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  • 1
    ISSN: 1433-0350
    Keywords: Cerebral arteriovenous malformation ; Children ; Surgery ; Embolization ; Radiosurgery ; Recurrence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A series of 62 children with cerebral arteriovenous malformations admitted to our department in the course of 17 years (1975–1992) was reviewed in a retrospective study. In 54 cases hemorrhagic stroke was the first presenting symptom, followed by epilepsy in five cases. On admission 26 children presented with a neurological deficit, and 21 were admitted with a grade 3 status according to Botterell. Fifty-one malformations were supratentorial (41 hemispheric, 10 deep-seated) while 11 were infratentorial. According to Mori's criteria, 28 lesions were small, 19 medium, and 15 large. Fifty-two children were operated on, with total excision of the malformation achieved in 47 cases. In two children the malformation recurred. The evolution of neurological disorders has been studied with a mean follow-up of 8.5 years. Fifty patients had a satisfactory outcome on the Glasgow Outcome Scale. Four children died. These results were compared with those reported elsewhere in the pediatric literature.
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  • 2
    ISSN: 1432-0533
    Keywords: Multi-infarct dementia with leukoencephalopathy ; Cerebrovascular disease ; Vascular smooth muscle cell disease ; Hereditary disease ; Chromosome 19
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by a cerebral non-atherosclerotic, non-amyloid angiopathy mainly affecting the small arteries penetrating the white matter. In the brain vessels of two patients with CADASIL, abnormal patches of granular osmiophilic material have recently been described. Here we report the observation of similar granular osmiophilic material within the vessel walls of muscle and skin biopsies from a 54-year-old woman belonging to a CADASIL family, who suffered from subcortical dementia with leukoencephalopathy demonstrated on neuroimaging. Postmortem examination disclosed changes of the vessel walls in all the organs chiefly leading to cerebral lesions. Ultrastructural study showed destruction of the vascular smooth muscle cells (VSMC) and the granular osmiophilic material already found in muscle and skin biopsies in this patient. Both changes were found all along the arterial tree. The findings of this study indicate that CADASIL is a systemic vascular disease involving arterial VSMC and that the lesions are different in each organ and vessel wall, depending on their fine structure. Moreover, it emphasizes that skin and muscle biopsies might be useful for diagnosis of and research into CADASIL.
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Multi-infarct dementia with ; leukoencephalopathy ; Cerebrovascular disease ; Vascular smooth muscle cell disease ; Hereditary ; disease ; Chromosome 19
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by a cerebral non-atherosclerotic, non-amyloid angiopathy mainly affecting the small arteries penetrating the white matter. In the brain vessels of two patients with CADASIL, abnormal patches of granular osmiophilic material have recently been described. Here we report the observation of similar granular osmiophilic material within the vessel walls of muscle and skin biopsies from a 54-year-old woman belonging to a CADASIL family, who suffered from subcortical dementia with leukoencephalopathy demonstrated on neuroimaging. Postmortem examination disclosed changes of the vessel walls in all the organs chiefly leading to cerebral lesions. Ultrastructural study showed destruction of the vascular smooth muscle cells (VSMC) and the granular osmiophilic material already found in muscle and skin biopsies in this patient. Both changes were found all along the arterial tree. The findings of this study indicate that CADASIL is a systemic vascular disease involving arterial VSMC and that the lesions are different in each organ and vessel wall, depending on their fine structure. Moreover, it emphasizes that skin and muscle biopsies might be useful for diagnosis of and research into CADASIL.
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  • 4
    ISSN: 1432-086X
    Keywords: Mandible ; Arteriovenous malformation ; Embolization
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mandibular arteriovenous malformation (AVM) is a potentially life-threatening pathology requiring radical treatment. We report two female patients, aged 14 and 16 years, in whom occlusion of the AVM with Ethibloc after percutaneous transosseous puncture achieved definitive recovery. The first case was treated by direct injection of Ethibloc following inefficient arterial embolization with Ivalon particles. In the second case, Ethibloc was injected without previous arterial embolization. Control examinations performed at 6 months and 2 years confirmed complete calcification of the mandible in the first case and a total obliteration of the AVM on angiography in the second case.
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  • 5
    ISSN: 1432-1459
    Keywords: White matter ; Magnetic resonance imaging ; Alzheimer's disease ; Corpus callosum ; Signal hyperintensity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have previously demonstrated with MRI that as well as marked white matter involvement in late-onset Alzheimer's disease (AD), atrophy of the corpus callosum may also be present. This finding prompted us to study possible correlations between atrophy of the corpus callosum and white matter hyperintensity (WMH) and between white matter lesions and the severity of the disease. We compared the corpus callosum and white matter lesions on MRI from 15 AD patients and 15 controls. The white matter lesions were scored according to the Scheltens' rating scale. We found a significant reduction of the area of the corpus callosum and more severe white matter lesions in AD patients than in controls. Both atrophy of the corpus callosum and the severity of lesions depended mainly on the diagnosis of senile dementia of the Alzheimer type and on age but not on the diagnosis of presenile AD. We demonstrated a negative correlation between white matter lesions scores and areas of corpus callosum in AD patients and no correlation between the white matter lesions and the severity of the disease. We demonstrated that white matter lesions including WMH and atrophy of the corpus callosum are more frequent in AD than in controls. The predominance of white matter lesions in senile AD may be explained by the combination of aging and disease processes.
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  • 6
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A newborn suffered immediate neonatal respiratory distress because of an obstructive, soft-tissue nasal mass. Clinical examination revealed a cleft palate with a protruding polypoid mass. CT and MRI showed a heterogeneous nasopharyngeal mass and associated intracranial abnormalities – duplication of the hypophysis and hypoplasia of the corpus callosum. Duplication of the hypophysis is a very rare malformation, only 13 cases having been previously described. The suggested pathogenesis is duplication of the prechordal plate and anterior end of the notochord during early embryological development.
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  • 7
    ISSN: 1432-1459
    Keywords: Key words Computed tomography ; Hippocampal atrophy ; Alzheimer’s disease ; Memory disorders ; Dementia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Medial temporal lobe atrophy determined by temporal lobe oriented computed tomography (CT), 1 year before death, is strongly associated with histopathologically confirmed Alzheimer’s disease (AD). The aim of this study was to assess the diagnostic accuracy of medial temporal lobe measurement for the diagnosis of AD in patients referred to a memory disorders clinic, especially those at an early stage of the disease. CT oriented to the temporal lobe was performed in 333 subjects aged 41–93 years consecutively recruited in a Memory Disorders Clinic: 124 had probable AD, Mini Mental State score (MMS) = 17 (8); 50 possible AD [MMS = 21 (5)]; and 119 patients had miscellaneous memory disorders [MMS = 22 (7): frontotemporal lobe dementia, subcortical dementia, cortical Lewy body disease, vascular dementia, Korsakoff syndrome, focal atrophy, etc.]. There were also 19 anxious/ depressed patients [MMS = 29 (1)] with normal performance on memory tests, and 21 controls. The minimum width of the medial temporal lobe was measured. The best cut-off to distinguish AD patients from non-AD patients was 11.5 mm, in agreement with data in the literature. At this threshold, 84% of probable AD patients had a positive test and 90% of controls and anxious/depressed patients had a negative test. For the diagnosis of probable AD, sensitivity of the measurement was 0.81, specificity 0.95, predictive positive value 0.99, predictive negative value 0.45, and diagnostic accuracy 0.83. The test was positive in half the possible AD patients, and half those with miscellaneous memory disorders. It was negative in all anxious/depressed patients. Therefore, temporal lobe oriented CT might be a valuable tool for assessment of medial temporal lobe atrophy in AD routine practice.
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