No abstract available.
Electroencephalography* ; Humans ; Neurologic Manifestations* ; Seizures*

Miller-Dieter syndrome consists of severe type I lissencephaly, abnormal facial appearance, and sometimes other birth defects. Lissencephaly is a brain malformation manifested by a smooth cerebral surface, thickened cortical mantle, and microscopic evidence of incomplete neuronal migration. It comprises the agyria-pachygyria spectrum of malformation, thus excluding polymicrogyria and other cortical dysplasia. Type I lissencephaly results from abnormal migration between about 10 and 14 weeks gestaion. The brain is often small, and the ventricle is enlarged posteriorly The corpus callosum may be small or absent. The structural pattern of the cerebral hemispheres and ventricles is distintly immature, reminiscent of fetal brain. The superficial cellular layer resembles an immature cortex, with some separation into zones similar to layers III, V, and VI of normal cortex, although the cell population is decreased. In 1963 Miller described a malformation syndrome in a brother and sister with postnatal growth deficiency, craniofacial defects, and serious abnormalities of neurologic function. Autopsy at 3 and 4month of age, respectively, revealed lissencephaly. Subsequently, Dieker reported four additional patients with this disorder and referred to it as the 'lissencephaly syndrome'. We have experienced a case with this syndrome. Then we report this rare case with brief review of literature.
Autopsy ; Brain ; Cerebrum ; Congenital Abnormalities ; Corpus Callosum ; Humans ; Lissencephaly ; Malformations of Cortical Development ; Neurons ; Siblings

No abstract available.
Cachexia* ; Ductus Arteriosus, Patent* ; Heart Failure* ; Heart* ; Humans ; Infant*

No abstract available.
Humans ; Microcomputers*

No abstract available.
Hypopituitarism* ; Magnetic Resonance Imaging ; Pituitary Gland*

No abstract available.
Epinephrine* ; Heart Arrest*

PURPOSE: Although the pudendal nerve terminal motor latency (PNTML) is normally used, there is no definite test that accurately reflects the function of the pudendal nerve. This study was undertaken to determine the relative accuracy of the various methods in measuring the function of the pudendal nerve. METHODS: Thirty one female patients (age 51.3+/-15.7) with a defecation disorder (constipation 20, fecal incontinence 11) were evaluated prospectively using a neurophysiologic test and balloon reflex manometry. Five parameters such as the right and left PNTML, anal mucosal electrosensitivity, latency and the amplitude of the rectoanal contractile reflex (RACR) were analyzed statistically for their correlation. RESULTS: There was no significant inter-test correlation among the parameters. However, the intra-test correlations between the parameters such as the right and left PNTML (r=0.9629, P<0.001)/latency and the RACR amplitude (r= -0.3770, P=0.0366) were found to be significant. CONCLUSION: The accuracy of these tests in evaluating the pudendal neuropathy could not be determined. However, because it can be assumed that a measurement of the RACR in addition to RNTML is technically accurate, it there will need to be more study for it to be used as an alternative to a PNTML measurement.
Defecation ; Fecal Incontinence ; Female ; Humans ; Manometry ; Prospective Studies ; Pudendal Nerve* ; Pudendal Neuralgia ; Reflex

The rectum is a unique visceral organ, of which afferents are not so obvious. In anorectal surgery ablating the rectum and/or perirectal structure, this issue comes with significant meaning about whether to preserve patient's normal defecatory function, or not. So we planned this study to evaluate which nervous system concerns the afferents from the rectum. METHODS: We recorded cerebral evoked potential (EPs) in 16 healthy male subjects after electrical and mechanical stimulation of the rectum, and compared their waving patterns regarding latencies and amplitudes of each peak with those occuring after electrical stimulation of the pudendal nerve. RESULTS: The EPs after electrical stimulation of the rectum showed distinctly different waving patterns in comparison to those after electrical stimulation of the pudendal nerve. But the EPs after mechanical stimulation of the rectum showed very similar waving patterns with those after electrical stimulation of the pudendal nerve. CONCLUSIONS: Rectal afferents of mechanical stimulation seem to be somatosensory, but those of electrical stimulation seem visceral. In that sense, sensory receptors of mechanical stimulation may lie in the perirectal structure, such as pelvic floor muscle and those of electrical stimulation lie in the rectum, itself.
Electric Stimulation ; Evoked Potentials* ; Humans ; Male ; Nervous System ; Pelvic Floor ; Pudendal Nerve ; Rectum ; Sensory Receptor Cells

No abstract available.
Emergencies* ; Emergency Service, Hospital*

The purpose of this study is to evaluate the diagnostic accuracy of various quantitative indices for the differentiation of benign from malignant primary soft tissue tumors by FDG-PET. A series of 32 patients with a variety of histologically or clinically confirmed benign (20) or malignant (12) soft tissue lesions were evaluated with emission whole body (5min/bed position) PET after injection of [18F]FDG. Regional 20min transmission scan for the attenuation correction and calculation of SUV was performed in 16 patients (10 benign, 6malignant) followed by dynamic acquisition for 56min. Postinjection transmission scan for the attenuation correction and calculation of SUV was executed in the other 16 patients (10 benign, 6 malignant). The following indices were obtained : the peak and average SUV (pSUV, aSUV) of lesions, tumor-to-background ratio acquired at images of 51 min p.i. (TBR51), tumor-to-background ratio of areas under time-activity curves (TBRarea) and the ratio between the activities of tumor ROI at 51 min p.i. and at the time which background ROI reaches maximum activity on the time-activity curves (T51/Tmax). The pSUV, aSUV, TBR51, and TBRarea, in malignant lesions were significantly higher than those in benign lesions. We set the cut-off values of pSUV, aSUV, TBR51, TBRarea and T51/Tmax for the differentiation of benign and malignant lesions at 3.5, 2.8, 5.1, 4.3 and 1.55, respectively. The sensitivity, specificity and accuracy were 91.7%, 80.0%, 84.4% by pSUV and aSUV, 83.3%, 85.0%, 84.4% by TBR51, 83.3%, 100%, 93.8% by TBRarea and 66.7%, 70.0%, 68.8% by Tsl/Tmax. The time-activity curves did not give additional information compared to SUV or TBR. The one false negative was a case with low-grade fibrosarcoma and all four false positives were cases with inflammatory change on histology. The visual analysis of FDG-PET also detected the metastatic lesions in malignant cases with comparable accuracy. In conclusion, all pSUV, aSUV, TBR51, and TBRarea are useful metabolic semi-quantitative indices with good accuracy for the differentiation of benign from malignant soft-tissue lesions.
Fibrosarcoma ; Humans ; Positron-Emission Tomography ; Sensitivity and Specificity