1/25. White matter dementia in cadasil.Cerebral white matter disorders may be associated with profound neurobehavioral dysfunction. We report a 62-year-old man who had a slowly progressive 25-year history of personality change, psychosis, mood disorder, and dementia. neurologic examination disclosed abulia, impaired memory retrieval, and preserved language, with only minimal motor impairment. Neuropsychological testing found a sustained attention deficit, cognitive slowing, impaired learning with intact recognition, and perseveration. magnetic resonance imaging of the brain revealed extensive leukoencephalopathy. Right frontal brain biopsy showed ill-defined white matter pallor with hyaline narrowing of white matter arterioles. Granular osmiophilic material adjacent to vascular smooth muscle cells on electron microscopy of a skin biopsy, and an arginine for cysteine replacement at position 169 in the 4 EGF motif of the notch 3 region on chromosome 19q12 established the diagnosis of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil). This case illustrates that cadasil can manifest as an isolated neurobehavioral disorder over an extended time period. The dementia associated with cadasil closely resembles that which may occur with other white matter disorders, and represents an example of white matter dementia.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/25. Vasculopathic changes of cadasil can be focal in skin biopsies.Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil) is a newly described cause of vascular dementia. Pathologic examination shows multiple small infarcts in the deep cerebral white matter together with a nonatherosclerotic, nonamyloid angiopathy involving the media of small cerebral arteries. Ultrastructurally, characteristic granular material is present in the basal lamina of vascular smooth muscle cells in cerebral and extracerebral blood vessels. The ultrastructural changes have also been demonstrated in skin biopsies of affected patients; consequently, some investigators have recently recommended skin biopsies for the diagnosis of cadasil. This study describes a 54-year-old male with a family history for strokes who had clinical and radiological features suggestive of cadasil. A skin biopsy was performed to confirm the diagnosis. Initially, the characteristic vasculopathic changes of cadasil were not identified within small blood vessel walls. However, multiple deeper sections in other areas showed electron-dense material associated with vascular smooth muscle cells, characteristic of cadasil. Subsequent genetic testing demonstrated a single nucleotide substitution at position 659 on chromosome 19p13.1 causing an amino-acid change (Cys --> Phe), a finding indicative of cadasil. The involvement of blood vessels within the dermis makes skin biopsy a useful adjunct in the diagnosis of cadasil. However, as illustrated by this case, the findings may be focal, requiring a thorough evaluation of the entire biopsy specimen.- - - - - - - - - - ranking = 2keywords = muscle (Clic here for more details about this article) |
3/25. Occlusion of the cerebral arteries in Recklinghausen's disease.Two children, aged 18 months and 6 years, who had Recklinghausen's disease, had occlusion of cerebral arteries. One child had no motor deficit but the other had right hemiparesis and partial occlusion of the left posterior cerebral artery, a fact not found in the literature.- - - - - - - - - - ranking = 1440.1716465067keywords = hemiparesis (Clic here for more details about this article) |
4/25. Neuroborreliosis causing focal cerebral arteriopathy in a child.A 9-year-old girl presented with an acute right-sided hemiparesis. Initially, the clinical presentation and stable vasculopathic abnormalities on MR and conventional angiography were suspicious of a so-called "transient cerebral arteriopathy". Mild but persistent pleocytosis and an elevated CSF IgG index led to an extensive search for infectious and immunological causes of cerebral vasculitis, eventually revealing neuroborreliosis. Although rare, infectious and potentially treatable causes of arterial ischemic stroke should be considered in every child with a documented cerebral arteriopathy.- - - - - - - - - - ranking = 1440.1716465067keywords = hemiparesis (Clic here for more details about this article) |
5/25. Isolated central nervous system arteriopathy with multiple aneurysms in an adolescent. A case report.A 17-year-old male suffered recurrent subarachnoid hemorrhages due to multiple aneurysms in the distal branches of the cerebral arteries. autopsy revealed arteriopathy as well as the aneurysms. The arteriopathy was widespread, affecting the distal branches of small and medium-sized muscular cerebral arteries, as well as the anterior and posterior spinal arteries, and vasocorona. The arteriopathy was characterized by prominent intimal thickening, discontinuity or absence of the internal elastic lamina, and thinning and/or disappearance of smooth muscle in the tunica media with fibroplasia. Slight intimal thickening was also observed in the arteries of the circle of willis and its major branches, as well as in the basilar artery. However, the arterioles, venules and veins showed no remarkable features. The arterial lesions were found only in the central nervous system. The multiple aneurysms in the distal branches of the cerebral arteries, which had produced the main symptoms and clinical signs, were due to the arteriopathy.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
6/25. A huge intracranial xanthogranuloma in the middle cranial fossa: case report.Xanthogranulomas of the dura presenting with clinical symptoms are rare. We report here a case of a huge dural xanthogranuloma in the middle cranial fossa, which caused hemiparesis through marked displacement and stenosis of the middle cerebral artery. Although such tumors usually arise in association with histiocytosis X or familial hyperlipoproteinemia, the present case was not associated with these diseases.- - - - - - - - - - ranking = 1440.1716465067keywords = hemiparesis (Clic here for more details about this article) |
7/25. dissection of the anterior and middle cerebral artery with fatal ischemia following kicks to the head.A 19-year-old male received kicks to his head in the course of a fight with other young people. On admission to hospital he became drowsy and developed a hemiparesis and a facial paralysis on the right. After 3-4 days of continuous improvement the patient suddenly fell unconscious again and died on the seventh day after the trauma due to an increase of the intracranial pressure. Neuropathologic examination revealed an extensive dissection of the wall of the left anterior and middle cerebral artery. The age of the recurring infarction in the dependent areas of blood supply corresponded to the biphasic clinical course.- - - - - - - - - - ranking = 1440.1716465067keywords = hemiparesis (Clic here for more details about this article) |
8/25. Varicella with delayed hemiplegia.We report 4 children who developed acute hemiplegia 7 weeks to 4 months after varicella infection. In 2 patients, carotid angiography demonstrated segmental narrowing and occlusion of the middle cerebral artery. Their clinical and angiographic features were similar to those associated with contralateral hemiplegia after herpes zoster ophthalmicus, the pathogenesis of which comprises cerebral angiitis due to varicella zoster viral infection. We believe that our patients had the same pathogenesis. In a survey of infectious diseases in our region, the frequency of varicella with delayed hemiparesis was roughly 1:6,500 varicella patients.- - - - - - - - - - ranking = 1440.1716465067keywords = hemiparesis (Clic here for more details about this article) |
9/25. Subcortical neglect: neuropsychological, SPECT, and neuropathological correlations with anterior choroidal artery territory infarction.In 2 patients with infarction in the territory of the right anterior choroidal artery, hemiparesis, hemihypesthesia (in 1), and hemianopia or superior quadrantanopia were associated with severe multimodal hemineglect, without anosognosia, disorientation, or asomatognosia. Single-photon emission tomography showed that marked hypoperfusion was not limited to the right posterior capsular region, but also involved the overlying parietal cortex, and to a lesser extent the frontal cortex. At autopsy in 1 patient, the infarct was nearly limited to the deep white matter of the temporal isthmus and the retrolenticular part of the internal capsule; only minute lesions were present in the globus pallidus, body of caudate, and amygdala. These findings are consistent with a disconnection phenomenon as the basis for subcortical neglect with ipsilateral deactivation of the parietofrontal cortex.- - - - - - - - - - ranking = 1440.1716465067keywords = hemiparesis (Clic here for more details about this article) |
10/25. iodine-123 IMP SPECT before and after bypass surgery in a patient with occlusion of left anterior and middle cerebral arteries with basal abnormal telangiectasis (unilateral moyamoya disease).A case of left anterior and middle cerebral arterial occlusion with angiographic features similar to moyamoya disease was reported. IMP SPECT of the patient revealed the success of bypass surgery clearly. The patient complained of transient right hemiparesis with aphasia 4 times. The cerebral arteriography disclosed occlusions of left anterior and middle cerebral arteries at their proximal portions. Right internal carotid and its branches were normal. I-123 IMP SPECT study showed hypoperfusion in left temporal lobe, basal ganglia with incomplete reperfusion on the delayed (4 hours after injection) SPECT images. After the superficial temporal-middle cerebral artery anastomosis, I-123 IMP SPECT showed improvement of the brain blood flow. I-123 IMP SPECT was very useful in detecting the ischemic areas and evaluating the revascularizing surgery in this case.- - - - - - - - - - ranking = 1440.1716465067keywords = hemiparesis (Clic here for more details about this article) |
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