Treatment leading to dramatic recovery in acute hemorrhagic leukoencephalitis

The clinical course, neuroimaging, neuropathological features, and management of a 7-year-old girl with acute hemorrhagic leukoencephalitis are reported. Treatment with subtotal bifrontal craniectomies for symptoms of imminent uncal herniation followed by high-dose corticosteroid therapy was associated with dramatic clinical recovery. This report highlights the utility of aggressive intervention in acute hemorrhagic leukoencephalitis, an otherwise fatal disorder.     

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.     

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.

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.     

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.     

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.     

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.     

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.     

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.     

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.     

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.     

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.     

Pathogenetic and therapeutic aspects of subacute sclerosing encephalitis (author's transl)]

This paper reports the case of a 20-year-old patient with subacute sclerosing leukoencephalitis who in spite of the severe illness was delivered of a healthy child be caesarean section. This child grew normally and showed no signs of disease of the CNS. The patient herself was treated immunsuppressively and the illness then reached a stationary state. The pathogenesis of subacute sclerosing leukoencephalitis, similarities of laboratory findings to multiple sclerosis, and problems of treatment are discussed.     

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.     

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.     

Multifocal progressive leukoencephalitis in a patient given fludarabine for chronic lymphoid leukemia

A 74-year- man was hospitalized for subacute aphasia and right hemiparesis. He had had chronic lymphoid leukemia for 11 years and had been treated 5 months earlier with 3 courses of fludarabine. Magnetic resonance imaging showed lesions of the temporo-occipital white matter compatible with progressive multifocal leukoencephalitis (PML). The presence of JC virus was demonstrated first by in situ hybridation after a brain biopsy and second with polymerase chain reaction on CSF. The diagnosis of PML was confirmed. The causality of fludarabine treatment is discussed.