Magnetic resonance imaging abnormalities with septic encephalopathy.

A 48 year old woman, status post renal transplantation six years earlier, died after a two week illness characterised by fever, recurrent seizures, and coma. Widespread abnormalities were seen on neuroimaging. A diagnosis of septic encephalopathy was established on postmortem. We describe the magnetic resonance imaging findings of bilateral basal ganglia, thalamic, cerebellar, brainstem, and cerebral abnormalities in this patient, which correlate with the pathophysiology of septic encephalopathy.

Myelodysplastic syndrome and sagittal sinus thrombosis.

After a 5-day illness beginning with severe headache, a 46-year-old man with myelodysplastic syndrome died of complications from cerebral venous thrombosis. Considering the underlying hematologic abnormality, multiple therapies, and potential for leukemic transformation, we suggest that patients with myelodysplastic syndrome may be at increased risk for developing cerebral venous thrombosis.

Idiopathic granulomatous angiitis of the CNS manifesting as diffuse white matter disease.

A 48-year-old man presented with progressive spastic paraparesis and diffuse white matter involvement on neuroimaging that suggested a primary demyelinating disease. Brain biopsy 3 years after onset of symptoms demonstrated idiopathic granulomatous angiitis. In patients with MRI features of diffuse white matter disease of uncertain etiology, open brain biopsy of leptomeninges and parenchyma should be considered to exclude idiopathic granulomatous angiitis of the CNS.

New syndrome of hydrocephalus, endocardial fibroelastosis, and cataracts (HEC syndrome).

We report on two unrelated male infants with similar findings of communicating hydrocephalus, endocardial fibroelastosis (EFE) and congenital cataracts, who died at 4 months of age. Both mothers reported an upper respiratory infection during the first trimester of pregnancy which was further complicated by polyhydramnios in the third trimester. The infants were diagnosed with bilateral congenital nuclear cataracts at birth. Serologic tests for toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, syphilis, and galactosemia screens were negative. Chromosome analyses were normal. Both children developed communicating hydrocephalus between one and three months after birth. Patient 1 died suddenly at 4 months following an upper respiratory infection. Patient 2 developed congestive heart failure and also died at 4 months. At autopsy, both infants had enlarged hearts with endocardial fibroelastosis. No identifiable organism could be isolated. We discuss the association of birth defects in widely separated organ systems in these patients and suggest that this may represent a genetic syndrome; however, a viral etiology cannot entirely be excluded. We believe this is a distinct disorder and propose the acronym HEC for hydrocephalus, EFE and cataracts.

Applications of lipid-coated microbubble ultrasonic contrast to tumor therapy.

Lipid-coated microbubbles (LCM) make an excellent diagnostic ultrasonic contrast agent in experimental tumor systems. LCM have been shown to aggregate in brain tumors and subcutaneous tumors after intravenous administration, and to provide persistent image enhancement for many minutes. In this work, experimental subcutaneous Walker Carcinosarcoma is insonated after the bubbles are given intravenously. Selective necrosis, lymphocyte proliferation and hemorrhage within the tumor can be demonstrated. Preliminary data are given to demonstrate this phenomenon. The mechanism of the effect is discussed in the context of both heating and cavitation.

Adrenoneuropathy: characteristic gross findings and association with Hashimoto's thyroiditis and accelerated atherosclerosis.

New understanding in the pathogenesis of adrenoneuropathy (ANP), formerly called adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AML), has led to greater awareness of the disease with resultant increase in the number of new cases being diagnosed. We report adrenoneuropathy in two siblings, one of whom had associated affective symptoms and died undiagnosed in prison. The other sibling died at the age of eight with severe cerebral demyelination. Affective symptoms have been described in ANP, but the association of Hashimoto's thyroiditis and accelerated atherosclerosis in this case is unique. We would like to stress the characteristic gross appearance of the adrenals in ANP that may help in identifying undiagnosed cases of adrenoneuropathy in surgical and autopsy specimens. Genetic counseling and family-pedigree analysis with diet restriction and diet therapy will be invaluable in such cases.

Case reports Rhizopus brain abscess: report of a case and review of the literature.

The case of a 34-year-old intravenous drug abuser who survived brain abscess due to Rhizopus arrhizus is presented. The diagnosis was made by brain biopsy. Fungal brain abscess is a rare complication of IV drug abuse. Phycomycetes cause venous thrombosis and infarction of brain tissue. Medical versus surgical options are discussed.

Meningioma: the role of a foreign body and irradiation in tumor formation.

A case of meningioma is reported. At the age of 18 years, the patient had undergone insertion of a Torkildsen shunt through a posteroparietal burr hole for obstructive hydrocephalus secondary to a tumor of the pineal region, of which no biopsy had been made. After the hydrocephalus was relieved, he underwent irradiation of the tumor. Thirty years later, he was treated for an intracranial meningioma wrapped around the shunt. The tumor followed the shunt in all of its intracranial course. Microscopy disclosed pieces of the shunt tube within the meningioma. The role of a foreign body and irradiation in the induction of meningiomas is discussed, and a comprehensive review of the literature is presented.

Aqueductal (pencil) glioma presenting as neurogenic pulmonary edema: a case report.

A case of neurogenic pulmonary edema due to hydrocephalus, without initial neurological deficit, is described. Computed tomography demonstrated a ring enhancing lesion in the tectum of the mesencephalon obstructing the aqueduct of Sylvius. The lesion, on autopsy, was a rare mesencephalic glioma described in the literature as a "pencil glioma" of the aqueduct.

Magnetic resonance imaging of symptomatic Rathke's cleft cyst: report of a case.

A case of symptomatic Rathke's cleft cyst is described. In spite of the intrasellar component and radiological and clinical evidence of involvement of the pituitary gland, the sella turcica was minimally involved. MRI afforded optimal visualization and localization of the intra- and suprasellar components vis-à-vis the pituitary gland, optic chiasm, infundibular stalk, and carotid arteries.

Directional effects of acute total-body x-irradiation: exposure rate versus homogeneity of exposure.

Survival patterns of NBL/Up mice exposed to 675-800 R of total-body x-radiation depended on direction and rate of exposure. Homogeneous dual exposures administered at 126 R/minute were more effective than those administered at 63 R/minute. Dorsal and dual exposures at 63 R/minute were equally effective, while absorption reduced the effectiveness of ventral exposures. Since cellular repair of hematopoietic tissue was initiated only during exposure, increased homogeneity without increased exposure rate would not increase effectiveness. With rate, direction, and homogeneity influencing the effectiveness of total-body exposures, fallacies inherent in converting total-body exposures to absorbed tissue dose became apparent.

Patterns of epithelial metaplasia in malignant gliomas. II. Squamous differentiation of epithelial-like formations in gliosarcomas and glioblastomas.

Six cases are reported (four gliosarcomas and two glioblastomas) in which the epithelial-like areas of glial anaplasia showed focal squamous cell differentiation, characterized by the development of epithelial whorls, keratin pearls and immunopositivity for cytokeratin. The expression of glial fibrillary acidic protein and the development of squamous metaplasia usually were mutually exclusive. Autopsy findings in two patients and clinical work-up in five failed to disclose a primary extraneural malignancy. It is suggested that squamous differentiation may represent an extreme form of epithelial metaplasia in a malignant glioma. This possibility should be kept in mind in the diagnostic evaluation of such cases, especially in view of the current emphasis on the immunomorphologic demonstration of intermediate filament tumor markers.

The association of progressive multifocal leukoencephalopathy and sarcoidosis.

A 59-year-old woman had a right homonymous hemianopsia, memory impairment for five months, a nonenhancing area in the left parieto-occipital region on CT scan, and bilateral reticulonodular infiltrates on chest x-ray film. Lung biopsy findings were consistent with sarcoidosis, a clinical diagnosis of CNS sarcoidosis made, and prednisone therapy begun. She deteriorated neurologically and died. At autopsy characteristic histologic and electron microscopic features of progressive multifocal leukoencephalopathy (PML) were found. We conclude that this and other cases demonstrate an association of PML and sarcoidosis and that steroid treatment is not a precondition. We also suggest an aggressive diagnostic approach in evaluating sarcoidosis with atypical neurologic deficits.

Foster-Kennedy syndrome caused by solitary intracranial plasmacytoma.

The first case of typical Foster-Kennedy syndrome, including decreased olfaction of the side of the optic atrophy due to a primary solitary intracranial extraskeletal plasmacytoma, is reported. Its appearance on a computed tomography scan was compatible with a meningioma or subdural hematoma. The patient demonstrated a chronic, corticosteroid-sensitive, relapsing optic neuropathy associated with an elevated sedimentation rate and a monoclonal gammopathy. The diagnosis, treatment, and prognosis of such tumors are reviewed.

Hybrid-versus-parental strain reaction. III. Comparative mortality in parental strain mice of the H-2k genotype.

Lethally irradiated parental strain male and female mice were inoculated with marrow from reciprocal hybrid donors of the same sex. Hybrid cells homozygous for the H-2k genotype could recognize the quantitatively greater antigenicity of the parental strain CBA male recipients only. The more severe reaction occurred when the maternal strain of the hybrid donor was allogeneic to the recipient, which indicates the maternal effect on the antigen recognition system. This demonstration of a maternal influence on the expression of paternally derived tissue antigens made it possible to attribute the secondary disease syndrome to a hybrid-versus-parental strain reaction. The apparent differences in mortality between male and female recipients could not be attributable to X or Y chromosome disparity without additional transfers of appropriate marrow from males to females and females to males. In all cases, the parental strain-versus-hybrid reaction resulted in greater mortality than that observed in the hybrid-versus-parental strain reaction.