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Biomedical subjects

C M Shaw

Publications and source records attributed to C M Shaw.

At least 19 recordsLinked to original sources

Radiation therapy for sarcoid of the thalamus/posterior third ventricle: case report.

There are a limited number of previously reported cases involving the use of radiation therapy for sarcoid of the brain. The case of a 22-year-old man with a thalamic/posterior third ventricle sarcoid mass that grew despite steroid medication is presented. The patient was treated with external beam radiation to a total dose of 20 Gy, with 2-Gy fractions over 14 elapsed days. A complete radiographic response was achieved 4 months after radiation was completed. Radiographic follow-up through 8 months postradiation shows no evidence of disease recurrence. Fractionated radiation therapy in low-to-moderate doses appears to be efficacious in steroid-refractory sarcoid of the brain.

Adult

Electrocorticography in patients with medically intractable temporal lobe seizures. I. Quantification of epileptiform discharges prior to resective surgery.

We studied retrospectively the intraoperative preresection electrocorticograms (ECoGs) of 72 patients undergoing surgery for medically intractable, mostly complex partial, temporal lobe seizures (TLS). Quantification of interictal epileptiform discharges (EDs) detected visually at each electrode location in 2 min recording epochs included computations of ED rates (EDs/min) and cumulative voltages (CuVs) (microV/min). Of 6388 EDs, 81% involved the infratemporal surface, 18% the lateral temporal surface and 1% the orbital frontal area. Forty-eight patients (67%) demonstrated multiple (up to 5 or more), temporally independent foci. Dominant foci in medial and lateral infratemporal locations were about equally common and were significantly more frequent than in lateral temporal locations. Rankings of ED CuVs and rates at individual cortical locations defined 4 areas of "relative interictal cortical epileptogenicity." These were arranged in an orderly pattern with the anterior parahippocampal gyrus and the inferomedial surface of the temporal tip displaying the highest and the lateral temporal and posterior infratemporal cortices showing the lowest propensity to the interictal epileptiform discharge. Individual areas were not characterized by distinct clinical seizure manifestations. Preresection ECoGs provide information on the epileptogenic dysfunction that involves most of the temporal lobe of patients with medically intractable TLS.

Adolescent

Electrocorticography in patients with medically intractable temporal lobe seizures. II. Quantification of epileptiform discharges following successive stages of resective surgery.

We quantified retrospectively the interictal epileptiform discharges (EDs) detected visually in the electrocorticograms (ECoGs) of 42 patients undergoing successive stages of anterior temporal lobectomy for medically intractable temporal lobe seizures (TLS). Following first resection sparing the hippocampus (H) and the parahippocampal gyrus (PHG), EDs were recorded on both structures in all patients and by far exceeded in amount those on residual lateral infratemporal and lateral temporal cortices. Frequently, EDs occurred apparently simultaneously but with opposite polarities on the H and the PHG, but more complex relationships were also evident in most individuals. These features likely reflected abnormal post-synaptic activity generated at different locations and cortical depths within the H, PHG, or both. Quantification of epileptiform activity and the effects of selective anterior hippocampectomy or parahippocampectomy suggested that both the H and PHG had remarkable epileptogenic potential. Levels of epileptiform activity were not significantly different in the H and PHG and in the H of subjects with and without H sclerosis. After final resection, including the amygdaloid nucleus (AN), anterior H and PHG, interictal EDs were present, although markedly diminished, in 35 patients. Postresection foci were significantly less numerous and extensive, and attained smaller maximal voltages, than did foci before and after first resection.

Adult

Chronic illness: a media perspective.

The media can play a major role in creating supportive environments. They influence the man in the street as well as the decision-maker. They help set norms; they contribute to fostering community action. The information they transmit carries prestige. Health workers know this. They realize the power of communication. But seldom do they know how to approach media professionals and to provide them with what they need most: adequate, to-the-point and lively information. This paper provides them with useful guidelines on how to develop effective cooperation with the media and "help the media to help them" transmit the health message and create supportive environments.

Attitude to Health

Comparison of postmortem magnetic resonance imaging and neuropathologic findings in the cerebral white matter.

Two types of high-signal intensity abnormalities are frequently found bilaterally in the cerebral white matter of brains of elderly patients on T2-weighted magnetic resonance imaging (MRI) scans. One is located in the immediate periventricular region; the other, in the deep subcortical white matter (centrum semiovale). The diagnostic implications of this second type continue to be uncertain. To determine the neuropathologic correlates of these lesions, the brains from seven elderly patients were fixed in buffered formaldehyde solution, subjected to MRI scanning, and examined neuropathologically. Variable degrees of bilateral periventricular (subependymal) sharply defined areas of high-signal intensity were found in all the brains, and the larger of these showed corresponding areas of myelin pallor with gliosis and dilated perivascular spaces. Discrete bilateral patches of high-signal intensity were found in the centrum semiovale in five patients. Myelin and axon stains showed varying degrees of diffuse white matter pallor in many areas examined, both with and without these areas of high-signal intensity on MRI scans. Neither the myelin nor the axon stains showed discrete white matter abnormalities that corresponded to the MRI findings. We believe that these changes, so commonly found on MRI scans in the elderly, reflect actual changes in the white matter but that their nature and clinical significance need to be elucidated.

Aged

Brain hemorrhages in cocaine-exposed human fetuses.

Autopsies of 4 fetuses exposed to maternal cocaine are reported. Brain examination revealed hemorrhages in 3 of the fetuses involving the germinal matrix. The hemorrhages resembled subependymal germinal matrix hemorrhages seen as postnatal complications in premature infants with idiopathic respiratory distress syndrome. One of the placentas had sonographic evidence of abruption which could not be confirmed pathologically. The findings are discussed in light of reports of neurobehavioral deficits and other congenital anomalies in children and animals exposed to cocaine in utero. Speculations about the pathophysiologic events leading to these findings are made.

Adult

The effect of age on the latency of radiation myelopathy.

The latent period to forelimb paresis following photon irradiation of the cervical spinal cord was evaluated in Sprague-Dawley rats ranging in age from 9 days to adulthood. The radiation was administered dorsally in single fractions, and in 15-day-old animals, to different lengths of the rostral cord and in doses ranging from 16 to 38 Gy. The duration of the latent period was found to be directly proportional to the age of the animal at the time of irradiation, and independent of radiation dose or the volume of the cervical cord which was irradiated. In the majority of paretic animals, the irradiated segment of the spinal cord demonstrated white matter necrosis. The results indicate that in the developing rat, the manifestations of radiation myelopathy are delayed by an interval determined in part by the age of the animal at the time of irradiation.

Age Factors

Cerebellar venous infarction: case report with clinicopathologic correlation.

A diabetic man developed a severe hyperosmolar state resulting in coma. CT of the head showed bilateral cerebellar hemorrhages. Despite medical treatment, he deteriorated and died. At autopsy, the straight sinus was thrombosed. There were bilateral, hemorrhagic, cerebellar venous infarctions. This condition is rare because of abundant collateral venous drainage.

Cerebellum

Acute failure of forebrain with sparing of brain-stem function. Electroencephalographic, multimodality evoked potential, and pathologic findings.

We describe a case of acute failure of forebrain with preservation of brain-stem function following cardiopulmonary arrest. This condition is distinguished from persistent vegetative or noncognitive states. Multimodality evoked potentials showed electroretinograms but no cerebral potentials to flash stimulation of both eyes; normal electrocochleograms and brain-stem auditory-evoked potentials to click stimulation of both ears; and Erb's point, N13, and P14 but no cortical responses to electrical excitation of both median nerves. Pathologic alterations consisted of pseudolaminar necrosis involving the whole cerebral cortex and other forebrain regions, with relative preservation of brain stem and spinal cord. Close correspondence existed between electrophysiologic, clinical, and major neuropathologic alterations.

Audiometry, Evoked Response

Transsynaptic spread of varicella zoster virus through the visual system: a mechanism of viral dissemination in the central nervous system.

We report a patient with pathologic evidence of anterograde spread of varicella zoster virus (VZV) through the visual system. A 29-year-old homosexual man developed the acquired immunodeficiency syndrome (AIDS) 2 months before the onset of left herpes zoster ophthalmicus. During the next 11 months, the zoster infection progressed to involve the left eye, with resultant keratitis, iritis, retinitis, and eventual blindness. Later, the patient developed bilateral blindness, left hemiparesis, and fatal pneumonia. At autopsy, the brain revealed destruction of the visual system and adjacent structures, with sparing of the remainder of the brain. Glial cells near the areas of necrosis showed Cowdry type A intranuclear inclusions. In situ hybridization with probes to VZV nucleic acid sequences were positive in the necrotic brain and retinal areas. Hybridization with probes to cytomegalovirus, herpes simplex virus type II, human immunodeficiency virus, and Epstein-Barr virus were negative. Electron microscopy revealed characteristic herpes group nucleocapsids. This case provides insight into the mechanisms of virus dissemination and the production of encephalitis.

Acquired Immunodeficiency Syndrome

Megalencephaly in sudden infant death syndrome.

The fresh brain weights of 79 infants with sudden infant death syndrome (SIDS) were tabulated and compared with expected "normal" brain weights. The series included 47 males and 32 females. Their ages ranged from 8 days to 16 months with a median of 2.5 months. The weights of all these brains were above the 50th percentile for age, and 64 of 79 brains (81%) were above the 95th percentile, including 15 (19%) at or above the 99.9th percentile. The ratio of the brain stem plus cerebellum weight to the whole brain weight of 93 formalin-fixed brains from victims of SIDS showed that the cerebrum was disproportionately heavier in most cases. No gross or microscopic evidence of cerebral edema was noted to account for the heavier weight. The significance of these findings is debatable. The increase in brain weight was less obvious in infants younger than 1 month of age but accelerated after that age. The brain weights of infants with SIDS may represent the really "normal" weights, since these patients were not known to be ill prior to death. If the brain weights of SIDS infants are really heavier than "normal," the possibility must be considered that disproportionately rapid growth of the brain during early infancy may be detrimental to the neural control of the cardiorespiratory system.

Brain

Congenital difficulties with swallowing and breathing associated with maternal polyhydramnios: neurocristopathy or medullary infarction?

Two babies with congenital difficulties in swallowing and breathing are presented and contrasted. Both were associated with maternal polyhydramnios, but one was due to medullary infarction and the other to a malformation ("neurocristopathy"). In the former case, isolated tenth and twelfth cranial nerve palsies provided the clue as to the correct etiology, whereas the latter baby had dyscoordination of swallowing and breathing with sleep apnea ("Ondine's curse"). It is somewhat ironic that the case without a difficult delivery had the infarct, probably prenatal in onset, whereas the one with a difficult delivery had the congenital malformation. Neural crest cells are known to migrate widely and to develop greatly different functions, but the fact that their central associations of neural tube origin may also be affected has not previously been emphasized in the etiopathogenesis of the congenital malformation. A variety of syndromes with combinations of many defects may be seen in which too many or too few cells are formed, with more or less serious consequences especially for the respiratory and gastrointestinal systems.

Breech Presentation

Choroid plexus cysts in fetuses with trisomy 18.

An association between fetal choroid plexus cysts and trisomy 18 has been suggested. However, the prevalence of such cysts in aneuploid fetuses is unknown. To determine this frequency, we studied 14 fetuses with trisomy 18 examined at the Central Laboratory for Human Embryology. Five fetuses were found to have choroid plexus cysts on postmortem ultrasound examination. All those with cysts were earlier than 26 weeks in gestation, and the prevalence among second-trimester fetuses was 71.4%. In contrast, such cysts are reported in less than 1% of the general population of second-trimester fetuses. Thus, choroid plexus cysts are common in trisomy 18, and the finding of such cysts on a second-trimester ultrasound examination should suggest further evaluation, including chromosome analysis.

Choroid Plexus

Chronic remitting-relapsing experimental allergic encephalomyelitis induced in monkeys with homologous myelin basic protein.

A chronic remitting-relapsing form of experimental allergic encephalomyelitis (EAE) has been produced in monkeys sensitized to homologous myelin basic protein in Freund's complete adjuvants by the technique of suboptimal treatment after the onset of disease. Not only does the clinical course resemble that of human multiple sclerosis more closely than does the clinical course of acute EAE, but so also does the histological reaction, with more-nearly pure demyelination, rather than the hyperacute hemorrhagic-necrotic lesions that occur so commonly in untreated monkeys with ordinary acute EAE.

Animals

Radiation, methotrexate, and white matter necrosis: laboratory evidence for neural radioprotection with preirradiation methotrexate.

To investigate the interactions between methotrexate (MTX) and irradiation of the central nervous system, adult rats were infused with MTX via the lateral cerebral ventricle either before, during, or following single fraction irradiation to the cervical spine. Single doses ranging from 1600 cGy to 3200 cGy were administered and the dose-response curve for forelimb paralysis was compared with that seen in irradiated animals which did not receive MTX. There was no effect on the dose-response curve when MTX was administered simultaneously with or following irradiation compared to radiation alone. When MTX was given prior to irradiation, however, the entire dose-response curve shifted in the direction of radioprotection by approximately 225 cGy. Histopathologic examinations were consistent with this observation, with animals pretreated with methotrexate demonstrating significantly less white matter necrosis than observed in untreated controls. Protection of normal CNS tissue from radionecrosis, and from the associated paralysis, may be achieved with preradiation methotrexate.

Animals

Acute haemorrhagic leucoencephalitis with a prolonged clinical course.

Two cases of pathologically proven acute haemorrhagic leucoencephalitis with clinical features resembling a cerebral tumour are reported. One patient with a left fronto-parieto-temporal mass showed progressive neurological deterioration leading to death in 3 months. Another patient with a right parieto-temporal mass had mild neurological symptoms for one and a half months before complete recovery except for a seizure disorder, following steroid and decompressive therapy. The cases show that acute haemorrhagic leucoencephalitis may not always be rapidly fatal, but may run a prolonged clinical course with recovery.

Adult

Rodent model of chemoradiotherapy-induced white matter necrosis.

This report describes a laboratory model that permits study of the radiochemotherapy interactions in the CNS. Rats are stereotaxically implanted with a cerebroventricular cannula attached to an osmotic minipump, which slowly infuses a chemotherapeutic agent into CSF for up to 14 days. The cervical cord is irradiated, and forelimb paralysis develops 4-6 months later at an effective dose for paresis in 50% of the animals; the doses with radiotherapy alone are 2,125 cGy for a single fraction and 2,950 cGy for split fractions. Investigations with the model indicate that mature CNS tissue is not sensitized to either single-fraction or split-dose irradiation with either simultaneous or post-radiation exposure to high concentrations of methotrexate.

Animals