RESUMO
A non-Jewish child had a congenital sensory and autonomic neuropathy, cerebral hypoplasia, and a posterior interhemispheric cyst. The clinical findings, neuropathologic changes in the spinal cord, the peripheral nerves, the sensory and autonomic ganglia, as well as sural nerve morphometry, showed similarities, but also differences from familial dysautonomia and hereditary sensory neuropathy, type IV. The relationship of this case to hereditary sensory neuropathies and dysautonomias is discussed, and it is suggested that this is an atypical form of familial dysautonomia with coincidental developmental abnormalities.
Assuntos
Disautonomia Familiar/patologia , Encefalopatias/complicações , Encefalopatias/patologia , Cistos/complicações , Cistos/patologia , Disautonomia Familiar/complicações , Feminino , Neuropatias Hereditárias Sensoriais e Autônomas/complicações , Neuropatias Hereditárias Sensoriais e Autônomas/patologia , Humanos , Recém-NascidoRESUMO
The ISIS Center at Georgetown University Medical Center has developed a comprehensive program for image-guided procedures in the spine. As part of this program, ISIS has developed a software application known as I-SPINE (ISIS's Spine Procedure Imaging Navigation Engine). I-SPINE is a Windows NT application, which is based on the Analyze/AVM libraries. The software architecture follows the Microsoft Foundation Classes (MFC) single document, multiple view paradigm. This has allowed the developers to add new visualization modules to I-SPINE that aid physicians in procedures outside the spine. One such procedure I-SPINE has been expanded for is uterine fibroid embolization. The idea is that by registering and subtracting post-embolization angiographic images from pre-treatment images the resulting image can be used to quantify the embolization effect on the fibroid circulation and predict the treatment response. The I-SPINE digital subtraction angiography (DSA) module allows the interventional radiologist to open a series of pre and post-embolization DSA images that shows the vascular structures of the uterus and the fibroid or fibroids. From these images, the radiologist selects an appropriate image from each series. The selected images are then hand registered using pixel shifting. Once the images are registered, the pixels are subtracted resulting in an image that shows the embolized arteries that were supplying the fibroids.