Cases reported "Dementia, Vascular"

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1/10. Spatz-Lindenberg disease: a rare cause of vascular dementia.

    BACKGROUND: Isolated cerebral thromboangiitis obliterans (Spatz-Lindenberg disease) is not well recognized as a cause of vascular dementia. CASE DESCRIPTION: A 58-year-old woman presented with dementia and pyramidal signs. neuroimaging showed multiple areas of white matter change. Brain biopsy showed intimal thickening of the walls of leptomeningeal and intraparenchymal arteries, almost to complete occlusion, with an intact internal elastic lamina and media and without inflammation or infiltration. The cortex showed only moderate gliosis. CONCLUSIONS: Spatz-Lindenberg disease should be considered in the differential diagnosis of vascular dementia. Additional studies of its pathogenesis are required to determine appropriate treatment.
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2/10. frontal lobe dementia with novel tauopathy: sporadic multiple system tauopathy with dementia.

    We present a novel tauopathy in a patient with a 10-yr history of progressive frontal lobe dementia and a negative family history. autopsy revealed mild atrophy of frontal and parietal lobes and severe atrophy of the temporal lobes. There were occasional filamentous tau-positive inclusions, but more interesting were numerous distinctive globular neuronal and glial tau-positive inclusions in both gray and white matter of the neocortex. Affected subcortical regions included substantia nigra, globus pallidus, subthalamic nucleus, and cerebellar dentate nucleus, in a distribution similar to progressive supranuclear palsy (PSP), but without significant accompanying neuronal loss or gliosis. Predominantly straight filaments were detected by electron microscopy (EM), while other inclusions were similar to fingerprint bodies. No twisted ribbons were detected. Immuno-EM studies revealed that only the filamentous inclusions were composed of tau. immunoblotting of sarkosyl-insoluble tau revealed 2 major bands of 64 and 68 kDa. Blotting analysis after dephosphorylation revealed predominantly 4-repeat tau. sequence analysis of tau revealed that there were no mutations in either exons 9-13 or the adjacent intronic sequences. The unique cortical tau pathology in this case of sporadic multiple system tauopathy with dementia adds a new pathologic profile to the spectrum of tauopathies.
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3/10. Mini-infarct encephalopathy associated with uncommon microvessel convolute formation presenting with presenile dementia.

    A female patient started to suffer from transient ischemic attacks when she was 47 years of age, followed by increasing predominantly left-side spastic tetraparesis, generalized seizures and progressive dementia over a period of 11 years. She died when she was 58 years of age. On gross examination the brain showed enlarged ventricles and arteriosclerotic changes of large extracerebral vessels of the circulus arteriosus. Microscopic examination of the atrophic brain showed innumerable incomplete microinfarcts in the white and gray matter throughout all parts of the brain. In the white matter these lesions were characterized by small foci of demyelination and loss of oligodendrocytes while occasionally some scavenger cells were seen. axons seemed to be unaffected or displayed irregular axonal regeneratory growth. Any inflammatory reaction failed. In the cerebral cortex and subcortical nuclei the lesions showed loss of neurons and decrease in synaptophysin expression. Intracerebral arteries showed fibrosis or fibrohyalinosis of the entire intracerebral small-vessel network. In addition, numerous uncommon clusters of angioma-like telangiectatic vessels were observed. Medium-sized ischemic infarcts were found in the right putamen and adjacent internal capsule region, left-side dorsolateral brain stem and cerebellar hemisphere as well as a left-side pyramidal tract degeneration. Contralateral pseudohypertrophy of the inferior olivary nucleus was seen. The clinical and the neuropathologic observations made in this patient are compatible with small vessel disease characterized by a multicentric special and not yet described type of incomplete mini-infarcts in cerebral cortex and white matter accompanied by some larger ischemic infarcts of the common type in brain stem and cerebellum.
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4/10. Neurobrucellosis presenting as leukoencephalopathy: the role of cytotoxic T lymphocytes.

    A 65-year-old man developed a leukoencephalopathy associated with neurobrucellosis. The disease followed a 15-month progressive course with neurologic symptoms, and magnetic resonance imaging revealed bilateral symmetrical T2 signal hyperintensities in the white matter. biopsy of the cerebral cortex and white matter was significant for nongranulomatous meningoencephalitis with reactive microgliosis and astrogliosis. The inflammatory infiltrate was predominantly composed of T lymphocytes, including numerous cytotoxic T cells. There was no evidence of significant myelin destruction. No organisms were detected microscopically, but elevated immunoglobulin g titers to Brucella were found in the cerebrospinal fluid. An abscess formed at the biopsy site, and brucella melitensis was cultured from abscess contents. Neurobrucellosis is difficult to diagnose outside endemic regions and is associated with leukoencephalopathy-like pathology. Cytotoxic T lymphocytes and microglia activation play an immunopathogenic role in this rare disease.
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5/10. Cortical blood flow and cognition after extracranial-intracranial bypass in a patient with severe carotid occlusive lesions. A three-year follow-up study.

    The long-term effect of extra-intracranial arterial bypass on cerebral circulation was examined. Cortical blood flow and cognitive ability were evaluated pre- and up to 3 years post-bypass in a 58-years-old man with severe carotid occlusive lesions, who presented with 3 transient cerebral ischaemic attacks which resulted in mental deterioration over 3 years. Regional cerebral blood flow (rCBF) was evaluated pre- and up to 33 months post-bypass by 123Iodine N-isopropyl-p-iodoamphetamine (IMP) single-photon emission CT (SPECT). Mental abilities were evaluated before and up to 33 months after surgery by the Hasegawa's dementia rating scale (HDRS). Pre-operatively, cerebral angiography showed left carotid siphon occlusion and hypoplastic stenosis of left anterior cerebral artery with collaterals from the anterior communicating artery. CT and MRI showed left temporo-parietal borderzone infarction and an enhanced T 1 lesion by gadolinium-DTPA at left periventriculum. rCBF showed extensive hypoperfusion in left anterior-parieto-temporal-cortex. HDRS scores deteriorated apparently on days 3, 5, which recovered gradually on days 8, 10, 75 after onset of mental deterioration. A bypass was performed 4 months after onset. rCBF showed gradual recovery in the left anterior-parietal cortex up to 33 months after bypass. Semiquantitative rCBF showed gradual decreases of regional asymmetry after the bypass. HDRS scores returned to their maximum up to 37 months after onset. Three-year follow-up shows improved cortical rCBF and cognition after the bypass.
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keywords = cortex
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6/10. Vascular chorea: case report with pathology.

    We report a patient with long-standing systemic hypertension who developed progressive generalized chorea and dementia beginning at 70 years of age with no family history or other features to suggest Huntington's disease. At postmortem examination, congophilic angiopathy and atherosclerosis causing neostriatal neuronal loss and gliosis were found, in addition to plaques and neurofibrillary tangles in the cortex. This case is a rare demonstration of a vascular pathology causing late onset generalized chorea in association with dementia due to Alzheimer's-type cortical changes.
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7/10. Occurrence of paroxysmal synchronous EEG discharges in subcortical arteriosclerotic encephalopathy (Binswanger's disease).

    A 72-year-old patient with von Recklinghausen's disease showed akinetic mutism within 6 months of the onset of dementia. The findings of diffuse cerebral atrophy on CT and periodic synchronous discharges (PSDs) in EEG suggested Creutzfeldt-Jakob disease. However, autopsy findings of diffuse softening of the subcortical white matter and marked arteriosclerotic changes of the subcortical arterioles with sparing of the cortex in the cerebrum confirmed a diagnosis of Binswanger's disease. Binswanger's disease should be included in the differential diagnosis of dementia showing PSDs in EEG.
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8/10. Hemineglect and seizures in Binswanger's disease: clinical-pathological report.

    The range of clinical effects from ischaemic damage to white matter in Binswanger's disease has not been fully characterised. Although focal deficits and seizures occur frequently, superficial infarcts often coexist, making the cause of these symptoms unclear. The case of a 69 year old woman is described who presented with acute left sided weakness and hemispatial neglect, followed a year later by electrographically documented seizures originating from the right hemisphere. Interim examinations showed bilateral pyramidal signs and mild intellectual decline. Serial CT and MRI studies showed bilateral diffuse ischaemic lesions of the cerebral white matter and old left sided lacunar infarcts but no evidence of acute infarction. Post-mortem examination showed gliosis and demyelination of the deep white matter which spared the subcortical arcuate fibres; this is consistent with Binswanger's disease. The neocortex was normal. This case and previous reports indicate that focal symptoms typically referable to the grey matter, including hemineglect and seizures, may occur as a manifestation of subcortical ischaemic injury to white matter in Binswanger's disease.
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9/10. A comparative I-123 IMP SPECT study in Binswanger's disease and Alzheimer's disease.

    The regional cerebral blood flow in 31 patients was semiquantitatively measured with SPECT using N-isopropyl iodo-amphetamine (I-123 IMP). Of the 31 patients, 9 had Binswanger's disease (BD) (mean age: 64.2 years), 10 had Alzheimer's disease (AD) (mean age: 66.8 years), and 12 were neurologically normal (mean age: 67.8 years). Tracer activity was determined in 7 pairs of cerebellar, cortical, and subcortical regions and was expressed as two ratios: the activity in each region of interest (ROI) compared to the mean count in the cerebellar region (ROI/CER ratio) and compared to the mean count in the occipital cortex (ROI/OCC ratio). patients with BD showed a reduction of the ROI/CER ratio in the temporal cortex, thalamus, and basal ganglia compared to normal subjects. However, the ROI/CER ratio for the occipital region was higher than that of normal controls, suggesting that cerebellar blood flow was reduced in patients with BD. AD patients showed a decreased ROI/CER ratio in the frontal, temporal, and parietal cortices. Both BD and AD patients showed decreased ROI/OCC ratios in all the cortical and subcortical regions compared to the neurologically normal patients. In BD patients, the most marked reduction of both ratios was found in the thalamus, while for AD patients the most prominent reduction was seen in the parietal cortex. Our results indicate that these two types of dementia show characteristic regional cerebral blood flow patterns, which may be associated with their clinical features.
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10/10. Disseminated neocortical and subcortical encephalopathy (DNSE) with widespread activation of brain macrophages: a new dementia disorder? autopsy reports of two postmenopausal women from families with mitochondrial dna mutations.

    In this study we present 2 postmenopausal women who showed clinical symptoms that resembled those of a rather well-defined group of vascular dementia disorders, termed subcortical dementia (Binswanger disease, cadasil). Patient 1 exhibited mitochondrial dna (mtDNA) variants in the ND5 gene at position 13,708 and the Cytb gene at position 15,257. These dna variants have been described in a number of neurologic disorders, but their pathogenetic potential is unclear. Patient 2 showed the same dna alterations and an additional mtDNA variant at position 15,812 in the Cytb gene. The principal neurohistologic features of the 2 atrophic brains presented here include: subtotal selective neuronal cell loss in the cortex and, to a lesser degree, in the basal ganglia (claustrum, putamen, globus pallidus), sparing palaeocortex and periarchaeocortex, and a very characteristic and diagnostic feature was detachment of astrocytic processes from capillary walls resulting in pericapillary space formation. These pericapillary spaces were partially filled with macrophages. The spaces were not associated with total breakdown of the blood vessel walls as demonstrated by the absence of erythrocytes, lymphocytes, or polymorphonuclear leukocytes outside the vascular bed of the brain; progressive subcortical encephalopathy, as it is seen in subcortical dementia (Binswanger), but lacking arterial lipohyalinosis. The cerebral grey and white matter revealed cuffing of arteries and arterioles by adventitial macrophages. The neocortical and subcortical changes were accompanied by myriads of activated macrophages filled with lipids. The pathology of our 2 cases differs from that of other neurodegenerative disorders and we suggest the term of "disseminated neocortical and subcortical encephalopathy (DNSE) with widespread activation of brain macrophages".
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