Atypical Presentation of Sjögren-Larsson Syndrome.

Sjögren-Larsson syndrome is a rare neurocutaneous disorder characterized by ichthyosis, spastic diplegia or tetraplegia, and intellectual disability. Herein, we describe a case of a Greek patient with ichthyosis and spasticity of the legs but with normal intelligence (IQ 95). This syndrome should be suspected when a child presents with ichthyosis and spastic diplegia or tetraplegia, even if intelligence is normal.

Spinal segmental dysgenesis: presentation of a rare spinal congenital abnormality.

Segmental spinal dysgenesis is a rare spinal congenital abnormality in which a segment of the spine and spinal cord fails to develop completely. This entity is often associated with other congenital anomalies such as multiple vertebral segmentation defects, congenital vertebral displacement, tethered cord, hydromyelia and syringohydromyelia.

3D MR Models and Volumetric Measurements of the Brain in Patients with Alzheimer's Disease.

This study aimed to describe the procedure we use to create 3D models of the brain parenchyma from MRI images and calculate the volume of the whole brain and different compartments of the brain in patients with Alzheimer's disease. The utility of the 3D models and volumetric measurements of the whole brain parenchyma and different brain structures is discussed. Thirty-six patients with Alzheimer's disease were examined during the last six months with MRI. Fourteen of them were men and 22 were women. The patients were between 53 and 67 years old. MR images were studied using an automatic algorithm. The images from MRI were segmented and then three-dimensional models of brain were produced to calculate the brain volume and the volume of the white matter, gray matter and CSF separately. The whole procedure was completed successfully in 34 patients. The procedure was unsuccessful in two patients due to movement artifacts in MR images. It is relatively easy to create 3D models of MR images and to obtain volumetric studies. If this procedure is adjusted in patients with Alzheimer's disease, we can provide information more clearly and accurately than single images alone. The information obtained can be used in daily clinical practice such as pharmaceutical treatment planning and results or in basic clinical research.

MRI evaluation of diastematomyelia and associated abnormalities.

Fourteen children were studied with a 1Tesla scanner to assess the role of magnetic resonance (MR) imaging in the recognition and evaluation of diastematomyelia and associated abnormalities. With MRI divided spinal cord was well imaged in its entire craniocaudal extent. Fibrous bands or bony septum when it contained a marrow cavity, were also well depicted. Direct visualization of the spinal cord itself is probably the most rewarding feature of MR imaging compared with other more conventional techniques. MR imaging demonstrated associated syringohydromyelia, segmentation anomalies, scoliosis, low position of the conus medularis and intradural lipoma that they were not detected with other radiologic studies.

Falx Cerebri Ossification: CT and MRI Evaluation.

During the last three years, CT and MRI brain scans of 40 patients revealed falx cerebri partial ossification as an incidental finding. The patients had been admitted for brain CT and MRI for several reasons. In most cases, there was no problem in the differential diagnosis of falx cerebri ossification during interpretation of the cases. In a few cases, the lesion should be distinguished from calcified meningioma, small hematoma in the interhemispheric fissure and in one case there was also meningeal infiltration of breast cancer. In these cases both CT and MRI scans of the brain were evaluated and a definite diagnosis was made.