Sturge-Weber-Dimitri disease? In association with an astrocytoma.

A patient with parieto-occipital cortical calcification characteristic of Sturge-Weber-Dimitri disease (SWDD) is of interest in that she lacked the facial portwine lesion and almost all other features of the disease. She subsequently developed an astrocytoma in the underlying white matter. Although the absence of the facial lesion in SWDD has previously been described, there has been no report of a glioma developing in such a patient. The association of SWDD and astrocytoma in this case most likely has been fortuitous.

Subarachnoid epithelial cyst of the cerebellum. Immunohistochemical and ultrastructural studies.

This report describes the immunohistochemical and ultrastructural studies of a subarachnoid epithelial cyst of the cerebellum found incidentally at autopsy of a 76-year-old man. The cyst was composed of an inner epithelial layer, a middle connective tissue layer and an outer arachnoid membrane. The epithelial layer was strongly positive for cytokeratin, carcinoembryonic antigen and epithelial membrane antigen, but negative for glial fibrillary acidic protein, S-100 protein, neuron-specific enolase and vimentin. Ultrastructurally, the epithelial layer had four distinct types of cells: ciliated cells, non-ciliated cells with coated microvilli, basal cells with tonofilaments and desmosomes, and cells with dense-core secretory granules (Kulchitsky cells). The latter two types of cells have not been described previously in epithelial cysts of the CNS. The lining epithelium closely resembled the upper respiratory epithelium. The findings suggest that the cyst was of endodermal rather than neuroectodermal origin.

Meningioma in the parotid region.

Meningiomas occurring ectopically in the neck are exceptional. We report two patients who presented as parotid and parapharyngeal masses and analyze them along with 27 previously reported cases. Typical patients present with parotid or parapharyngeal masses and cranial nerve palsies. Meningioma is not considered in differential diagnosis. The tumor is an extension of an unsuspected intracranial mass, occasionally with associated multiple meningiomas or other neurogenic tumors. Ectopic meningiomas should be considered in patients with parotid and parapharyngeal masses, particularly those with cranial nerve deficits; jugular foramen syndrome is most characteristic. Associated occult intracranial and temporal bone tumors and the cranial form of neurofibromatosis should be suspected. Treatment of cervical meningiomas is excision. The most important aspect of intracranial-extracranial lesions is recognition and treatment of the intracranial portion.

A glioependymal cyst of the cerebellopontine angle. Immunohistochemical and ultrastructural studies.

Epithelial cysts in the subarachnoid space are infrequently reported and appear to be histogenetically heterogenous. This report describes the gross, immunohistochemical and ultrastructural findings of an asymptomatic isolated 3-cm epithelial cyst in the cerebellopontine angle. The cyst wall was composed of an inner glial layer with a luminal ependymal lining and an outer fibrous layer with no external lining cells. The lining ependymal cells had vacuoles, bleb-like protrusions, normal and abnormal cilia, and microvilli, the later were sometimes distended and detached into the lumen. They possessed neither pinocytic vesicles nor a basement membrane. The glial layer contained astrocytes, glial bundles and ependymal cells, often in pairs and forming intercellular lumina with cilia and microvilli. Some single and paired ependymal cells contained abnormal cilia and intracytoplasmic lumina. The blood vessels within the glial layer had elongated tight junctions and were fenestrated. The glial layer was demarcated from the outer fibrous layer by a continuous basement membrane. This cyst appears to have originated from a leptomeningeal neuroglial heterotopia and may represent a continued proliferative growth rather than degenerative change of the heterotopia.

Angiographic features of gliosarcoma.

Gliosarcoma is a brain neoplasm that is being recognized with increasing frequency. We discuss the radiographic findings in 14 pathologically proven cases. At angiography in nine cases, four showed mixed dural and pial vascular supply to the lesion. Early cortical venous drainage, irregular tumor vessels, and a prominent vascular stain with well-defined tumor margins were seen in the majority of cases. CT showed an irregular enhancing rim surrounding a necrotic center in 14 cases. Most lesions were peripherally located and invaded dura.

Cerebral Paecilomyces javanicus infection. An ultrastructural study.

A 41-year-old diabetic woman developed Paecilomyces javanicus endocarditis and subsequent cerebral fungal embolism and vasculitis with massive infarction and hemorrhage 6 years after a mitral valve replacement with porcine heterograft. The organism was identified by the culture from the infected brain tissue and aortic valve. Cerebral infection due to fungus Paecilomyces is rare. This report documents the cerebral manifestations of fungus Paecilomyces javanicus and describes for the first time the ultrastructure of such an organism obtained from the infected tissue. Their ultrastructural findings, similar to those described in Paecilomyces farinosus derived from culture, include a double-layered cell wall, a triple unit plasma membrane, abundant cytoplasmic lipid droplets, vacuoles, membranous profiles and septal formation. This report further demonstrates hyphae with packed 50A filaments and granulofibrillary material and features suggestive of intrahyphal hyphae, not previously described in fungus Paecilomyces.

Neuropathologic findings in thanatophoric dysplasia.

Neuropathologic study of eight cases of thanatophoric dysplasia (dwarfism) reveals developmental abnormalities including hypoplasia of posterior fossa, megalencephaly, cerebral gyral disorganization, hippocampal malformation, neuronal heterotopia, nuclear dysplasia, and abnormal axonal bundles. There are no noticeable differences in CNS abnormalities between thanatophoric dysplasia with and without cloverleaf skull (Kleeblattschädel). The CNS abnormalities, likely the result of abnormal neuronal migration and cytoarchitectonic disarrangement, are apparently not caused by skeletal abnormalities. The observation suggests that CNS abnormalities represent a characteristic and distinct manifestation of thanatophoric dysplasia.

Alterations in coxsackievirus B4 heart muscle disease in ICR Swiss mice by anti-thymocyte serum.

Coxsackievirus B4 (CB4) infection in infant ICR Swiss mice induces synchronous peaks in both virus titres and pathologic changes in the heart. Among surviving mice, transmural necrosis is followed by fibrosis and ventricular aneurysm. Rabbit antimouse thymocyte serum (ATS) was given before CB4 infection to determine the effects of thymus-dependent functions upon the course of disease. The mortality in ATS-treated mice was 75.9% (65 of 83 mice died) compared to 21.3% (16 of 75 mice died) in normal rabbit serum-treated controls. Pathologic studies showed that ATS-treated mice had more extensive myofibre necrosis and subsequent mineralization, but during the first 10 days of infection, leukocytic infiltration was decreased. Splenic follicles were not present until the 17th day after infection in this treated group. Serum CB4-neutralizing antibodies were similar in mice from the group treated with ATS and normal rabbit serum. These findings indicate that ATS-suppressible functions contribute importantly to virus elimination, perhaps by an increase in macrophage phagocytosis of CB4.

A mouse model of transmural myocardial necrosis due to coxsackievirus B4: observations over 12 months.

The course of coxsackievirus B4 necrotizing myocarditis was studied over a 12-month period in 712 ICR Swiss mice inoculated at less than 48 h of age. Affected animals were sacrificed at intervals until 1 year. Microscopically, focal myocardial necrosis, which was often transmural in extent with mixed inflammatory exudate, and subsequent fibrous replacement were induced in 75% of the examined animals. The left ventricle (63 of 69 subjects, 91.3%), interventricular septum (39 of 69 subjects, 56.5%) and right ventricle (26 of 69 subjects, 37.7%) were most frequently involved. Thinning of the ventricular wall and grossly apparent localized ventricular bulges (aneurysms) were seen in 22 subjects (the left ventricle 16 times, the right ventricle 5 times, and the interventricular septum 5 times). Coronary arteries were normal in all instances.

Secondary intracranial subarachnoid hemorrhage due to spinal missile injury.

Fresh intracranial subarachnoid hemorrhage may occur secondary to blast-type injury of the spinal cord. This phenomenon is demonstrated in four cases of gunshot and shotgun wounds involving the spinal column. The significance of such a finding is that the subarachnoid hemorrhage should not be construed to represent an independent injury. Such an erroneous conclusion could jeopardize a theory of self-defense in a homicidal shooting.

Osteosarcoma of meninges: clinical, light, and ultrastructural observations of a case.

A primary osteosarcoma of the meninges based on clinical, light, and ultrastructural findings is reported. The neoplasm appears to complete Cushing and Eisenhardt's postulate and expectation of a malignant counterpart of osteoblastic meningioma. The neoplasm lends support to the concept of a primitive multipotential mesenchymal cell in the meninges capable of giving rise to every variety of neoplasms within that potential.

Pituitary carcinoma mimics the ectopic adrenocorticotropin syndrome.

A middle-aged man presented with weight loss, hypokalemic alkalosis, diabetes, hypertension, and generalized melanosis. Marked elevation of urinary free cortisol (655 micrograms/24 h) and plasma ACTH (2445 PG/ML) SUGGESTED THE DIAGNOSIS OF ECTOPIC ACTH syndrome. The plasma concentrations of cortisol and urinary 17-hydroxycorticosteroids increased paradoxically during the administration of dexamethasone without a corresponding change in the plasma ACTH level. Metyrapone administered over 24 h also markedly incrased both urinary free cortisol and 17-hydroxycorticosteroids. Selective venous sampling of plasma ACTH did not reveal a gradient between jugular vein and peripheral venous blood. The laboratory findings supported the diagnosis of ectopic ACTH syndrome. However, belated occurrence of visual changes necessitated surgical exploration, resulting in the diagnosis of pituitary carcinoma. A fluorescent antibody to ACTH reacted strongly with the atypical pituitary cells. This rare case documents that severe melanosis in Cushing's disease can occur without prior adrenalectomy and is consistent with the diagnosis of pituitary carcinoma. Furthermore, melanosis observed in patients with pituitary carcinoma is associated with ACTH levels similar to those occurrring in the ectopic ACTH or Nelson's syndrome.

Age-dependent pathogenicity of group B coxsackieviruses in Swiss-Webster mice: infectivity for myocardium and pancreas.

Coxsackieviruses B1-B4 were inoculated intraperitoneally into 48-hr-old, 14-day-old, and three- to five-month-old Swiss-Webster mice. Immediate death occurred only among mice less than 48 hr old, which died from fulminant encephalitis. Older mice usually survived. Myocarditis ensued in mice less than 48 hr old that were infected with coxsackieviruses B1 and B4. Several of the surviving mice developed left ventricular aneurysms, which resulted from transmural necrotizing myocarditis. In this group (coxsackieviruses B1 and B4), pathologic changes in the heart were synchronous with maximal cardiac titers of virus. Fourteen-day-old mice infected with coxsackieviruses B2 and B3 developed nontransmural necrotizing myocarditis in which maximal pathologic changes followed peak cardiac titers of virus by several days, whereas three- to five-month-old mice infected with coxsackieviruses B1, B2, B3, or B4 showed maximal susceptibility to destructive lesions in the exocrine glandular pancreas. Therefore, specific susceptibilities to infection with coxsackieviruses group B vary with age of the mouse, virus type (and strain), and organ.

Candida pachymeningitis with multiple cranial nerve pareses.

A 66-year-old woman complained of right-sided headache and was found to have progressive dysfunction of cranial nerves V and VIII through XII on the right side. At autopsy, there was a granulomatous pachymengitis involving the floor of the right middle and posterior cranial fossae due to Candida tropicalis infection. Inflammatory tissue compressed the clinically affected cranial nerves.

Adult form of subactute necrotizing encephalomyelopathy.

Subacute necrotizing encephalomyelopathy (SNE) usually appears before the age of 2 years. Variability in its clinical presentation make antemortem diagnosis difficult. The morphologic diagnosis depends on the present of necrotizing lesions with a characteristic distribution within the CNS. We describe a patient with the uncommon adult form of SNE. The pathologic changes are those accepted as diagnostic of the more common infantile SNE, and permit a separation from Wernicke's encephalopathy. In the adult, the disease is thought to be due to a less severe thiamine-related metabolic defect than that of the infantile form. The infrequent clinical recognition in the adult form may be due in part to lack of medical awareness.