An Autopsied Case of Acute Hemorrhagic Leukoencephalitis

Abstract: 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 microscopywere 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.     

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.     

Neuropathology of two fatal cases of measles in the 1988-1989 Houston epidemic

The clinical course and autopsy findings of 2 patients with measles encephalitis that occurred during the 1988-1989 Houston epidemic are reported. A previously healthy 25-month-old boy had serologically-proved measles, hemophagocytic syndrome, and acute disseminated demyelinating encephalitis. A 19-year-old male with acute lymphocytic leukemia had proved measles pneumonia and acute hemorrhagic leukoencephalitis. These patients represent a broad spectrum of measles-induced immunopathic complications of the central nervous system.     

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.     

Neuroimaging in postinfectious demyelination and nutritional disorders of the central nervous system

The myelin sheath and oligodendrocytes in the brain may be damaged by autoimmune-mediated inflammatory processes secondary to postinfectious demyelination or nutritional and vitamin deficiency. This article describes acute disseminated encephalomyelitis, acute hemorrhagic leukoencephalitis, acute necrotizing encephalopathy, and tumefactive demyelination as well as osmotic demyelination, Wernicke encephalopathy, Marchiafava-Bignami disease, and subacute combined degeneration of the spinal cord. Although some characteristic MR imaging features allow radiologists to suggest a diagnosis, these may overlap, and images should be interpreted in light of clinical symptoms and laboratory investigations.     

Postinfectious leukoencephalitis complicating Mycoplasma pneumoniae infection

Neuropathological findings in a patient with fatal neurological complications due to infection with Mycoplasma pneumoniae were similar to those seen in postinfectious encephalitis and acute hemorrhagic leukoencephalitis. This case supports the hypothesis that immune mechanisms play a role in the pathogenesis of neurological symptoms during mycoplasmal infections.     

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.     

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.     

Value of susceptibility-weighted imaging in acute hemorrhagic leukoencephalitis

Acute hemorrhagic leukoencephalitis (AHL) is a rapidly progressive disease in the spectrum of acute disseminated encephalomyelitis. Timely accurate diagnosis is crucial but challenging clinically and radiologically. However, imaging findings of AHL are quite specific when susceptibility-weighted imaging is utilized. The purpose of this report is to present the imaging findings of autopsy-proven AHL and thus to facilitate rapid recognition and treatment.     

Computerized tomography as a diagnostic aid in acute hemorrhagic leukoencephalitis

Computerized tomography (CT) in a pathologically proven case of acute hemorrhagic leukoencephalitis (AHL) showed a mass effect and increased absorption coefficient in the right hemisphere within 18 hours of the onset of neurological symptoms. The changes corresponded to the site of white matter edema, necrosis, and petechial hemorrhages demonstrated postmortem. The early changes of CT reflect the hyperacute nature of AHL and differ from those of herpes simplex encephalitis.     

Migraine prevalence in eating disorders and pathophysiological correlations

Acute hemorrhagic leukoencephalitis (AHL) is a rare demyelinating disease mainly affecting children, characterized by acute onset, progressive course and high mortality. A 62-year-old man was admitted to our Unit for diplopia and ataxia ensuing 2 weeks after the onset of pneumonia. MRI T2-weighted images showed signal hyperintensities in the brainstem. Antibodies against Mycoplasma Pneumoniae and cold agglutinins were found. Two weeks later the patient had a worsening of his conditions: he developed left hemiplegia with motor focal seizures and the day after he was deeply comatose (GCS = 4). A second MRI scan showed extensive hyperintensities involving the whole right hemisphere white matter with a small parietal hemorrhagic area. The clinical and neuroimaging features suggested the diagnosis of AHL, Aciclovir in association with steroid therapy were administered and then plasmapheresis was started. After 30 days of coma, the patient gradually reacquired consciousness and motor functions; anyway a left hemiplegia persisted.     

Atypical Acute Hemorrhagic Leukoencephalitis (Hurst’s disease) Presenting with Focal Hemorrhagic Brainstem Lesion

Background  

Acute hemorrhagic leukoencephalitis (AHL; Hurst’s disease) is a rare, severe, inflammatory CNS disease that is typically diffuse, multifocal and associated with petechial hemorrhage. The objective of this study is to report the clinical, radiologic, and pathologic findings in a fatal AHL case with focal brainstem involvement and gross hemorrhage.     

Cerebral phaeohyphomycosis caused by a dematiaceous scopulariopsis species

This report describes a rapidly fatal case of cerebral phaeohyphomycosis in a 33-year-old immunocompetent male. The infection presented as a single large lesion in the deep white matter of one temporal lobe, which was then removed surgically. Histologic features observed in the lobectomy specimen were characterized by perivascular sleeves of mononuclear cells accompanied by hemorrhages. These were reminiscent of acute hemorrhagic leukoencephalitis except for the presence of rare fungal organisms and sparse multinucleated giant cells similar to those occurring in AIDS. During the four days following surgery, a large focus of cerebritis with massive invasion of fungi developed in each centrum semiovale around the ventriculostomy sites. Fungal culture of the brain obtained at autopsy grew an organism consistent with a Scopulariopsis species.     

Fulminant form of acute disseminated encephalomyelitis: successful treatment with hypothermia.

We described herein a case of the fulminant form of acute disseminated encephalomyelitis (ADEM) that developed after mycoplasma pneumonia. A 28-year-old man who presented with fever, headache, and writing difficulty was admitted to our hospital in August 1997. He developed hernia on the 3rd hospital day. Surgical decompression and intravenous prednisolone failed to halt his progressive deterioration. We introduced systemic hypothermia and he has shown marked recovery; despite having Broca's type aphasia, he could comprehend spoken language and communicate with others by gesture. Head MRI demonstrated diffuse high signals over the white matter on fluid attenuated inversion recovery (FLAIR) images, which suggested extensive demyelination. The clinical course, imaging studies and presence of polymorphonuclear dominant leucocytosis in the blood and CSF in the patient are somewhat similar to findings in acute hemorrhagic leukoencephalitis, however, the result of a brain biopsy was inconclusive. The fulminant form of ADEM is usually fatal. Treatments such as corticosteroids, intravenous immunoglobulin, and surgical decompression have been performed to improve the prognosis. Our case results indicate that hypothermia, which suppresses both brain edema and immune response, may be included in the repertoire of treatment for the fulminant form of ADEM.     

Fulminant acute disseminated encephalomyelitis in children

Acute disseminated encephalomyelitis (ADEM) is a typically monophasic inflammatory demyelinating disease of the central nervous system with a favorable outcome. However, 2% of ADEM involves acute hemorrhagic leukoencephalitis (AHLE), which is a fulminant and hyperacute variant of ADEM with a poor outcome and high mortality. There are limited case reports of fulminant ADEM including AHLE in children. Herein, we report two pediatric cases of fulminant ADEM. Both cases had a rapid deterioration of consciousness, repetitive seizures, and brain edema on neuroimaging, in addition to atypical neuroradiological findings on magnetic resonance imaging (MRI), a reversible splenial lesion in case 1, and bilateral frontal and occipital cortical lesions in case 2. Both cases were treated with early high-dose methyl-prednisolone and immunoglobulin, while therapeutic hypothermia was also initiated in case 2 after the patient exhibited a decerebrate posture and irregular breathing pattern. Both cases had a favorable outcome. Further case reports on pediatric fulminant ADEM are required to clarify the various clinical types, and to examine the efficacy of various treatment modalities for fulminant ADEM and AHLE in children.     

Acute diffuse leukoencephalitis in HIV-1 infection.

The clinical, neuroradiological, and cerebrospinal fluid findings of a case with acute diffuse leukoencephalitis, a demyelinating disease associated with human immunodeficiency virus infection of the brain, is reported. The patient presented with acute tetraparesis as the primary manifestation of a previously symptom free HIV infection. Cerebrospinal fluid analysis showed enhanced inflammatory abnormalities with high concentrations of P24 antigen. MRI showed diffuse white matter hyper-intensities in both hemispheres. In the follow up over 22 months, the neurological deficits disappeared after antiretroviral treatment in good correlation with improvements in MRI as well as in inflammatory cerebrospinal fluid abnormalities.     

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 (Hursts 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 Hursts disease linked to EBV.     

Dengue: neuropathological findings in 5 fatal cases from Brazil

Neuropathological examination of 5 patients with dengue who died of shock in Rio de Janeiro during an outbreak in summer 1987, showed nonspecific lesions (edema, vascular congestion, hemorrhagic foci and perivascular lymphocytic infiltrates). In one case with delayed marked neurological symptoms, several foci of perivenous demyelination were observed. Neurological manifestations are various and not uncommon in dengue, but their anatomical substratum is not known. An immunopathological mechanism has been postulated in some cases but has never been demonstrated morphologically. The perivenous leukoencephalitis observed in one of our cases could represent the morphological substratum of such an immunological mechanism.     

影响急性脑梗死出血性转化的危险因素

目的 探讨急性脑梗死的出血性转化的危险因素。方法 收集2012年1月～2015年1月在湖北省恩施州利川市人民医院神经内科住院的急性脑梗死患者的临床及实验室检查资料,并在入院后10 d内行头颅CT复查,采用多变量logistic回归分析确定出血性转化的独立危险因素。结果 共纳入345例急性脑梗死患者,其中男205例,女140例,101例发生出血性转化。出血性转化组的年龄、脑梗死体积、脑卒中史或TIA史、高血压病、糖尿病、抗凝药和房颤的比例均显著高于非出血性转化组(P<0.05),而2组抗血小板聚集药、他汀类、高脂血症史、吸烟或饮酒史无明显差异(P>0.05)。多变量logistic回归分析显示年龄(OR=1.168,95%,CI=1.059～3.412; P=0.021)、梗死体积(OR=3.461,95%C1=1.317～6.270; P=0.044)和房颤(OR=1.284,95%C1= 1.117～2.903; P=0.015)为出血性转化的独立危险因素。结论 急性脑梗死患者出血性转化的发生率为29.3%,年龄、脑梗死体积和房颤为出血性转化的独立危险因素,绝大多数出血性转化不会加重临床症状,临床症状加重的患者主要是脑实质血肿型。