[Demyelinizing leukoencephalitis: Van Bogaert's subacute sclerosing leukoencephalitis].
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Encephalitis↗
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[Virogenic leukoencephalitis of man. On the etiology and pathogenesis of van Bogaert's subacute sclerosing leukoencephalitis].
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Brain↗
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↗
Response to acyclovir in two cases of herpes zoster leukoencephalitis and review of the literature.
Herpes zoster leukoencephalitis is a rare complication of varicella-zoster virus infection. Associated with high mortality, the majority of cases have been discovered postmortem; today, however, magnetic resonance imaging is being used successfully as an aid in the diagnosis of this disease. The first two reported cases of HIV-infected patients with herpes zoster leukoencephalitis who recovered clinically and showed complete resolution of the magnetic resonance demyelination images after acyclovir treatment are described. In addition, the cases of herpes zoster leukoencephalitis reported in the literature to date are reviewed.
AIDS-Related Opportunistic Infections↗
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↗
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↗
Posterior leukoencephalopathy in a girl with acute haemorrhagic leukoencephalitis.
In children, posterior leukoencephalopathy is frequently associated with hypertensive encephalopathy, anticancer chemotherapy, treatment with immunosuppressive drugs in patients with organ transplantation, transfusion or human immunodeficiency virus infection. Posterior leukoencephalopathy in these children appears as a complicating illness and resolves once precipitating factor (e.g. cancer chemotherapy) is removed. Here we are reporting a fatal case of acute haemorrhatic leukoencephalitis in a 13 year old girl, imaging abnormalities are also suggestive of posterior leukoencephalopathy. Posterior leukoencephalopathy in our patient possibly, is a part of post-infectious haemorrhagic leukoencephalitis, rather than because of ischaemia or cerebral oedema secondary either to abrupt increase in blood pressure or following administration of immunosuppressive drugs.
Adolescent↗
[Weston Hurst acute haemorrhagic leukoencephalitis. Neuropathological study of one case].
INTRODUCTION: 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. CASE REPORT: The patient was a 21 year old female who presented with an acute encephalopathy and generalized seizures following a 15 day febrile syndrome attributed to amygdalitis; a laboratory work up, including CSF, was non diagnostic, and a brain CT scan revealed diffuse cerebral edema. After 12 days the patient died from nosocomial pneumonia and multi organ failure; neuropathological examination of the brain confirmed the diagnosis of Hurst acute hemorrhagic leukoencephalitis, with a weak perilesional inflammatory reaction, unlike the usual picture in AHL. DISCUSSION: AHL should be a part of the differential diagnosis of acute encephalopathic diseases, particularly if preceded by systemic infections. The atypical laboratory findings, and the impossibilty of performing a brain MRI were obstacles to the diagnosis in this case. The relative paucity of the perivascular infiltrate is an atypical finding, and could be due to apoptotic clearance of the inflammatory cells, as has been described in other autoimmune demyelinating diseases.
Adult↗
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↗
[Acute Weston Hurst necrotizing hemorrhagic leukoencephalitis].
The clinical and pathological findings of a 43-year-old woman, diagnosed as having acute hemorrhagic leukoencephalitis at postmortem examination, are presented. The acute hemorrhagic leukoencephalitis affects mainly young adults and is the most fulminant from of demyelinating disease. It is frequently preceded by a respiratory infection. Diagnosis is facilitated by CT scanning and MRI, which reveal the massive lesion in the cerebral white matter. Many cases terminate fatally in 2 or 4 days, but in others survival is longer. The pathological findings are distinctive.
Acute Disease↗
[Acute hemorrhagic leukoencephalitis in a woman with Goodpasture syndrome].
A case of acute leukoencephalitis in a 17-year-old female with Goodpasture syndrome is described. Acute leukoencephalitis is a rare inflammatory-degenerative disease (practically not studied so far) affecting the brain white matter. The disease develops acutely as a rule after respiratory viral infection and has a fatal exitus. Multiple hemorrhages in the brain white matter with demyelinization and axons death are observed histologically.
Adolescent↗
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↗
Ependymitis, leukoencephalitis, hydrocephalus, and thrombotic vasculitis following chronic infection by mouse hepatitis virus 3 (MHV 3).
Mouse hepatitis virus 3 (MHV 3) is either avirulent (resistant mice), hepatotropic (susceptible mice), or neurotropic (semisusceptible mice), depending on the strain of mice infected. In semisusceptible mice, infection led first to a transient meningitis, ependymitis, and leukoencephalitis, followed by a permanent communicating hydrocephalus and, later on, to a chronic thrombotic vasculitis affecting meningeal and parenchymal vessels at the brain stem level. Small foci of ischemic necrosis related to vascular occlusions were seen in the dorsal brain stem. Cyclophosphamide treatment of semisusceptible mice significantly reduced the meningeal infiltrates but did not prevent the development of hydrocephalus and other neuropathologic changes. Identical lesions occurred in fully susceptible mice infected with a low dose of virus, but no neurologic disorder could be induced in genetically resistant mice even following immunosuppression or intracranial inoculation. The leukoencephalitis differed from the demyelinating lesions observed with MHV 4. Vascular lesions were of particular interest. More attention should be given to the possibility of virus induced chronic cerebral vasculitis in man.
Animals↗
Up-regulation of mRNA for matrix metalloproteinases-9 and -14 in advanced lesions of demyelinating canine distemper leukoencephalitis.
Matrix metalloproteinases (MMPs) comprise a family of proteolytic zinc- and calcium-dependent enzymes that are capable of disrupting the blood-brain barrier and mediating the destruction of extracellular matrix and myelin components. MMPs are also involved in facilitating leukocyte migration into inflammatory sites of the central nervous system. To determine the cellular localization and the amount of mRNA for MMP-9, MMP-14 and a tissue inhibitor of metalloproteinases (TIMP-1) in dogs with spontaneous demyelinating distemper encephalitis, formalin-fixed paraffin-embedded cerebella were investigated by in situ hybridization using specific digoxigenin-labeled RNA probes. Additionally, immunohistochemistry was performed to characterize the different types of plaques of demyelinating leukoencephalitis. Furthermore, virus antigen and mRNA were detected by immunohistochemistry and in situ hybridization. Healthy control dogs revealed a weak signal for mRNA for MMP-9, MMP-14, and TIMP-1 in various numbers of neurons, astrocytes, microglial cells and oligodendrocytes. In the cerebella of dogs with distemper, a strong increase of both number and staining intensity of MMP-9, MMP-14, and TIMP-1 mRNA-expressing cells, mainly in subacute inflammatory lesions and chronic plaques, was observed. The number of cells expressing mRNA for MMP-9 and MMP-14 increased about two- to threefold compared to TIMP-1 mRNA-expressing cells, whereas staining intensity of individual cells was similar. In early lesions, especially astrocytes and activated macrophages/microglial cells displayed a positive signal for MMPs and TIMP-1, whereas in older lesions activated microglia/macrophages and infiltrating lymphocytes represented the main source for MMP-9, MMP-14, and TIMP-1 mRNA synthesis as revealed by double-labeling techniques. In summary, the proportionally higher increase of MMP mRNA-expressing cells might indicate an MMP/TIMP imbalance as a cause for lesion initiation and progression in demyelinating canine distemper leukoencephalitis.
Animals↗
MRI in varicella-zoster virus leukoencephalitis in the immunocompromised host.
Immunocompromised patients are at increased risk for CNS and disseminated varicella zoster virus (VZV) infection. In this report we present the MR findings in a leukemic patient with active, biopsy-proven VZV leukoencephalitis. The characteristic MR features of this infection were clustered subcortical plaque-like lesions demonstrating rapid demyelination. Active lesions enhanced with intravenous contrast medium administration. Edema and hemorrhage were not prominent early findings but developed as the infection evolved. These findings were strikingly similar to those reported in prior autopsy studies of immunocompromised patients with VZV leukoencephalitis.
Adolescent↗
Subacute sclerosing leukoencephalitis: ultrastructure of intranuclear and intracytoplasmic inclusions.
Intranuclear inclusions found in glial cells of two cases of subacute sclerosing leukoencephalitis have been examined in the electron microscope. The inclusions are composed of filamentous tubules 170 to 230 angstroms in diameter, which bear a general resemblance to the intranuclear structures observed in cells infected with measles virus. This finding provides further suggestive evidence that a virus may be involved in the pathogenesis of subacute sclerosing leukoencephalitis.
Cerebellum↗
[Progressive multifocal leukoencephalitis complicating polymyositis].
INTRODUCTION: Progressive multifocal leukoencephalitis (PML) must be evoked in patients presenting with a systemic disease during which multiple neurological deficiencies rapidly worsen. OBSERVATION: A 17 year-old girl suffering from histologically confirmed polymyositis was treated with corticosteroids. Two years after the diagnosis she exhibited global signs of cerebral damage with fever and magnetic resonance imaging evoked leukoencephalitis. COMMENTS: An affection of the central nervous system, PML is characterised by the existence of multiple areas of demyelination in the hemispheric white substance of the cerebral trunk and sometimes the cerebellum, whereas the grey substance is usually spared. This entity occurs more frequently in HIV-infected patients, but also in patients in whom the immunodeficiency may have other causes, such as the treatment for a systemic disease for example.
Adolescent↗
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↗