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Biomedical subjects

H J Manz

Publications and source records attributed to H J Manz.

At least 73 records · Page 4Linked to original sources

Large arachnoid cysts at the cranial base.

Two infants who presented with macrocephaly had very large noncommunicating cysts at the cranial base that were displacing the brain. Only one of the patients was hydrocephalic; he eventually underwent ventriculoperitoneal shunting after craniotomy and attempted internal drainage of the cyst. The other infant had a cystoperitoneal shunt. Both have done well after 4 and 6 years of follow-up, respectively. These large arachnoid cysts are bilateral lesions with the potential for severe surgical complications and a questionable possibility of cure. The authors recommend systematic radiological investigation to decide the appropriate therapy, including an analysis of the patency of the subarachnoid pathways with radionuclide cisternography and cystography. Simple cystoperitoneal shunting may be the appropriate therapy for most of these large lesions. (Neurosurgery, 6: 76--81, 1980).

Arachnoid↗

Unilateral megalencephaly, cerebral cortical dysplasia, neuronal hypertrophy, and heterotopia: cytomorphometric, fluorometric cytochemical, and biochemical analyses.

A 13-month-old boy with intractable seizures, left hemiparesis, and psychomotor retardation due to right unilateral megalencephaly, died in hypovolemic shock 1 day after hemispherectomy. The gyral pattern of the hypermegalic hemisphere was simplified and coarse. The cortical cytoarchitecture was disarrayed by a population of giant neurons. Hippocampus and calcarine cortex were cytoarchitectonically normal, as was the entire left cerebral hemisphere. Neuronal heterotopias were present in the right centrum semiovale and both cerebellar hemispheres. Cytomorphometric study of parietal cortex of each cerebral hemisphere revealed a 4-fold increase in neuronal nuclear, and 11-fold increase in neuronal nucleolar, volume in the hypermegalic hemisphere, whereas glial nuclear volume was only one-third as great, in part because of edema of the left hemisphere. Microfluorometric cytochemical analysis demonstrated a 16% increase in neuronal DNA, 40% increase in total neuronal RNA, 12% increase in glial DNA, and 15% increase in glial RNA on the right. Biochemical analysis of tissue extracts disclosed increases in the right hemisphere of 40%, 56%, and 66%, respectively, for DNA, RNA, and protein. The data suggest heteroploidy of chromosomal DNA and enhanced transcription and translation in the hypermegalic hemisphere. Thus, a defect in regulation of cell metabolism may account for the morphologic and clinical abnormalities.

Brain↗

Delayed radiation necrosis of brainstem related to fast neutron beam irradiation: case report and literature review.

A 42-year-old man developed necrosis of the brainstem 10 months following fast neutron irradiation of a recurrent adenocystic carcinoma of the right submandibular salivary gland. The neoplasm had been diagnosed 15 years earlier. Neck dissection and several local excisions failed to control local extension, so that 7000 rad 4.8 MeV x-rays were administered. The right mandible and part of the tongue and palate were resected for recurrence and chemotherapy and transfer factor were given. Because of cranial neuropathy and erosion of the skull base, fast neutron radiation (2.080 rads) was administered five and one-half years after the x-ray therapy, but to a nonoverlapping field. Ten months later, rapidly progressive symptoms and signs of multiple cranial nerve palsies on the right side and left hemihypesthesia and hemiplegia appeared. Clinical manifestations of brainstem necrosis lasted 4 months. Postmortem examination demonstrated radionecrosis of pons and upper medulla, predominantly on the right side. This case illustrates the efficacy of neutron radiation in eradicating local carcinoma and also the serious complications of this therapeutic modality.

Adult↗

Dissecting aneurysm of cerebral arteries in childhood and adolescence. Case report and literature review of 20 cases.

A nine year old girl died with a massive infarct of the right cerebral hemisphere causing transtentorial and foramen magnum herniation. The infarction was secondary to an idiopathic dissecting aneurysm. The case is unusual in that the supraclinoid segment of the right internal carotid artery, the anterior cerebral artery, and the middle cerebral artery and its three branches were embedded and sectioned longitudinally. The dissection commenced in the supraclinoid segment of the right internal carotid artery, extended into the middle and anterior cerebral arteries, and was accompanied by thrombosis of the false lumen. A literature review of 20 pediatric cases indicates the malignant natural course of the disease (76% mortality in the first two months), and emphasizes the characteristic angiographic "string sign". The diagnosis of cerebral arterial dissection during life depends on angiography and a high index of suspicion.

Adolescent↗

Herpes simplex type 2 encephalitis concurrent with known cerebral metastases.

A 62-year-old woman developed neurologic deficits 7 months after pulmonary lobectomy for alveolar cell carcinoma of the lung. CT scan of the head demonstrated two metastases with marked peritumoral edema. Administration of Decadron, chemotherapy and 3,000 rad cranial radiation resulted in dramatic improvement of dysphasia and right hand paresis. Almost 2 months later, rhythmic, involuntary movements of the left hand developed. There was progression to multifocal seizures, grand mal seizures, postictal depression, status epilepticus, and coma, with death 9 days after onset of the movement disorder. Bronchoalveolar carcinoma was widely disseminated in lungs and bones, and as three metastases in brain. Bland "ischemic" necrosis in a pseudolaminar pattern was present in the neocortex. Innumerable Cowdry type A intranuclear inclusion bodies were seen in neurons, astrocytes, and oligodenodroglia. Immunofluorescence demonstrated Herpes simplex virus type 2 antigen and electron microscopy revealed virions with the morphology of the Herpes group. The case is significant for (1) the concurrence of intracranial metastases and Herpes simplex encephalitis, and (2) the causal agent, Herpes simplex virus type 2. The implication for the clinical neurocientist is the potential in a patient with systemic cancer, for the causation of neurologic complications by more than one factor or mechanism.

Adenocarcinoma, Bronchiolo-Alveolar↗

Correlation between volumetric CT imaging and autopsy measurements of glioblastoma size.

A three-dimensional reconstruction and display technique (THREAD SYSTEM) for serial computed tomography (CT) was employed in monitoring tumor volumes in two children under chemotherapy for glioblastoma multiforme of the cerebral hemispheres. Progressive diminution of tumor bulk was documented in the first patient and an increase in volume in the second. The first patient expired of the complications of his therapy and the second of transtentorial herniation. Independent measurements of the tumors as determined by a CT scan near the times of death and tumor dimensions measured at autopsy revealed good correlations between the radiographic and the anatomical data. The final CT measurement of tumor volume of 20 cm3 compared with an autopsy calculation of 13.3 cm3 in the first case. In the second case, CT volume was 417 cm3 and the actual volume of the primary tumor mass at autopsy was 437 cm3. The results suggest that the THREAD system is a practical method for monitoring the results of radiotherapy and chemotherapy in certain types of cerebral neoplasms.

Adolescent↗

Myopathy of chronic organophosphate poisoning: a clinical entity?

The acute aspects of organophosphate poisoning are well understood. Persistent weakness and muscular wasting in some cases have been attributed to neuropathic effects resulting in muscular atrophy from denervation. Recently, necrosis of skeletal muscle has been induced by chronic inhibition of cholinesterase with organic phosphates in a reproducible experimental model. The literature on organophosphate poisoning in man alludes to cases in which it appears plausible to postulate this mechanism as a cause of a residual myopathy. A new case in which chronic organophosphate intoxication may be implicated in the etiology of a proximal myopathy is presented. Reports of additional cases, followed up from the onset of symptoms, are needed to determine whether primary myopathic effects are a clinically significant complication of chronic organophosphate poisoning.

Adult↗

Cycloleucine uptake in the brainstem of thiamine-deficient rats.

Immature female rats were subjected to acute dietary deficiency of thiamine. An autoradiographic method was used in the semi-quantitative determination of concentration of 1-aminocyclopentane-1-carboxylic acid-carboxylic-14C (cycloleucine) in brainstem regions after intravenous administration of tracer quantities. The time course of tissue concentrations was followed and compared with that of normal and isocaloric control animals. Our data indicate that thiamine deficiency of sufficient magnitude to induce brainstem lesions has an effect on the transport of cycloleucine. The initial 2 min values in the nuclear areas are appreciably reduced whereas the 6 min values are significantly elevated as compared with controls; no change was demonstrated in the white matter of the inferior cerebellar peduncle. The effect on transport appears to be more on the mechanism of efflux than of influx in terms of blood brain barrier function.

Amino Acids↗

Glucose uptake in the brainstem of thiamine-deficient rats.

Acute dietary deficiency of thiamine was produced in immature female rats. Uptake of glucose by brainstem nuclei was determined by autoradiographic examination of tissue concentrations of 14-C-3-O-methyl-d-glucose following a test dose, and compared with levels in normal and isocaloric control animals. The experiment showed that glucose uptake was depressed in the lesions of thiamine deficiency as compared with the controls, that the depression occurred with the occurrence of morphologic evidence of tissue edema, and that the depression was temporally independent of the breakdown of the blood-brain barrier to protein which is found in the late, necrotic lesions.

Animals↗

Vascular permeability to horseradish peroxidase in brainstem lesions of thiamine-deficient rats.

In the early brainstem lesions of acute dietary thiamine deficiency in rats, an outstanding feature is the occurrence of edema. This study, using horseradish peroxidase as a marker, confirms and extends our previous observations using fluorescent dye-labeled albumin that vascular permeability to proteins remains essentially intact during this phase, and becomes altered only when necrosis and hemorrhage supervene. In addition, however, although a minor degree of pinocytotic transendothelial transport and pericytic uptake of horseradish peroxidase was found in both normal animals and in the early lesions, in the late lesions there was markedly enhanced pinocytotic transport but no evidence that interendothelial junctions were disrupted. Reaction product was present in the vascular basement membrane regions and in the extracellular spaces of the neuropil, as in other forms of reactive edema. The localized cerebral edema in thiamine deficiency lesions is thus a biphasic phenomenon. It occurs initially in the absence of vascular permeability change and perhaps as the result of defective active transport related to the deficiency state. In contrast, the late edema is the result of gross breakdown of barrier function to protein, with enhanced pinocytotic transport and extracellular accumulation of the marker in the neuropil.

Animals↗