Detection of an autoantibody from Pug dogs with necrotizing encephalitis (Pug dog encephalitis). (1/26)

An autoantibody against canine brain tissue was detected in the cerebrospinal fluid (CSF) and serum of two Pug dogs (Nos. 1 and 2) by indirect immunofluorescence assay (IFA). Dog No. 1, a 2-year-old male, exhibited severe depression, ataxia, and generalized seizures and died 2 months after the onset of symptoms. Dog No. 2, a 9-month-old male, exhibited severe generalized seizures and died 17 months after the onset of symptoms. Histopathologic examination revealed a moderate to severe multifocal accumulation of lymphocytes, plasma cells, and a few neutrophils in both the gray and white matter of the cerebrum in dog No. 1. In dog No. 2, the cellular infiltrates were mild, but there was a severe, diffuse, and multifocal necrosis in the cerebral cortex with prominent astrocytosis. With the aid of IFA using fluorescein isothiocyanate-labeled antidog IgG goat serum and a confocal imaging system, specific reactions for glial cells were detected in the CSF of these Pug dogs but not in six canine control CSF samples. Double-labeling IFA using CSF from these Pug dogs and a rabbit antiserum against glial fibrillary acidic protein (GFAP) revealed that the autoantibody recognized GFAP-positive astrocytes and their cytoplasmic projections. By immunoblot analysis, the autoantibody from CSF of these Pug dogs recognized two common positive bands at 58 and 54 kd, which corresponded to the molecular mass of human GFAP. The role of this autoantibody for astrocytes is not yet clear. However, if the presence of the autoantibody is a specific feature of Pug dog encephalitis, it will be a useful clinical diagnostic marker and a key to the pathogenesis of this unique canine neurologic disease.  (+info)

Neuropsychology: music of the hemispheres. (2/26)

Music may be the food of love but it is also good fodder for cognitive scientists. Here we highlight a recent study of a neuropsychological patient who has lost her ability to read music, but not text, in the absence of any other musical deficit.  (+info)

Two episodes of leukoencephalitis associated with recombinant hepatitis B vaccination in a single patient. (3/26)

Cases of central nervous system demyelination have been reported after recombinant hepatitis B vaccination, but no causal link has been clearly demonstrated. We present the first case report involving the occurrence of 2 episodes of leukoencephalitis in a previously healthy patient after vaccination and rechallenge with hepatitis B vaccine.  (+info)

Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review. (4/26)

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.  (+info)

Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report. (5/26)

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.  (+info)

Acute necrotizing encephalopathy: diffusion MR imaging and localized proton MR spectroscopic findings in two infants. (6/26)

In this report, we describe the findings of diffusion MR imaging and proton MR spectroscopy in two infants with acute necrotizing encephalopathy in which there was characteristic symmetrical involvement of the thalami. Diffusion MR images of the lesions showed that the observed apparent diffusion coefficient (ADC) decrease was more prominent in the first patient, who had more severe brain damage and a poorer clinical outcome, than in the second. Proton MR spectroscopy detected an increase in the glutamate/glutamine complex and mobile lipids in the first case but only a small increase of lactate in the second. Diffusion MR imaging and proton MR spectroscopy may provide useful information not only for diagnosis but also for estimating the severity and clinical outcome of acute necrotizing encephalopathy.  (+info)

Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report. (7/26)

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.  (+info)

Diffusion-weighted MR imaging findings of acute necrotizing encephalopathy. (8/26)

Multiple, symmetrical brain lesions affecting the bilateral thalami and cerebral white matter, which often show a concentric structure on CT and MR images, characterize acute necrotizing encephalopathy (ANE) of childhood. We describe the imaging findings of a 2-year-old child with ANE obtained with diffusion-weighted MR imaging. We discuss the significance of these findings, as well as the pathophysiology of ANE lesions, with reference to the appearance of the disease as revealed by diffusion-weighted MR imaging.  (+info)

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