Biomedical subjects
P Demaerel
Publications and source records attributed to P Demaerel.
Acute hydrocephalus in nonketotic hyperglycinemia.
We present four patients with typical neonatal onset non-ketotic hyperglycinemia (NKH) who developed hydrocephalus requiring shunting in early infancy. Brain imaging revealed acute hydrocephalus, a megacisterna magna or posterior fossa cyst, pronounced atrophy of the white matter, and an extremely thin corpus callosum in all. The three older patients had profound developmental disabilities. This suggests that the development of hydrocephalus in NKH is an additional poor prognostic sign.
Direct caroticocavernous fistula and traumatic dissection of the ipsilateral internal carotid artery: endovascular treatment.
After severe craniocerebral trauma a 14-year-old boy developed progressive exophthalmos with venous congestion and chemosis, due to a direct caroticocavernous fistula. Angiography revealed traumatic occlusion of the ipsilateral internal carotid artery and absence of the inferior petrosal sinus. After failure of an approach via the anterior and posterior communicating arteries, the cavernous sinus was successfully catheterised through the occluded internal carotid artery, and embolisation performed with coils.
Quiz case of the month. Progressive lumbar pain irradiating over the right thigh. Diagnosis: schwannoma.
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Symptomatic unilateral dolicho-ectasia of the intracranial arteries in a child.
Dolicho-ectasia of the left posterior cerebral artery and internal carotid artery in a 5-year-old boy is reported. There was compression of the brainstem, the mammillary bodies and the optic tract. The value of MR angiography in the diagnosis of this vascular anomaly is demonstrated. Dolicho-ectasia of more than one cerebral artery in a child is extremely rare.
Septo-optic dysplasia in combination with a pigmented skin lesion: a case report with nosological discussion.
In this case report a patient with bilateral optic nerve hypoplasia, schizencephaly and a pigmented skin lesion is described. The diagnosis of de Morsier syndrome or septo-optic dysplasia is put forward on the basis of the diagnosis of optic nerve hypoplasia. The differential diagnosis with Jadassohn's naevus phakomatosis is discussed. The importance of direct ophthalmoscopy of optic nerve abnormalities is stressed, as well as of magnetic resonance imaging, which has become a guideline in the classification of this syndrome.
Magnetic resonance imaging in psychiatry.
Recent technical advances in MR imaging have led to several new applications in psychiatric imaging. There is a renewed interest in the further elucidation of the etiopathogenesis of some psychiatric disorders and clinical-radiological correlations are increasingly reported. Volume measurements are frequently used and in the near future, functional MR imaging carries high expectations. A brief survey is given of the recent applications of these new techniques in schizophrenia, affective disorders, dementia, anorexia nervosa, and other less common disorders.
Endovascular treatment of a ruptured paraclinoid aneurysm of the carotid syphon achieved using endovascular stent and endosaccular coil placement.
We herein report a case of a ruptured superior hypophyseal aneurysm of the left supraclinoid carotid artery that could not be treated with a Guglielmi detachable coil (GDC), even in combination with a supporting nondetachable balloon. After an unsuccessful attempt at surgical clipping, treatment consisted of the placement of a stent over the neck of the aneurysm, advancement of a microcatheter through the stent mesh, and endosaccular embolization with a GDC. The late clinical outcome was excellent.
Diffusion-weighted MRI in sporadic Creutzfeldt-Jakob disease.
Diffusion-weighted MRI (DWI) was used in three patients with autopsy-proven sporadic Creutzfeldt-Jakob disease (CJD) to provide a rapid noninvasive way to make this sometimes confusing diagnosis. DWI prompted the diagnosis of CJD at an early stage and appears to be particularly useful for monitoring the progression of the disease. We suggest that patients with suspected CJD and no abnormalities on T2- and proton density-weighted images may have cortical involvement on DWI.
Rostral vermian cortical dysplasia: MRI.
The MRI findings in rostral vermian dysplasia are described for the first time. Defective foliation and abnormal fissuration of the rostral vermis can clearly be depicted on coronal images. The abnormalities are limited to the anterior lobe of the vermis and its hemisphere extension. A hypothesis is put forward to explain the abnormalities. It is suggested that the vermian changes result from an intrauterine insult at the end of the first trimester. There appears to be a variable degree of expression and associated cerebellar and cerebral cortical abnormalities can be seen. The clinical significance of these findings remains incompletely understood but may be related to the severity of the abnormalities. It is also suggested that a mild degree of vermian rostral dysplasia may represent an incidental imaging finding.
Sciatica caused by a dilated epidural vein: MR findings.
We report the MR imaging findings in a 41-year-old woman presenting with sudden low back pain and sciatica. At surgery a dilated epidural vein was found compressing the nerve root. The MR findings may suggest the diagnosis. Magnetic resonance imaging of a dilated epidural vein or varix causing sciatica has not been reported until now.
MRI of non-ischemic vascular disease: aneurysms and vascular malformations.
Due to flow-void phenomena, MRI is of great value in the demonstration of cerebral aneurysms and vascular malformations as well as of related parenchymal changes and hemorrhagic complications. Magnetic resonance angiography can produce vascular images which are of importance in the diagnosis and follow-up of the lesions.
Primary brain stem tethering: a rare cause of geniculate neuralgia.
This rare case of brain stem tethering presented with chronic and progressive geniculate neuralgia. In view of the fact that an occipital subcutaneous lipoma had been resected in childhood, it probably concerned a primary tethering, fitting in with an occult occipital dysraphism. Magnetic resonance imaging (MRI) clearly demonstrated an underlying tethering, causing a distortion of the brain stem. Consequently, this led to the hypothesis that the geniculate neuralgia could be explained by traction on the lower cranial nerves secondary to the brain stem displacement. Untethering resulted in a considerable decrease of the neuralgia. MRI proved to be essential in the diagnosis and treatment of this unusual case.
Retinitis pigmentosa in association with acromegaly: a case report.
Retinal abnormalities, unrelated to visual pathway compression, in acromegalic patients were originally described by Small in 1972 (Smail JM. Primary pigmentary degeneration of the retina and acromegaly in a case of pituitary adenoma. Br J Ophthalmol 1972; 56: 25-31). He illustrated a case of primary pigmentary degeneration of the retina occurring in a patient with a chromophobe adenoma of the pituitary gland. To the best of our knowledge this remains the sole, published case of this association. We report on two male patients, with an acromegalic appearance, one caused by a pituitary adenoma, the other associated with a Rathke's cleft cyst, presenting to our department of ophthalmology with the clinical picture of pigmentary degeneration of the retina.
Automatic 3-D segmentation of internal structures of the head in MR images using a combination of similarity and free-form transformations: Part I, Methodology and validation on normal subjects.
The study presented in this paper tests the hypothesis that the combination of a global similarity transformation and local free-form deformations can be used for the accurate segmentation of internal structures in MR images of the brain. To quantitatively evaluate our approach, the entire brain, the cerebellum, and the head of the caudate have been segmented manually by two raters on one of the volumes (the reference volume) and mapped back onto all the other volumes, using the computed transformations. The contours so obtained have been compared to contours drawn manually around the structures of interest in each individual brain. Manual delineation was performed twice by the same two raters to test inter- and intrarater variability. For the brain and the cerebellum, results indicate that for each rater, contours obtained manually and contours obtained automatically by deforming his own atlas are virtually indistinguishable. Furthermore, contours obtained manually by one rater and contours obtained automatically by deforming this rater's own atlas are more similar than contours obtained manually by two raters. For the caudate, manual intra- and interrater similarity indexes remain slightly better than manual versus automatic indexes, mainly because of the spatial resolution of the images used in this study. Qualitative results also suggest that this method can be used for the segmentation of more complex structures, such as the hippocampus.
Meningoradiculitis due to borreliosis presenting as low back pain only.
We report a child with Borrelia burgdorferi meningoradiculitis. This entity, also known as Bannwarth syndrome, is rare and its presentation with low back pain only is even more unusual. The MRI findings can suggest the diagnosis.
Fast FLAIR MRI in childhood white-matter abnormalities.
We compared a fast fluid-attenuated inversion recovery (FLAIR) pulse sequence with a dual-echo short tau fast inversion-recovery (DESTTIR) sequence in 20 children with white matter abnormalities. Although the overall image quality of DESTTIR images was better, the lesion-to-background contrast was significantly higher with the fast FLAIR pulse sequence and lesion detection was more accurate.
Primary multifocal leptomeningeal gliomatosis.
Only nine cases of primary multifocal leptomeningeal gliomatosis (PMLG) have been reported. We describe the first child with malignant features of PMLG who was diagnosed during life. The differential diagnosis based on imaging findings includes infectious and lymphoproliferative disorders.