Biomedical subjects
Publications and source records attributed to J H Sher.
This case report is a neuropathological study of a ten-month-old infant with unilateral megalencephaly . In this anomaly neuronal migration defect and disturbances of cortical organization resulting in micropolygyria were the most striking neuropathological feature.
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The present study is a review of four new cases of lissencephaly and two others previously reported. This study demonstrates that lissencephaly is a gross feature of the brain occurring in two different groups of cortical malformations. The first group, the classic agyria syndrome extensively analyzed by Jellinger and Rett [8] includes two types of abnormal cortical organization. They may be found in familial syndromes and also can appear sporadically. The second group includes smooth brains with the internal features of polymicrogyria and a more severely disorganized cortex. This type appears in familial lissencephaly in the cerebro-oculo-muscular syndrome, belonging to the same group as Fukuyama congenital-cerebro-muscular dystrophy. The other incidences of this type of cortical malformation require further investigation. The clinico-pathological differential diagnosis of two types of lissencephaly are also discussed.
Five patients with the clinical diagnosis of fetal alcohol syndrome (FAS) died at the ages of 8 and 4 months and 17, 4 and 2 days. Neuropathological examination revealed microencephalic brains in all cases, without morphological evidence of maturation delay. One of them showed agenesis of the corpus callosum and hypoplasia of the cerebellar vermis. Five of them had only small dysgenetic changes, consisting mainly of glio or glioneuronal meningeal or parenchymal heterotopias. Our findings indicate that the brain is commonly but not affected in FAS. The influence of alcohol and its metabolites, as well as undernutrition, and use of other drugs by the mothers, should be taken into account as possible etiologic factors.
This paper describes a man with previously undetected prostatic carcinoma who presented with mild symptoms due to an orbital metastasis. The case is unusual in that the patient was relatively asymptomatic and had 6/7 vision. Carcinoma of the prostate metastatic to the orbit is rare; this is only the 22nd case in the literature.
The suitability and effectiveness of hydrophilic soft contact lenses and donor corneal buttons for interlamellar refractive keratoplasty were evaluated in a study with rabbits. In each experimental eye a trephined button of Bausch & Lomb Soflens (polymacon), Cooper Permalens (perfilcon A) or rabbit cornea 6.5 mm in diameter was inserted in a corneal pocket created with a lamellar dissector. Among the inserts available for long-term follow-up all five of the Soflens inserts showed "melting" (perforation and sloughing) by 3 weeks, whereas six of the seven Permalens inserts and all four of the donor corneal inserts were clear at 3 to 6 months. Myopia was induced and the corneal curvature steepened with all but the high-minus Soflens inserts. Thus, interlamellar refractive keratoplasty with soft contact lenses of high water content or lathe-cut donor corneal buttons has potential for the correction of aphakia in rabbits and may well have similar potential in humans.
Mice with genetic muscular dystrophy were treated with intraperitoneal injections of the proteinase inhibitor leupeptin, beginning before the onset of weakness. A significant number of the treated animals failed to develop histological evidence of dystrophy, compared with controls. Leupeptin treatment prevented (or delayed) the onset of muscular dystrophy in this experiment.
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An acute reversible myopathy characterized by extensive lysis of myosin occurred in a patient who suffered from severe shock, hypoxia, and acidosis. This new clinical pathologic entity illustrates an unusual structural change in muscle associated with these catastrophic clinical circumstances.
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A child treated for a desmoplastic medulloblastoma of the left cerebellar hemisphere at the age of 10 months developed a malignant astrocytoma in the same site 11 years later. Theories of origin of the second tumor, particularly in relation to concepts of the genesis of medulloblastoma in general, are discussed.
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A case of diencephalic-midbrain hemorrhage presenting with excessive thirst, coma, and death is discussed. Some diencephalic dysfunction syndromes are reviewed, as well as their physiological basis. Thirst and drinking behavior can provide a valuable localizing symptom and sign in clinical-pathological correlations.
The brains of ten narcotic addicts who had died from an overdose of methadone, and the brains of ten rats, given methadone for one month, were examined by the immunofluorescent technic. Positive neuronal fluorescence was seen primarily in the limbic systems of both species as well as in closely associated areas. In the human, Purkinje cell fluorescence was seen in the cerebellum, whereas only stellate cell staining was observed in rat cerebellum. Neurons of the hippocampal denate gyrus often fluoresced in man while only pyramidal cell staining was seen in this region of rat brain. The method should prove to be of value in the detection and tracing of narcotic drugs and may be helpful in investigations of drug tolerance and dependence.
The authors report a patient with sickle cell anemia who suffered from paraplegia of 18 months duration due to spinal cord compression by a hemopoietic mass. Recovery following removal of the mass was complete.
Specific neuronal staining for methadone was demonstrated by immunofluorescence in the brain tissue of ten addicts who had died from drug overdose. Areas of the brain most frequently involved were the hypothalamus, thalamus, hippocampus, amygdala, cerebellum and brain stem. Toxicological studies confirmed the presence of methadone in each case.