Biomedical subjects
Publications and source records attributed to A Beltramello.
Chondrosarcomas are extremely rare intracranial cartilaginous tumors of which the myxoid variant is the least reported in the literature. They develop extradurally and generally infiltrate the dura only in advanced stages or at recurrence. We describe the case of a 55-year-old woman with a posterior cranial fossa myxoid chondrosarcoma which had a primarily intradural extension.
INTRODUCTION: Neuroimaging and experimental studies have related cytomegalovirus (CMV) to certain neuronal migration disorders. MATERIAL AND METHODS: To define the electroclinical picture of children with epilepsy associated with disorders of cortical development (DCD) and congenital CMV infection, we conducted a clinical, electroencephalographic and neuroradiological study of 10 children with this condition. RESULTS: Eighty per cent of them had dismorphic traits, or malformations outside CNS. All showed other neuroradiological signs (cerebral calcification, white matter damage, porencephaly). Six patients with bihemispheric DCD (agyria-pachigyria, 2; 'poligyria', 1; schizencephaly, 1; bilateral opercular DCD, 2) showed: Tetraparesis, severe or profound mental deficiency, early onset epilepsy (mean age at onset: 11 months) with spasms, tonic seizures, partial seizures, and multifocal paroxysms or unusual diffuse sharp Alfa-Beta EEG activity. One child developed Epilepsia Partialis Continua. Children with bilateral opercular DCD evolved to a continuous spike and wave (SW) electrical status during wakefulness and sleep, linked to a worsening of psychomotor derangement. Four patients with unilateral hemispheric DCD (pachigyric or 'poligyric') showed: Congenital hemiparesis, mild intellectual deficiency, motor seizures (orofacial, hemiclonic, generalized) beginning in the third year of live, atypical absences with focal phenomena, frequent focal rhythmic SW discharges during wakefulness, and continuous SW status during sleep (CSWS). CONCLUSIONS: A wide spectrum of DCD due to congenital CMV infection is documented. Characteristic electroclinical pictures related to the extent and topographical distribution of the DCD are recognized, which may lead to an appropriate diagnosis and prognosis.
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A notchlike bone defect in the basiocciput due to a prominent fossa navicularis was incidentally discovered in a patient referred for radiologic evaluation of sinusitis. MR images showed that the osseous defect was filled with lymphoid tissue of the pharyngeal tonsil. The occurrence of this anatomic variant is discussed, with reference to ancient anatomic works.
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Eclampsia is a rare condition peculiar to pregnant and puerperal women, characterized by clinical pre-eclampsia (hypertension, proteinuria, edema) and generalized seizures. Three cases of eclamptic encephalopathy are reported: CT and MRI demonstrated transient abnormalities in the cortical and subcortical regions of the posterior areas of the brain - namely, parieto-occipital lobes - associated with occasional involvement of basal ganglia and/or brainstem. Pathogenesis is still unclear. Strict similarity with the pathological findings characterizing hypertensive encephalopathy suggests that a focal impairment in cerebral autoregulation may be the cause of vasodilation and fluid extravasation leading to hydrostatic edema; selective involvement of posterior areas could be explained by their lesser degree of adrenergic innervation supporting circulatory autoregulation mechanisms.
Krabbe's disease with adult onset is rare; neurological symptoms begin in childhood or at a juvenile age. Two brothers with adult onset of the disease are here reported; 1 sibling developed parapareto-ataxic gait while the other was asymptomatic. Magnetic resonance imaging showed areas of demyelination in the white matter of the brain, while nerve conduction was completely normal. In both patients deficiency of galactosylceramide beta-galactosidase was comparable to the infantile form.
Three patients with a severe somatosensory deficit consequent on damage of the right somatosensory cortices were required, while blindfolded, to point with their insensate thumb to select positions on the other left fingers. Given the absence of feedback, the motor performance of the insensate thumb appeared grossly impaired in all patients. However, all patients attained end-points with an accuracy greater than chance. This result suggests that spatial accuracy may not rely entirely on sensory feedback. A good accuracy of pointing was evinced also in potentially facilitating conditions where somatosensory and motor cues coming from the intact side during simultaneous movement of both thumbs, vision of stimulated point and final thumb position, and visuomotor imagery were available. Furthermore, in one patient, the accuracy of the insensate thumb in cued conditions was higher than in a reference baseline condition, thus indicating that motor and cognitive cues can help the motor performance of patients with cortical somatosensory lesions.
The authors emphasize the importance of MRI as a means to functionally evaluate patients in primary (PR) and secondary (SR) rhinoseptoplasty. Forty-eight subjects were appraised who underwent various types of corrective nose surgery: 31 PR, 12 SR, 4 introgenic perforations, and 1 dermoid cyst of the nasal dorsum. MRI allows the nasal structures at the level of the valve to be visualized, as well as the medial and lateral walls of the nasal fossa in all of its components. MRI studies improve pre-surgical evaluation (alar cartilages, iaterdomal distance, and valvular configuration), and the exam of structures and anomalies which are hard to evaluate by the rhinoscopy (turbino-septal-synchias, perforations, losses of bone-cartilaginous substance). Moreover, through MRI the nasal respiratory surface (NRS) is determined and used by the authors to quantify the improvement of the postsurgical nasal respiratory function. All of these points gain importance during the planning of primary, secondary, and/or corrective surgery.
We report on the neuropsychological and neurolinguistic features of a bilingual patient, E.M., who presented with an uncommon pattern of aphasic deficit consequent to subcortical lesions mainly involving the left basal ganglia. Not only are reports of bilingual subcortical aphasia rare, but E.M.'s deficit is particularly uncommon for it concerns the most used mother tongue (Venetian) much more than a less practiced second language (standard Italian). In this patient, the linguistic deficit in mother tongue production has been observed in spontaneous speech and in cross language translation tasks, where an asymmetrical paradoxical performance has been revealed. Indeed, unlike neurologically intact subjects, E.M. has more difficulties when translating into her mother tongue than into her second language. Although E.M.'s mother tongue is prevalently an oral language, the asymmetrical translation pattern is similar in written and oral translation tasks, thus ruling out the possibility that the deficit simply reflects differences between written and oral language codes. Finally, another remarkable feature of E.M.'s impairment is its stability over almost 5 years from the stroke. We propose that this unusual type of recovery in E.M. is related to the higher degree of automatization of the first language with respect to the second one. This proposal fits with the role of basal ganglia in automatized motor and cognitive performance.
The value of functional and morphological neuroimaging in the early diagnosis of Alzheimer's disease (AD) is still debated. Described here are cerebral perfusion and linear measures of medial temporal lobe atrophy in 2 monozygotic twins discordant for AD who were investigated with technetium 99m-hexamethylpropy-leneamineoxime single-photon emission tomography (SPET) and magnetic resonance (MR) imaging. Both showed pathological cortical perfusion findings on SPET, while medical temporal lobe atrophy was present only in the affected twin. MR measures of medial temporal lobe atrophy have greater agreement with clinical data than do SPET measures of cerebral perfusion. Evaluation of atrophy may be useful in the early diagnosis of AD.
A 16 year old girl did a handstand for fun, returned to her feet, experienced a sudden pain in her back, and became progressively paraplegic within 30 hours. MRI showed lumbar cord swelling, multiple Schmorl's nodes, a collapsed T11-T12 disc space, and intraspongious disc prolapse into the T12 vertebral body. These findings, related to the initial manoeuvre, suggested that an acute vertical disc herniation could have occurred as the first step in a process leading to spinal cord infarction due to fibrocartilaginous emboli from the nucleus pulposus of the intervertebral disc. The medical literature so far reports 32 cases of fibrocartilaginous embolism (FCE) of the spinal cord, all at necropsy, with the exception of one histologically demonstrated in a living patient. A clinical diagnosis of FCE would be desirable for many important reasons, but was never made. This causes severe limitations in the knowledge of the disease and precludes any therapeutic possibility. On the basis of the clinical features and findings in the present case, compared with data from the reported cases, a first attempt is made to identify the clinical context within which new information obtainable through MRI examination can lead to a reliable clinical diagnosis of FCE. The vexed question of the pathogenesis has been reviewed. An increased intraosseous pressure within the vertebral body, due to acute vertical disc herniation, seems to offer a consistent pathogenetic explanation and some therapeutic prospects.
OBJECTIVES: To evaluate the pattern of regional brain atrophy in patients with frontotemporal dementia by comparing it with that in patients with Alzheimer's disease and normal controls. METHODS: Fourteen patients with frontotemporal dementia, 13 with moderate, and 33 with mild Alzheimer's disease, and 31 controls were studied. Atrophy was evaluated with linear measures in the anterior brain, medial temporal lobe, and hippocampal formation regions using MRI. RESULTS: Patients with frontotemporal dementia had greater atrophy in the anterior brain regions than patients with Alzheimer's disease or controls. Atrophy of the hippocampal formation, which best discriminates Alzheimer's disease from controls, was present also in patients with frontotemporal dementia. By contrast, atrophy of the medial temporal lobe, which is also present in Alzheimer's disease, was absent in frontotemporal dementia. CONCLUSION: A pattern of atrophy in the frontal lobes and hippocampal formation with sparing of the medial temporal lobe might be distinctive of frontotemporal dementia. Hippocampal involvement might not be specific for Alzheimer's disease and specific patterns of atrophy might be distinctive of some forms of degenerative dementia.
PURPOSE: To assess the sensitivity of linear measures of brain atrophy in the diagnosis of Alzheimer disease (AD) in the early stages. METHODS: Linear measures of regional frontal (Bifrontal index, interhemispheric fissure width), medial temporal lobe (interuncal distance, minimum thickness of the medial temporal lobe), and hippocampal (hippocampal height, width of the choroid fissure, width of the temporal horn) atrophy were made on magnified MR images obtained in 46 patients with AD (33 with mild severity and 13 with moderate severity) and in 31 control subjects. Gaussian modeling was used to compute sensitivity with specificity set at 95%. Discriminant analysis was used to identify measures independently contributing to the ability to discriminate AD patients from control subjects. RESULTS: The measure with the best sensitivity in discriminating AD patients from control subjects was the width of the temporal horn. A compound measure of width of the temporal horn, width of the choroid fissure, height of the hippocampus, and interuncal distance could discriminate patients with mild AD from control subjects with 86% sensitivity. Cross validation in patients with moderate AD confirmed the usefulness of the model (81% sensitivity). Measures of hippocampal atrophy alone could discriminate patients with mild AD from control subjects with 83% sensitivity; in patients with moderate AD, cross validation produced 87% sensitivity. CONCLUSIONS: Linear measures of hippocampal atrophy can be a useful adjunct in the routine diagnosis of AD, even in its early stages.
We evaluated the presence and type of vascular lesions with computerized tomography (CT) in 94 cognitively impaired elderly patients who had been defined as degenerative (Alzheimer's disease, AD) or vascular (multi-infarct dementia, MID) dementia patients on the basis of clinical data. Twenty-six percent of the 77 AD, but only 59% of the 17 MID patients had vascular lesions. The most represented vascular lesions were leukoaraiosis in AD and hemispherical cortical lesions in MID patients. Age, signs indicative of, and risk factors for vascular disease were associated with vascular lesions on CT. We conclude that CT scan is a necessary complement to historical and clinical data in the detection of vascularity in demented patients.
We describe a patient with defective tissue plasminogen activator (t-PA) release who developed internal cerebral vein thrombosis. She recovered completely and, as shown by MRI, favourable outcome was probably related to vascular recanalisation. Other members of the pedigree had a similar fibrinolytic deficiency without clinical manifestations. The use of oral contraceptives may have contributed to the patient's hypercoagulable state.
Iomeprol is a nonionic monomer which provides injectable formulations with the lowest osmolalities and the lowest viscosities among the currently available low-osmolar radiographic contrast agents of the same category. These favourable physico-chemical characteristics, together with high water solubility and low molecular toxicity, make this agent particularly suitable for carotid angiography, a procedure in which contrast-induced blood-brain barrier damage is maximal. This single-centre, randomised, double-blind, parallel-group study aimed at comparing the safety, tolerance and efficacy of 200 mgI/ml solutions of iomeprol and iopamidol in 100 consenting adult inpatients undergoing carotid intra-arterial digital subtraction angiography (IA-DSA). Both agents appeared to be safe and well tolerated. Only minor and transient adverse events occurred throughout the study. Electroencephalographic and neurological examinations performed pre- and post-contrast showed no abnormality induced by the test compounds. Vessel opacification was good or excellent in the vast majority of technically adequate examinations. Information provided by the angiographic examination was useful for subsequent patient management in almost all cases. It is concluded that iomeprol-200 and iopamidol-200 are safe and effective contrast media when used for carotid IA-DSA, an examination which proves useful not only for the diagnosis of vascular lesions, but also to best direct the management of patients scheduled for surgery of intracranial tumors.
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