Compliance to treatment of latent tuberculosis infection in a region of Israel.

Background: One of the measures adopted in Israel since 1959 as part of the tuberculosis control program was screening children aged 12-13 years old. The screening comprised single-step tuberculin skin testing using the Mantoux method.

Objectives: To assess the efficacy of tuberculin skin screening for TB in schoolchildren in southwestern Israel as well as the compliance to treatment for latent tuberculosis infection.

Supraophthalmic intracarotid infusion of BCNU for malignant glioma.

We treated five patients with 11 supraophthalmic infusions of BCNU at 200 mg/m2 every 2 months. All three patients with residual tumors showed marked CT response after one infusion. Two patients with bilateral tumors had no response on the contralateral side.

Sarcoma arising in metastatic carcinoma in the brain. A second instance.

A second instance in which a carcinoma metastatic to the brain has induced the formation of a sarcoma in the associated cerebral blood vessels, is reported. This is analogous to the more common gliosarcoma, a tumor in which a primary glioma, most often anaplastic astrocytoma (glioblastoma multiforme), has induced the formation of a similar sarcoma, with both neoplastic tissues in the same tumor mass. The formation of the sarcoma is attributed to a neoplastic change in the markedly hyperplastic endothelial cells of the cerebral blood vessels that are very commonly found with anaplastic astrocytomas and are often found in relation to metastatic carcinoma.

Neural defects in Say-Gerald (VATER) syndrome.

The Say-Gerald (VATER) syndrome consists of vertebral defects, anal atresia, tracheoesophageal fistula, radial dysplasia and renal defects. 2 children with Say-Gerald (VATER) syndrome were autopsied: the first child was found to have hydrocephalus, aqueductal stenosis and probable craniosynostosis, and the second child had hypoplasia of the nerve roots and anterior and posterior horns of the spinal cord, corresponding to the hypoplastic limb. It is suggested that the Say-Gerald syndrome is a multifocal developmental disorder in which central nervous system anomalies may be found.

Cerebellocele and associated central nervous system anomalies in the Meckel syndrome.

Three cases with the Meckel syndrome were autopsied and found to have: arhinencephaly , polymicrogyria , aqueductal stenosis, heterotopia of glial tissue, hypoplasia or agenesis of the cerebellar vermis, cranium bifidum associated with large occipital ventriculocele and others. The anomalies at the level of posterior fossa in this condition are classified as those belonging to the Chiari type III group of anomalies. This unusual set of anomalies which forms pathogenetic link between the Dandy-Walker and Chiari-Arnold group of anomalies in the posterior fossa seems to be very frequent in the Meckel syndrome.

Intracranial lipomas, hydrocephalus and other CNS anomalies in oculoauriculo-vertebral dysplasia (Goldenhar-Gorlin syndrome).

13 cases of Goldenhar-Gorlin syndrome are presented in which numerous central nervous system anomalies have been found. These include occipital encephalocele, hydrocephalus, aqueductal stenosis, agenesis of corpus callosum, multiple congenital lipomas and many others. Pertinent literature has been reviewed.

Encephalocele (cerebellocele) in the Goldenhar-Gorlin syndrome.

A patient with unilateral preauricular appendages and occipital mass is described who died on the fifth day of life. An autopsy demonstrated congenital megabladder and megaureters and dysplastic kidneys. The neuropathological examination revealed cranium bifidum in the occipital region, defective posterior arch of the C1 vertebral body, agenesis of vermis and a large cerebellocele.

Iniencephaly: a neuropathologic study.

Five cases of iniencephaly are reviewed. Numerous central nervous system malformations were found at all levels, including microencephaly, polymicrogyria, heterotopic glial tissue in the leptomeninges, atresia of the ventricular system, marked disorganization of the brain stem, vermian agenesis, large cerebellar cyst, and disorganization of the spinal cord tissue. The cerebellum was normal in one case.

Extensive advanced maturation of medulloblastoma to astrocytoma and ependymoma.

Four cases of medulloblastoma in children are reported in which a marked degree of differentiation towards neoplastic adult glioma cells was observed. The original immature and highly malignant tissue had largely disappeared in three and completely disappeared in the fourth. The mature tissues which replaced them included astrocytoma tissues in all four, but ependymoma tissues predominated in two.

The effect of saponification on the mucopolysaccharides of the ground substance of the human brain: the relation to focal edema and multiple sclerosis.

The acid mucopolysaccharides of brain tissues are disclosed by their metachromatic staining with toluidine blue following saponification with potassium hydroxide, presumably as a result of the liberation of acid groups previously esterified. Earlier histochemical studies had disclosed the presence of neutral mucopolysaccharides by staining with the periodic acid-Schiff technique, and such staining is intensified by prior saponification. Many biochemical studies have reported the presence of both acid and neutral mucopolysaccharides in brain tissues.

An axoplasmic component of the Schmidt-Lanterman deformation.

In three instances, peripheral nerves containing numerous Schmidt-Lanterman discontinuities of myelin revealed transverse, wedge-shaped, or arrowhead-shaped axoplasmic excrescences visible with axon stains. These were arranged with a frequency and length like that of the myelin clefts, but the axonal materials were located within the myelin masses bordering the clefts, not in the clefts themselves. These axoplasmic changes are not consistent with most assumptions concerning the physiologic functions of the clefts, and supports the concept that the clefts are artifactual, due possibly to mechanical factors acting an abnormal nerve fibers.

The influence of the ground substance on the extracellular water of normal and edematous human brain: focal edema and the demyelinating diseases, including multiple sclerosis.

The presence of a ground substance in brain provides a mechanism by which edema localized to one region of the white matter might occur without spreading diffusely into the adjacent tissues. The most common such localization is the sparing of the arcuate white matter when the deeper white matter is markedly edematous. This may be related to the higher concentration of mucopolysaccharides in the former.

The mucopolysaccharides of the ground substance of the human brain.

The concept of the presence of mucopolysaccharides forming an interstitial ground substance in central nervous tissues is revived. The biochemical reports of both neutral and acid mucopolysaccharides in both gray and white matter of the normal brain are reviewed. The earlier histochemical reports and the histochemical studies in this laboratory are appraised and are thought to demonstrate the presence of neutral mucopolysaccharides in both gray and white matter, more evident in the former.

Subarachnoid dissemination of thoracic chordoma.

Chordoma at the T-9 level, originally manifested by lumbosacral radiculopathy, developed in a 50-year-old man. The patient underwent three operations and radiation therapy for this condition, but ten years later, thoracic myelopathy developed, followed by left facial weakness. On autopsy, extensive dissemination of chordoma was found along the base of the brain and in the leptomeninges of the spinal cord.

Mixed mesenchymal tumors: meningioma and nerve sheath tumor.

Two spinal tumors are reported which consisted of an admixture of meningiomatous and nerve sheath tumor tissues. The former was represented by whorled tissues in both cases, syncytial areas in one and psammoma bodies in the other. The latter was represented by cells with parallel oriented, elongated nuclei and intercellular connective tissue fibers, which contained neurons and myelinated axons.

The role of edema in diffuse sclerosis and other leukoencephalopathies.

The pathologic changes associated with broad zones of demyelination of the cerebral white matter, the diffuse sclerosis group, are analyzed with respect to the contribution of cerebral edema to their pathogenesis. The characteristics of cerebral edema in general are reviewed, and the interpretation offered that only one type of cerebral edema, that characterized by an extracellular accumulation of fluid, occurs in man. In the diffuse sclerosis group, edema is most readily recognized by the tendency for the arcuate zone of the white matter to be spared when the deeper white matter is severely degenerated.

Laminar scars in cerebral white matter: a perinatal injury due to edema.

Branched plate-like demyelinated lesions were present in the gyral and central white matter of two individuals, 3 and 54 years of age. The degenerated areas contained very few axons and were densely gliotic, and in the older case, contained connective tissue fibers and were continuous with a large parenchymal cyst. The lesions were covered on both sides by normally myelinated white matter, often representing only the subcortical arcuate white matter, occasionally being considerably broader.

Maturation and anaplasia in neuronal tumors of the peripheral nervous system; with observations on the glial-like tissues in the ganglioneuroblastoma.

Six cases of peripheral ganglioneuroblastoma are described and analyzed; in general these contained neuroblasts, mature ganglion cells and cells of intermediate degrees of maturity. Portions of the tumor resembled the glial tissues of the central nervous system, but this is spurious, no glial cells or fibers being present. Such tissues contained cells of intermediate maturity, immature axons which characteristically stain poorly with silver stains for axons, and a paucity of connective tissue fibers.

Subacute necrotizing encephalomyelopathy in a neonatal infant.

This infant, born after a normal pregnancy by cesarean section, appeared entirely normal for the first 44 hours of life, at which time she was found to have become completely apneic, suddenly and without known cause. The infant was resuscitated, placed in a respirator, treated energetically but with limited response, and expired 15 days later. At autopsy, bilaterally symmetric lesions were found in the walls of the third ventricle, the periaqueductal gray matter and the floor of the fourth ventricle, characterized by marked destruction of most neural elements, but with a selective preservation of neurons and their processes.