal case of pediatric AHLE confirmed by brain biopsy. Pathologic diagnosis of this condition may be critical to exclude more-common processes and to expedite the decision to administer high-dose corticosteroid therapy, which is potentially lifesaving.

Anti-Inflammatory Agents↗
PubMed1991

Acute hemorrhagic leukoencephalitis.

We report an autopsy proven case of acute hemorrhagic leukoencephalitis. There was a very rapid evolution leading to death in less than 24 hours. CT of the brain revealed widespread hypodensities in both frontal lobes. In the cerebrospinal fluid as well as in the peripheral blood, there was a moderate leucocytosis. The literature is reviewed.

Acute Disease↗
PubMed1986

An autopsied case of acute hemorrhagic leukoencephalitis.

This is a clinico-pathological study of one case of acute hemorrhagic leukoencephalitis occurring in a patient with lung cancer. The main changes observed by light microscopy were in the white matter. All of these changes were the result of hemorrhage and demyelination around the venous vessels. Glial mesenchymal reactions were minimal. In view of these findings, this case supports the hypothesis that immune mechanisms play a role in the pathogenesis of neuropsychiatric symptoms during malignant illnesses. Here we will discuss the problem of differential diagnoses of acute hemorrhagic leukoencephalitis and its close morphologic similarities to perivenous encephalitis. Acute hemorrhagic leukoencephalitis and perivenous encephalitis may be a single entity of a demyelinating disease.

Acute Disease↗
PubMed2005

Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report.

We report a patient with acute hemorrhagic leukoencephalitis with a focus on serial MR imaging findings. Initial MR imaging of a 42-year-old woman revealed a 2.5-cm focal nonhemorrhagic lesion in the left thalamus and internal capsule. Twenty-four days later, fever and altered consciousness developed, and MR imaging showed huge masslike lesions in both frontal lobes, mainly involving the white matter and the genu of the corpus callosum, with massive edematous swelling that contained multifocal small hemorrhages. Most lesions showed high apparent diffusion coefficient value with peripheral small areas of low apparent diffusion coefficient. On follow-up MR imaging obtained 49 days after initial MR imaging, the lesions progressed with increase in extent and development of rim-enhancing necrosis, despite steroid therapy. Following stereotactic biopsy and subsequent high-dose steroid treatment, the patient recovered with some neurologic sequelae. MR imaging obtained at 72 and 126 days revealed residual necrosis and cerebromalacia in the both frontal lobes.

Adrenal Cortex Hormones↗
PubMed1991

Acute hemorrhagic leukoencephalitis. A successful recovery.

A 50-year-old woman developed acute hemorrhagic leukoencephalitis approximately 7 days after the onset of a benign respiratory infection. Mycoplasmal pneumonia was suspected because of Coomb's positive hemolysis, cold agglutinins, and sensitivity to erythromycin base but was not proved. Acute hemorrhagic leukoencephalitis was demonstrated by brain biopsy 24 hours after admission. The patient recovered without lasting sequelae following reduction of increased intracranial pressure by mannitol, hyperventilation, and phenobarbital and prolonged immunosuppression by plasmapheresis, steroids, and cyclophosphamide.

Cerebral Hemorrhage↗
PubMedDate not supplied

Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis.

A case of biopsy-proven acute hemorrhagic leukoencephalitis is reported. The early computed tomography scans showed extensive bilateral hypodensities with mass effects and foci of microhemorrhages. Bilateral asymmetric hyperintensities in the mesiotemporal and frontal lobes and massive edema were found on T2-weighted and fluid-attenuated inversion recovery magnetic resonance images in a pattern classic for herpes simplex encephalitis. This fulminant demyelinating disease progresses to coma and death within days. Early diagnosis with neuroimaging studies and rapid correlation with the clinical findings of this disease are vital for the institution of potentially lifesaving treatments.

Adult↗
PubMedDate not supplied

Acute hemorrhagic leukoencephalitis.

The clinical and pathological findings of a ten-year-old girl, diagnosed as having acute hemorrhagic leukoencephalitis at postmortem examination, are presented. The diagnosis of this disease is usually established at autopsy. In this paper, emphasis is given to the diagnosis of acute hemorrhagic leukoencephalitis, and the possibility of its clinical recognition is discussed.

Acute Disease↗