Ph. Demaerel

Desmoplastic Infantile Ganglioglioma A Potentially Malignant Tumor

Desmoplastic infantile ganglioglioma is a rare intracranial tumor of early childhood with a usually excellent prognosis despite malignant features both radiologically and histologically. We present the case of a desmoplastic infantile ganglioglioma with histologically highly anaplastic features and both intracerebral and pial metastases. After partial resection the tumor was rapidly progressive and new metastases appeared. A combination of vincristine and carboplatinum was used according to the Low Grade Glioma Protocol of the International Society of Pediatric Oncology, with a temporary good response. When histologically characterized by highly anaplastic features, it seems the biologic behavior of this tumor remains uncertain. The aggressive behavior and the responsiveness to chemotherapy in this case may challenge the belief in the benign nature of these rare tumors.

Diffusion-weighted MR imaging findings in a patient with herpes simplex encephalitis

INTRODUCTION Herpes simplex meningoencephalitis is one of the most common viral central nervous system infection in adults. Early diagnosis is essential for treatment. CASE REPORT We present a case of a 68-year-old female patient with herpes simplex infection. On admission, she was in severe clinical condition. Diffusion-weighted (DW) magnetic resonance imaging detected brain involvement better than conventional sequences. After acyclovir therapy, the patient fully recovered. CONCLUSION DW magnetic resonance imaging is expected to provide a more sensitive imaging in herpes simplex patients than conventional sequences.

Lhermitte-Duclos disease is a clinical manifestation of Cowden's syndrome.

BACKGROUND Lhermitte-Duclos disease (LDD) is a hamartomatous overgrowth of cerebellar ganglion cells, which replace granular cells and Purkinje cells. In recent years several cases involving the association between LDD and Cowdens syndrome (CS), an autosomal dominant condition characterized by multiple hamartomas and neoplastic lesions in skin and internal organs, have been reported. METHODS We reviewed the medical records and imaging studies of six patients with LDD who were treated at our institution, and we looked at other possible symptoms of CS. RESULTS Other clinical findings suggestive of CS were apparent in five patients: These included mucocutaneous lesions, acral keratosis, thyroid adenoma, fibrocystic disease, ovarian cyst, intestinal polyposis, and arteriovenous malformation. Only in the youngest patient, a 5-year-old boy, were no cutaneous or other signs found, despite extensive clinical and ultrasound examination. CONCLUSION Our observations strengthen the hypothesis that LDD is a neurological manifestation of CS. Patients with LDD should receive a thorough dermatological and systemic screening, because some of the lesions (breast, etc...) can develop into malignant tumors.

Interhemispheric lipoma with variable callosal dysgenesis: relationship between embryology, morphology, and symptomatology.

Eight interhemispheric lipomas (five tubulonodular lipomas and three curvilinear lipomas) were examined by magnetic resonance imaging (MRI). The purpose was to further investigate the relationship between the morphology of the different subtypes and the clinical presentation. The imaging findings were reviewed in light of a recent theory on the development of the corpus callosum. Interhemispheric lipomas should be considered as one entity with a variable expression depending on the severity and/or the time of the insult. Curvilinear lipomas can be either small or extensive and are usually not symptomatic. Tubulonodular lipomas can be either predominantly anterior or posterior in location. The anterior subtype appears to be a more severe form of tubulonodular lipoma. The associated structural abnormalities are most likely responsible for the symptoms, rather than the lipoma itself. Magnetic resonance imaging allows a more precise timing of the insult, resulting in the development of a lipoma. The knowledge of the embryology between the 6th and the 20 h week is important to explain these abnormalities. Until now it has been accepted that the corpus callosum develops in an orderly fashion. A recent theory has demonstrated that this is not necessarily true, and that fibers can cross the midline at any place irrespective of the normal development. This theory explains the sometimes amorphous appearance of the remnant of the corpus callosum if a lipoma is present.

Intra-axial brain tumours

The radiological diagnosis and differential diagnosis of intra-axial tumours no longer relies on CT scan and routine MR sequences alone. Standard multiplanar imaging has to be combined with fMRI to allow the exact anatomic location of the lesion and precise determination of the extension of the tumour. Perfusion and diffusion MR is becoming more and more important in the differential diagnosis of cerebral mass lesions and in the grading and typing of gliomas. More sophisticated techniques such as diffusion tensor imaging and spectroscopy will further enhance the value of the radiological studies.

MRI of the pathological female pelvis

The current status of the use of MRI in the diagnosis and staging of pelvic masses is reviewed, emphasizing the positive and negative aspects of this imaging technique.

Brain MR Imaging in dietarily treated phenylketonuria

Magnetic resonance imaging is the most efficient imaging modality to evaluate brain gray and white matter of patients with metabolic diseases [1, 2, 3]. The main purpose of out study was to investigate the relation between brain MRI abnormalities and the phenylalanine (phe) and tyrosine (tyr) blood levels in 38 phenylketonuria (PKU) patients. Increased periventricular white matter intensity on T2-weighted brain images was the only pahtologic finding in 24 patients. Brain MRI abnormalities were scored (4) and correlated with the individual mean phe and phe/ tyr levels during 1 year preceding MR examination and with phe tolerance. The appearance of MRI abnormalities on brain T2-weighted images correlates with a threshold mean phe level (averaged over the year preceding the examination).

MR Imaging of Lhermitte-Duclos disease: report of two cases

Two cases of Lhermitte-Duclos of the cerebellum are presented. MRI was essential in the radiological diagnosis of both lesions by the demonstration, both on T1- and T2 weighted images of thickened cerebellar folia.

MRI in spinal lumbosacral dysraphism

The value of magnetic resonance imaging (MRI) in lumbosacral spinal dysraphism is reviewed. Althoigh some technical problems still remain to be overcome, it is obvious tha MRI is the examination of choice for the preoperative assessment of these congenital disesses.

Abstracts of papers for full membership resumes des travaux de titulariat samenvatting van de titulariaatswerken

Purpose:To document the brain imaging findings in leukemia. These include manifestations of primary disease, complications of therapy, and infection due to immune suppression.