PubMed HealthSearch

Biomedical subjects

C L Scholtz

Publications and source records attributed to C L Scholtz.

At least 19 recordsLinked to original sources

The effect of the microphthalmia gene on pre-natal optic nerve development in the mouse.

The purpose of this study was to examine the effect of the microphthalmia gene on pre-natal optic nerve development in the mouse. Coronal serial sections of wild-type, heterozygote and homozygous microphthalmic embryonic optic nerves are examined throughout gestation. No obvious morphological abnormality was identified in the heterozygote. The microphthalmic optic stalk/nerve was larger than that of the wild-type and heterozygote and there was persistence of the optic stalk throughout gestation. This was due to a high mitotic rate and reduced cell death in the dorsal layer of the microphthalmic optic stalk as well as persistence of the optic ventricle throughout gestation. The latter was associated with persistence of intermediate-type junctions between the neuroepithelial cells lining the ventricle and failure of the cells on the dorsal aspect of the distal stalk to degenerate. Possible mechanisms for the disappearance of the optic ventricle in the normal optic stalk are suggested.

Animals

Axonal injury in closed head injury by assault: a quantitative study.

Due to the controversy in the literature regarding the time course of axonal balloon formation in human material, we wished to determine if it was possible to diagnose axonal injury before the development of axonal balloonings. The hypothesis was that the presence of axonal swellings or axonal enlargements associated with a glial reaction could be used as a diagnostic aid in human axonal injury before 12 hours. The brains of eight individuals that survived for less than 48 hours following head injury, and also had evidence of axonal injury using the criteria of Vanezis et al. (1987), were systematically studied by looking at axonal swellings, axonal balloonings, reactive astrocytes, maximum diameter of axonal enlargements and density of axonal enlargements. Controls were eight selected cases without neurological disease. The variables studied were assessed in 25 fields from ten different areas of the brain, using silver stains and immunoperoxidase for glial fibrillary acidic protein (GFAP). Logarithms of one plus the count of each variable were taken from the raw data and these were analysed using percentile distribution and the median, the t-test, Mann-Whitney U test and the Wilcoxon signed rank test. We conclude that quantitation of axonal damage allows the detection of mild degrees of axonal injury that could be overlooked on routine examination, and that the criteria of axonal enlargements, rather than axonal balloonings, are indications of axonal damage, cannot be endorsed with the evidence provided.

Axons

The glial reaction in closed head injuries.

The development of the glial reaction in human closed head injury has been investigated using morphometry and statistical analysis. The brains of eight individuals that survived less than 48 h following closed head injury were analysed using immunoperoxidase for glial fibrillary acidic protein (GFAP). Controls were eight patients without neurological disease. The density of reactive astrocytes was estimated in 25 fields in each of 10 different areas sampled bilaterally avoiding the subpial and subependymal zones, and the perivascular white matter. There was great variation between the zones within and between groups, and considerable variation between individuals. The raw data were expressed as logarithms averaged and analysed using the median and non-parametric statistics. The corpus callosum in the head injury group showed the highest densities of reactive astrocytes, particularly in the splenium which achieved statistical significance using the non-parametric tests. This pattern was not reproduced in the control group. Although there was overlap between the head injured and control individuals, the head injury group had relatively higher densities in all zones, and showed an overall increase in the density of reactive astrocytes. This achieved statistical significance in the corpus callosum, the occipital subcortical white matter, and the cerebellum. This study has shown that the glial reaction is often prominent in the corpus callosum irrespective of the presence of a primary lesion although the pattern varies from case to case.

Adult

The prenatal development of the optic fissure in colobomatous microphthalmia.

The coloboma in the cinnamon mouse homozygous for the microphthalmia gene is caused when optic fissure closure, which normally occurs between the 11th and the 13th gestational day, does not occur. This study sought to determine the cause of this fusion failure, and to identify any foci of fusion that occur later in gestation. Microphthalmic fetuses from the 11th-20th gestational day were obtained by datemating cinnamon mice heterozygous for the microphthalmia gene. Coronal serial sections of the eyes were examined at light and electron microscopy. Initially, the fissure margins became apposed only in the posterior aspects of the eye. A failure of basement membrane disintegration at the fissure margins prevented fusion at the 12th and 13th days. On the 14th day, small foci of basement membrane disintegration were identified in the area of the developing optic disc. Although the fusion zone enlarged later in gestation, it was limited to the area of the optic disc and showed that the two retinal layers did not separate. This study has shown that abnormal growth and invagination lead to delayed apposition of the optic fissure margins. These features together with a failure of basement membrane disintegration appear to be the main factors involved in coloboma formation. It is suggested that the excessive number of outer-layer cells that are inverted into the fissure, as well as abnormal or reduced numbers of phagocytic cells, may affect the persistence of the basement membrane. Alternatively, a primary defect of the pigment epithelial cell may lead to the development of the hypercellular and nonpigmented outer layer associated with the lack of basement membrane disintegration and nonfusion in this mutant.

Animals

Selective and asymmetric vulnerability of corticospinal and spinocerebellar tracts in motor neuron disease.

The spinal cords of 10 cases of motor neuron disease were compared with those of six age-matched controls using myelin and silver impregnation methods, and the Marchi reaction for myelin degradation products. These studies revealed striking asymmetry in involvement of the lateral and anterior corticospinal tracts, without concordance in the pattern of involvement of these crossed and uncrossed corticospinal pathways. In addition there was prominent involvement of the posterior and anterior spinocerebellar tracts, but less marked abnormality was seen in the reticulospinal pathways. These findings highlight the asymmetrical involvement of the upper and lower motor neuron components of the motor system that is a characteristic feature of the disease, and demonstrate that involvement of the spinocerebellar system is a frequent finding.

Anterior Horn Cells

The effect of the gene for microphthalmia (mi) on the dorsal lateral geniculate nucleus of the cinnamon mouse.

The dystrophic retina of the cinnamon mouse homozygous for the gene for microphthalmia (mi/mi) has a population of large ganglion cells. Unilateral enucleation and examination of the dorsal lateral geniculate nucleus using the Fink-Heimer technique showed that, while there was continuing degeneration argyrophilia in the dorsal lateral geniculate nucleus secondary to the retinopathy, there was additional degeneration attributable to enucleation. In addition, the pattern of degeneration indicated that the axon terminals were less mature than those of the cinnamon and heterozygous (mi/+) mice. Quantitative study of the dorsal lateral geniculate nucleus in the homozygous (mi/mi) mouse showed that the nucleus is small with fewer neurons and that the markers for protein metabolism, namely volume of nucleoli and cytoplasmic RNA, are reduced when compared to the cinnamon and heterozygous (mi/+) mice. It is concluded that the portion of the retino-geniculate pathway represented by the large ganglion cells in the retina, develops in the absence of patterned visual stimuli, but is less mature and has a more limited functional activity than controls.

Aging

White matter damage following acute head injury.

The study of a series of brains from patients who had a severe head injury and died within 72 h without a lucid interval showed that there was a step-wise progression in the development of retraction balls. At 2 h after injury sinusoidal enlargement of the axons was evident. This progressed over 16 h when the lesions appeared as retraction balls which were fully developed at 72 h. There was a similar increase of staining with an immunoperoxidase method for glial fibrillary acid protein (GFAP) initially around blood vessels spreading diffusely into the white matter. The number of reactive astrocytes also increased. In a control case where the corpus callosum was torn at post-mortem there were sinusoidally distended and torn axons in the absence of GFAP staining. It is proposed that there are three components to a head injury. First, mechanical injury as seen in the control case; second, the development of retraction balls which are an active process probably representing damaged axons which cannot undergo repair where the sinusoidal swellings develop into retraction balls and third, an astrocytic reaction. The sinusoidal change, when present on its own, may not be separable from post-mortem trauma. However, when it is associated with an astrocytic response it should be correlated with coma in the same way as retraction balls.

Axons

Omnipause neurons in two cases of opsoclonus associated with oat cell carcinoma of the lung.

Opsoclonus is an involuntary eye movement disorder in which there are chaotic, usually conjugate, multidimensional saccadic eye movements. In this paper 2 cases of opsoclonus are reported, as a paraneoplastic phenomenon in association with oat cell carcinoma of the lung. It has previously been hypothesized that opsoclonus results from dysfunction of a group of premotor neurons in the brainstem called omnipause neurons. We describe the location of these cells in man by homology with animal studies, and describe the light microscopic appearance of these neurons in the 2 cases of opsoclonus. Although these neurons appeared normal it is still possible that their function was disturbed as a result of metabolic or neurotransmitter abnormalities.

Aged

Diffuse axonal injury in early infancy.

Diffuse axonal injury typified by retraction balls and axonal swellings was identified in the brains of a series of infants, 5 months old and younger, who had suffered closed head injuries. These axonal discontinuities were shown by using Nauomenko and Feigin's silver method, which is particularly useful for showing fine axons such as those found in the developing brain. Diffuse axonal injury in early infancy may occur in the same way as that described in adults. The low incidence of intracerebral haematomata suggests that recurrent trauma to the head from a combination of direct contact and shaking results in axonal damage to the poorly myelinated axons and that blood vessels are rarely damaged.

Axons

Complicated colobomatous microphthalmia in the microphthalmic (mi/mi) mouse.

A study of the development of the eye in the cinnamon mouse, homozygous for the gene for microphthalmia (mi), has shown that the microphthalmia is due to failure of secondary vitreous formation associated with a coloboma. The retina is dystrophic but there is a residual population of large ganglion cells and the optic nerve also contains ganglion cells. All these ganglion cells have cytoplasm similar to the retinal ganglion cells in the normal controls. It is postulated that they communicate with axons in the optic nerve. In addition, the outer epithelial layer of the eye cup, which normally becomes pigmented, forms retinal tissue in the homozygous mouse and this is also true of the dorsal part of the eyestalk near the eye.

Animals

MRI in acoustic neuroma: a review of 35 patients.

This retrospective study is aimed to assess the diagnostic efficacy of MRI in relation to contrast enhanced CT and air-CT-cisternography. MRI examinations were performed in 35 patients with suspected neurosensorial damage and suggestive of acoustic neuroma: 27 presented on MRI with unilateral tumors, 3 patients had a bilateral tumor and 5 patients were negative on all imaging modalities. The total number of acoustic neuromas detected was therefore 33. To date microscopic analysis has been performed on 12 tumors and histological data based on type Antoni A and Antoni B classification is available. Contrast enhanced CT detected 19 tumors, yielding an overall sensitivity rate of 58%. Air-CT cisternography identified an additional 5 tumors with a sensitivity rate of 100%. MRI identified 33 acoustic neuromas in 30 patients and was negative in 5 patients (sensitivity and accuracy 100%). Considering sensitivity in relation to location, MRI was much better than contrast enhanced CT for internal auditory canal (IAC) tumors (100% versus 36%) and better for cerebello-pontine angle tumors (CPA) tumors (100% versus 68%). The evolution of MRI technique, the various pulse sequences used and their actual selection is discussed. Seven patients received a paramagnetic contrast agent (Gadolinium-DTPA) with the additional benefit of a better demonstration of the tumor. The results suggest that MRI is the best non invasive technique for demonstrating acoustic neuromas.

Adolescent

Diffuse meningocerebral angiodysplasia and renal agenesis: a case report.

A third case of meningocerebral angiodysplasia involving the cerebral cortex, with necrosis of both cortex and white matter is described in a neonate with Potter's syndrome. It is suggested that cortical vessel anomalies act as a local intracerebral shunt to produce periventricular infarction. This disorder differs from pure meningeal angiectasis without cerebral infarction and from the classic arteriovenous malformation of the central nervous system with massive systemic shunt, cardiomegaly, and neonatal cardiac failure. Meningocerebral angiodysplasia and renal agenesis appear to form a rare but distinct association.

Abnormalities, Multiple

Toxocara canis larvae in the brain of a British child.

The clinical and autopsy findings of a two and a half year-old infant with Toxocara sp. infection of the brain and granulomatous lesions in the liver are reported. The cause of death was non-accidental injury. The relationship between Toxocara infection and behavioural disorders is discussed.

Ascariasis

Cerebellar degeneration in dominantly inherited spastic paraplegia.

The clinical features of five affected members in three generations of a family with dominantly inherited Strumpell's spastic paraplegia are described, together with the pathological findings in two cases. The late presentation and slow progression of the disease encompass features of the types I and II of other authors illustrating the heterogeneous expression of the disorder. Cerebellar involvement was evident clinically and pathologically.

Cerebellar Ataxia

Aggressive osteoblastoma.

The clinical, radiological and pathological features of two cases of an osteogenic tumour with long survival are described. The tumours have the histological pattern of benign osteoblastoma with other more cellular and aggressive features suggestive of a low grade osteosarcoma. They are locally invasive but the absence of metastases indicates that separation from both entities is justified clinically and pathologically. The term aggressive osteoblastoma is suggested.

Adult

The quantitative histochemistry of ribonucleic acid using gallocyanin.

A method for the cytophotometric estimation of ribonucleic acid in tissue sections using gallocyanin-chrome alum is described. The dye obeys Beer's law in gelatin sections. The effect of deoxyribonuclease on the staining of ribonucleic acid is also investigated. The results indicate that this method is of value in the quantitation of ribonucleic acid.

Alum Compounds