Haemorrhagic and perivenous encephalitis: a clinical-pathological review of 38 cases.

Abstract

Acute haemorrhagic leucoencephalitis (AHL) and post-infectious perivenous encephalitis (PVE) associated with childhood mumps, measles, chickenpox, and vaccination are important diseases of the central nervous system for two reasons. First, encephalitis and PVE associated with these illnesses are numerically important.They account for over 15% of childhood cases of encephalitis reported annually in the United States (Polk, 1971). Second, PVE and AHL both closely resemble experimental allergic encephalitis (EAE), which is considered to be an excellent model for the study of presumed immunological disease of the central nervous system (Levine, 1971). Thus, if EAE is to be an experimental model of PVE and AHL, then the pathology of both these diseases should be clear. Although the pathology of AHL has been well defined in the past (Hurst, 1941;Adams et al., 1949), PVE has generally been considered to be a demyelinating disease (Greenfield and Norman, 1963;, and this categorization has masked appreciation of the actual diverse micropathological findings associated with the (Accepted 22 January 1975.) disease. With the exception of the treatise by Carpenter and Lampert (1972), there are no recent descriptions of the variable pathology of PVE which must be accounted for by any causal theory of AHL and PVE.For these reasons we present the previously unreported' clinical and pathological features of 30 cases of PVE and eight cases of AHL from the files of the Armed Forces Institute of Pathology and the University of Missouri Medical Center. METHODSNecropsy cases of PVE and AHL were selected which had good clinical documentation of the primary viral disease, clinical nervous system (CNS) symptoms, and sufficient pathological material (at least five blocks from various areas) for special stains. The PVE cases included 17 cases of rubeola, four of rubella, six of mumps, two of varicella, and one post-vaccination case. Special stains for myelin, axons, and glia were used on most of the cases in which these stains had not already been performed.

Hemorrhage in Posterior Reversible Encephalopathy Syndrome: Imaging and Clinical Features

“…65,66 Histologically, ADEM demonstrates a predominantly lymphocytic/macrophage perivascular immune cell reaction and infiltrate, characteristic sleeve-like demyelination, and occasional minute perivascular hemorrhage. 62,67,68 In contrast, AHL typically demonstrates a prominent perivascular neutrophilic infiltrate, with accompanying necrotizing vasculitis and more extensive petechial hemorrhages, with a characteristic perivascular ball or ring appearance. [67][68][69][70] MR imaging in ADEM has demonstrated lesions in the thalami, basal ganglia, and brain stem in addition to typical periventricular and hemispheric white matter involvement.…”

“…62,67,68 In contrast, AHL typically demonstrates a prominent perivascular neutrophilic infiltrate, with accompanying necrotizing vasculitis and more extensive petechial hemorrhages, with a characteristic perivascular ball or ring appearance. [67][68][69][70] MR imaging in ADEM has demonstrated lesions in the thalami, basal ganglia, and brain stem in addition to typical periventricular and hemispheric white matter involvement. [63][64][65] In patients with AHL, MR imaging has demonstrated both minute focal hemorrhages and larger focal hematomas in the hemispheres, frontal lobes, deep white matter, and thalami.…”

Pathology of acute disseminated encephalomyelitis

“…At autopsy, edema is often the most conspicuous finding or in contrast to MS, the lesions in ADEM may not be visible macroscopically [3,4]. The first, and maybe the most well characterized pathological difference between ADEM and MS is that MS lesions are characterized by a sharp-edged plaque, that has never been seen in ADEM which is characterized with margins that are rather indistinct [5].…”

“…The first, and maybe the most well characterized pathological difference between ADEM and MS is that MS lesions are characterized by a sharp-edged plaque, that has never been seen in ADEM which is characterized with margins that are rather indistinct [5]. Second characteristic of ADEM are 'sleeves' of demyelination ( Figure 1) that surround venules and are associated with significant inflammatory infiltrates dominated by T lymphocytes and macrophages ( Figure 2a) [3]. Rarely, granulocytes or plasma cells are seen [6].…”

Possible Acute Hemorrhagic Leukoencephalitis Manifesting as Intracerebral Hemorrhage on Computed Tomography. Case Report.

“…1,2,7,10) Therefore, early diagnosis and aggressive treatment are extremely important. [3][4][5][6][7]9) AHL is often preceded by an upper respiratory infection or several viral diseases and autoimmune mechanisms may be involved in the pathogenesis.…”