1/241. technetium-99m-HmPAO brain SPECT in infantile Gaucher's disease.The authors report serial technetium-99m hexamethylpropylene-amine-oxime brain single photon emission computed tomography (SPECT) findings in two infants with Gaucher's disease type 2. Detailed neurologic and laboratory examinations, including bone marrow biopsies and enzymatic assays, were described. Serial brain magnetic resonance imaging studies in one patient illustrated the progressive cerebral atrophy in the frontal and temporal lobes. The SPECT in both cases demonstrated positive findings of initial scattered hypoperfusion, with extending to hypoperfusion of the entire cerebrum after 4 months of clinical deterioration. These changes in the SPECT findings may reflect progressive degeneration of the cerebrum in Gaucher's disease type 2. brain SPECT may provide useful information on cerebral flow and metabolic distribution corresponding to the neurologic deficits of neuronopathic Gaucher's disease.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
2/241. fucosidosis: immunological studies and chronological neuroradiological changes.A 3.5-y-old boy of Arabic origin had the clinical features of both type 1 and type 2 fucosidosis, consistent with an intermediate form of the disease. The activity of his leucocyte alpha L-fucosidase was absent. He presented with recurrent sinopulmonary infection and otitis media in addition to paronychia and a periapical dental abscess. Investigation of his systemic immune function did not reveal a significant underlying defect, but subtle abnormalities, particularly of antibody production and secretory IgA, cannot be excluded. The cranial magnetic resonance images showed periventricular and subcortical white matter abnormalities and mild cortical atrophy in addition to globus pallidus changes.- - - - - - - - - - ranking = 0.41718594607225keywords = intermediate (Clic here for more details about this article) |
3/241. MR measurement of regional relative cerebral blood volume in epilepsy.The purpose of this study was to evaluate the utility of magnetic resonance (MR) relative cerebral blood volume (rCBV) maps for studying regional hemodynamic changes in interictal and ictal epilepsy patients. Ten epilepsy patients were examined on a 1.5 T MR system. Nine patients were investigated interictally and one patient ictally. In the nine interictal patients, the dynamic plane was defined coronally through the hippocampus symmetrically. For the ictal patient, an axial dynamic plane was defined and the patient was scanned during seizure. Positron emission tomography (PET) studies were performed in 8 of the 10 patients. Lower rCBV of the left hippocampus was predicted by rCBV maps in seven of the nine interictal patients. The mean ratios of rCBV were 1.96 for left hippocampus/white matter and 2.49 for right hippocampus/white matter. The difference between these two ratios is statistically significant (P = 0.01, t-test). In two of the nine interictal temporal lobe epilepsy patients, lower rCBV areas were observed in the right hippocampus. In the ictal patient, the regional rCBV map demonstrated increased blood volume in the lesions. In eight of eight patients who underwent PET studies, MR rCBV findings were consistent with PET findings. The results show that regional hemodynamic changes in epilepsy can be evaluated with dynamic contrast-enhanced MR imaging. MR rCBV maps are sensitive to characterize seizure foci both ictally and interictally.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
4/241. Cerebellar atrophy: an important feature of carbohydrate deficient glycoprotein syndrome type 1.We report three children, all younger than 2 years of age, presenting with cerebellar atrophy related to carbohydrate-deficient glycoprotein syndrome type 1, an autosomal recessive metabolic disease. One patient had multisystem disease; two others had mental retardation with ataxia. In all cases the cerebellar atrophy was diagnosed on magnetic resonance imaging and, in one case, confirmed by autopsy. The cerebellar atrophy predominantly affected the anterior lobe. Vertical orientation of the tentorium cerebelli from the neonatal period in two cases suggests antenatal onset of the disease. Biological tests confirmed the diagnosis in all cases.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
5/241. Diaschisis in chronic viral encephalitis with Koshevnikov syndrome.The authors report a 61-year-old man with chronic viral encephalitis and Koshevnikov syndrome occurring 42 months after initial symptom of right hemiparesis. Serial computed tomography of the brain showed changes in the attenuation of the left temporal lobe lesion over time. Magnetic resonance images of the brain showed enlargement of left temporoparietooccipital lobes with cortical gyral enhancement on T1-weighted images following intravenous administration of gadolinium-DTPA. 99mTc-HMPAO single-photon emission computerized tomography showed increased radioactivity and hyperperfusion in the left temporoparietal region with paradoxically decreased local tissue perfusion at the contralateral right hemisphere. Follow-up magnetic resonance images of the brain 4 years later showed atrophy of bilateral cerebral hemispheres. We postulate that a "transcallosal diaschisis" with subsequent degeneration is a possible mechanism. A brain biopsy from the left temporal lobe lesion showed pictures compatible with viral encephalitis probably herpes simplex encephalitis.- - - - - - - - - - ranking = 3keywords = lobe (Clic here for more details about this article) |
6/241. Progressive hippocampal atrophy in chronic intractable temporal lobe epilepsy.We report on a 28-year-old man with long-standing intractable complex partial and secondary generalized seizures, whose magnetic resonance imaging scans 4 years apart documented progressive decrease in the left hippocampal volume. Left anterior temporal lobectomy with amygdalohippocampectomy rendered the patient seizure free at 12 months' follow-up. The findings demonstrate that patients with uncontrolled temporal lobe seizures may develop progressive atrophy of the hippocampus, in the absence of status epilepticus.- - - - - - - - - - ranking = 6keywords = lobe (Clic here for more details about this article) |
7/241. MRI, quantitative MRI, SPECT, and neuropsychological findings following carbon monoxide poisoning.Carbon monoxide (CO) poisoning has been shown to result in neuropathologic changes and cognitive impairments due to anoxia and other related biochemical mechanisms. The present study investigated brain-behaviour relationships between neuropsychological outcome and SPECT, MRI, and Quantitative magnetic resonance imaging (QMRI) in 21 patients with CO poisoning. Ninety-three per cent of the patients exhibited a variety of cognitive impairments, including impaired attention, memory, executive function, and mental processing speed. Ninety-five per cent of the patients experienced affective changes including depression and anxiety. The results from the imaging studies revealed that 38% of the patients had abnormal clinical MRI scans, 67% had abnormal SPECT scans, and 67% had QMRI findings including hippocampal atrophy and/or diffuse cortical atrophy evidenced by an enlarged ventricle-to-brain ratio (VBR). Hippocampal atrophy was also found on QMRI. SPECT and QMRI appear to be sensitive tools which can be used to identify the neuropathological changes and cerebral perfusion defects which occur following CO poisoning. Cerebral perfusion defects include frontal and temporal lobe hypoperfusion. Significant relationships existed between the various imaging techniques and neuropsychological impairments. The data from this study indicate that a multi-faceted approach to clinical evaluation of the neuropathological and neurobehavioural changes following CO poisoning may provide comprehensive information regarding the neuroanatomical and neurobehavioural effects of CO poisoning.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
8/241. multimodal imaging of residual function and compensatory resource allocation in cortical atrophy: a case study of parietal lobe function in a patient with Huntington's disease.In a case of Huntington's disease (HD) with dementia and pronounced parieto-frontal atrophy, the functional state of the affected regions was investigated using functional magnetic resonance imaging (fMRI) and fluorodeoxyglucose-positron emission tomography (FDG-PET). It was observed that although parietal areas showed extensive atrophy and reduced resting glucose metabolism, the patient performed with similar accuracy but with longer response time in a visuospatial task compared with healthy control subjects. At the same time, the blood oxygen level-dependent (BOLD) fMRI signal in these areas, which are involved in visuospatial processing, showed a similar task-dependent modulation as in control subjects. The signal amplitude (signal percent change) of the task-dependent activation was even higher for the HD patient than in the control group. This residual functionality of parietal areas involved in visuospatial processing could account for the patient's performance in the task concerned, which contrasted with his poor performance in other cognitive tasks. The increased percent-signal change suggests that a higher neuronal effort was necessary to reach a similar degree of accuracy as in control subjects, fitting well with the longer reaction time. We propose that fMRI should be considered as a tool for the assessment of functionality of morphologically abnormal cortex and for the investigation of compensatory resource allocation in neurodegenerative disorders.- - - - - - - - - - ranking = 4keywords = lobe (Clic here for more details about this article) |
9/241. Primary progressive aphasia: a case report.We report a 69-year-old male patient whose motor aphasia started at the age of 61. The language disability remained isolated and progressed over a period of eight years without any additional cognitive deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) showed moderate cortical atrophy with frontal dominance. Single photon emission tomography (SPECT) showed hypoperfusion in the frontotemporoparietal region, positron emission tomography (PET) demonstrated a global cortical reduction of glucose utilization with a lesser decrement in the occipital lobes. The clinical symptoms and the neuropsychological findings fit the diagnosis of primary progressive aphasia.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
10/241. Primary antiphospholipid syndrome and cerebral atrophy: a rare association?Neurologic complications are common in patients with antiphospholipid syndrome. In this article, we report the case of a young woman with neurologic disorders, a history of hypertension and transient ischemic attacks, and cerebral atrophy associated with primary antiphospholipid syndrome (PAPS). magnetic resonance imaging of the brain showed multiple ischemic lesions and remarkable atrophy of frontal and parietal lobes. Cerebral atrophy in patients with PAPS can be considered as a feature of this disease. The case is discussed on the basis of relevant past literature. Although there are few reports on neuroradiologic findings in patients with PAPS, cerebral atrophy has been described. Because PAPS is more frequently recognized today than in the past, this condition should be included in the differential diagnosis of cerebral atrophy, particularly in young patients.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
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