Agyria-pachygyria: clinical,neuroimaging, and neurophysiologic correlations

Agyria-pachygyria complex is a disorder of neuronal migration and organization. Patients suffer either motor or intellectual retardation. We report our experiences of 10 patients with agyria-pachygyria complex and evaluate their clinical features, electroencephalography, and evoked potentials. Of nine electroencephalography examinations, five patients demonstrated characteristically high-amplitude fast activity. One of nine patients had an abnormal brainstem auditory-evoked potential. Three of seven patients had abnormal goggled visual-evoked potential. Six patients received somatosensory-evoked potential examinations, and five of these were abnormal, including four with prolonged central conduction times. Of the 10 patients, eight survived with variable intellectual and motor retardation; two died of sepsis. Patients with grades 1-4 agyria-pachygyria had high incidences of somatosensory-evoked potential abnormalities and also suffered worse neurologic outcomes. Normal brainstem auditory-evoked potential but abnormal cortical somatosensory-evoked potential components and prolonged central conduction time in these patients indicate that agyria-pachygyria is a supratentorial disease. We conclude that somatosensory-evoked potential examination is supplemental to neuroimaging in predicting the neurologic prognosis of patients with agyria-pachygyria.     

High resolution computed tomography of the temporal bone in infants and children: a review

Pathologic conditions involving the temporal bone in infants and children are now commonly diagnosed by computed tomography (CT). The importance of utilizing high resolution computed tomography (HRCT) with magnified, thin (1.5 mm) scans in both the axial and coronal planes is stressed in order to obtain the maximal anatomic information that is critical for proper diagnosis. A retrospective analysis of 388 cases reveals a positive diagnostic yield ranging from 65% in temporal bone trauma to 20% in clinically suspected neoplasm. Selected case reports of congenital abnormalities, inflammatory processes, traumatic lesions, and tumors will be presented. We conclude that HRCT is an excellent diagnostic modality and should be used liberally to evaluate the temporal bone in children.     

Pregnancy rates with fresh versus computer-controlled cryopreserved semen for artificial insemination by donor in a private practice setting

From late 1970 through December 1987, 580 women began 733 treatment programs (a total of 3405 insemination cycles) with fresh semen for artificial insemination by donor. The unadjusted pregnancy rate was 48.5% and the cumulative pregnancy rate after seven cycles by life-table analysis was 59.6% with a monthly fecundability of 11.9%. From 1988 to the present, 113 women have begun 115 treatment programs (a total of 371 insemination cycles) with cryopreserved semen for artificial insemination by donor. The unadjusted pregnancy rate to date is 18.3% and the cumulative pregnancy rate after seven cycles by life-table analysis is 48.6% with a monthly fecundability of 5.9%. This report shows that using cryopreserved semen for artificial insemination by donor will take somewhat longer to produce a pregnancy but the ultimate pregnancy rate will be similar to that with fresh semen for artificial insemination by donor.     

Etiologic mechanisms of hematopoietic failure

Our understanding of the pathophysiology of aplastic anemia has lagged behind advances in treatment of the disease. The data available indicate that the heterogeneous assortment of drugs, as well as the array of chemical, physical, and infectious agents that are clinically associated with aplastic anemia, probably exert their action through restricted pathways. Based on certain clinical features of aplastic anemia, animal models of hematopoietic failure, and considerations of the organization of the hematopoietic system, we propose two general types of aplastic anemia as a conceptual framework for further studies: type I aplastic anemia, representing a congenital or acquired stem cell defect that can only be cured by bone marrow transplantation, and type II aplastic anemia, which is caused by reversible suppression of normal stem cells by external agents and is amenable to immunosuppressive therapy.     

The chromosomal complements of cleaved human embryos resulting from in vitro fertilization

Chromosome preparations were made from 25 cleaved abnormal human embryos at the two-to eight-cell stage after in vitro fertilization. Morphologically, these embryos showed either variable degrees of degeneration or an abnormal number of pronuclei before first cleavage. Among 14 successfully karyotyped embryos, only 3 had a normal chromosomal complement. Eleven showed chromosomal abnormalities, including triploidy, hapioidy, and mosaicism. This finding documents a high incldence of chromosomal errors in morphologically abnormal early preimplantation embryos.     

Prevention of neonatal macrosomia in gestational diabetes by the use of intensive dietary therapy and home glucose monitoring

This study was undertaken to determine if intensive dietary therapy, home blood glucose monitoring, and the selective use of insulin can be effective in preventing fetal macrosomia. All patients were screened at 24 to 28 weeks' gestation using a modification of O'Sullivan's criteria. The 153 patients diagnosed as gestational diabetics by the study protocol were placed on a 1800 to 2000 Kcal American Diabetes Association diet and taught home glucose monitoring. Insulin therapy was initiated only if blood glucose control was inadequate. There were no significant differences (p greater than 0.05) between the study and reference populations in regard to mean birthweight or the incidence of macrosomia. Since our study criteria for diagnosing gestational diabetes were slightly different from those of the National Diabetes Data Group (NDDG), data from 99 patients meeting the NDDG criteria were analyzed in a similar manner. No significant differences were found between this subgroup and the reference population. Since only 7.2% of our study patients required insulin, we conclude that the incidence of fetal macrosomia in gestational diabetes can be kept equal to that of the general population by a program of intensive dietary therapy and home glucose monitoring, with insulin being used only therapeutically, not prophylactically.     

Microscopic characteristics of round window problems in otology

Round window membrane ruptures and perilymph leakage can present the complication or concomitant of sudden deafness and barotrauma. Pathological involvement of the round window membrane can occur in many otological diseases such as otosclerosis, otitis media, tumors, etc. Microscopic manipulations might result in an inadvertent round window membrane lesion and cochlear deafness. In recent years purposeful surgical lesions, including procedures for vertigo and cochlear implant surgery, are being made in the round window membrane. Examples of pathology involving the round window in human temporal bones are described. To understand better the clinical features of early round window rupture in humans, controlled punctuate lesions were made in 36 chinchilla ears and studied sequentially. Round window membrane healing occurred as early as 3 days post-lesion, and was complete in all ears but 1 of the combined 7, 9 and 11-day group. Healing occurred primarily as a result of middle ear epithelial ingrowth and mesothelial cell reaction in the adjacent perilymphatic space of the scala tympani. These cellular events are described and discussed from an histological and clinical point of view.