BACKGROUND AND PURPOSE:Wernicke encephalopathy is a severe neurologic disorder that results from a dietary vitamin B1 deficiency. It is characterized by changes in consciousness, ocular abnormalities, and ataxia. This study was undertaken to analyze and compare findings on MR imaging and neurologic symptoms at clinical presentations of patients with Wernicke encephalopathy with and without a history of alcohol abuse.
Mucopolysaccharidoses (MPSs) are a group of inherited disorders due to lysosomal enzyme deficiencies which lead to multi-organ accumulation of glycosaminoglycans. Some forms of MPS disorders are characterized by various degrees of mental retardation. Magnetic Resonance Imaging (MRI) is the primary imaging technique to detect CNS alterations. The aim of this study is to evaluate the correlation between white matter (WM) alterations and the presence of mental retardation. We analyzed 20 patients with different forms of MPSs, 11 with mental retardation and 9 with a normal cognitive function; all of them underwent brain MRI and received a score on the basis of the alterations (WM alterations; perivascular, subarachnoid, and ventricular space enlargement; abnormalities of the basal ganglia, of the corpus callosum and of the atlanto-axial joint). All patients with mental retardation presented severe WM alterations, while only five out of the nine subjects without mental retardation showed certainly WM abnormalities. As far as the other cerebral abnormalities are concerned, no difference between the two groups has been found. These data seem to show that there is a significant correlation between the presence of WM alterations and mental retardation.
Multiple glioma is a well-recognized but uncommon entity. They are grouped in two categories: multifocal and multicentric gliomas. Multifocal gliomas grow through dissemination along an established route, spreading through commissural pathways, CSF channels, or the blood or by local extension through satellite formation; at the opposite end of the spectrum, multicentric gliomas are widely separated lesions whose simultaneous presence cannot be attributed to any of the above pathways. Reports in the literature refer to single cases or small series of multicentric gliomas, almost always in adult patients, their occurrence in children being even less frequent. We report the case of a 12-year-old boy with multicentric glioma, atypical acute clinical onset and fast growth of three other tumors in 8 months, and then discuss the problems of diagnosis and therapy.
Caudal brain displacement is inconstantly reported as an MRI feature of spontaneous intracranial hypotension (SIH). We reviewed the clinical data and MRI of eight patients diagnosed as having SIH and investigated the possibility of more precise assessment. On midsagittal images we measured four anatomical landmarks: the position of the cerebellar tonsils, fourth ventricle, and infundibular recess, plus the angle between the bicommissural line and a line tangential to the floor of the fourth ventricle; midsagittal images from 89 normal controls were also measured. On statistical analysis, all measurements differed in the two groups, and the difference was significant for the cerebellar tonsils, fourth ventricle, and infundibular recess. Some overlap between patients and controls was found for each measurement; however, all the patients had two (two patients) or more (six) values outside the range in normal controls range or not above their 1st quartile. Measurement of the position of the third ventricle seemed particularly sensitive. We suggest that examination of midsagittal images can help in diagnosing clinically suspected SIH.
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