1/12. Intracerebral hemorrhage caused by cerebral amyloid angiopathy: a case report.cerebral amyloid angiopathy (CAA) accounts for approximately 10% of spontaneous intracerebral hemorrhages (ICH), and typically occurs in the cortex and subcortical white matter. It is characterized by the deposition of amyloid fibrils in the leptomeningeal, cortical and subcortical arteries. Pathologically, amyloid is stained pink with congo red and shows yellow-green birefringence when viewed under polarized light. Although there have been many reports of CAA in the literature, it has rarely been described in taiwan. This is the report of a case of a 75-year-old man with ICH caused by CAA. The postoperative course was uneventful. The incidence of this disease increases with age. The authors, therefore, suggest conducting a brain biopsy and special stain for CAA in each operative case of spontaneous ICH, especially in the elderly.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/12. Familial British dementia with amyloid angiopathy: early clinical, neuropsychological and imaging findings.Familial British dementia with amyloid angiopathy (FBD) is an autosomal dominant condition characterized by a dementia, progressive spastic tetraparesis and cerebellar ataxia with onset in the sixth decade. A point mutation in the BRI gene has been shown to be the genetic abnormality. Genealogical work with the large family originally reported by Worster-Drought and updated by Plant has identified nine generations dating back to the late eighteenth century. The pedigree now includes six living affected patients, 35 historical cases, and 52 descendants at risk of having inherited the disease. A common ancestor has been identified between the large pedigree and a case report of 'familial cerebellar ataxia with amyloid angiopathy'. An autopsy case from a separate family with an identical condition is described but no common ancestor with the large pedigree has been found. Case histories have been researched and updated in each pedigree. Eleven individuals at risk of FBD, aged between 44 and 56 years, agreed to undergo a clinical and neuropsychological assessment along with MRI brain imaging in order to clarify early diagnostic features. Five of the eleven were thought to show early clinical signs of the disease. Neurological examination was abnormal in three, with limb and gait ataxia and mild spastic paraparesis. Three had impaired recognition and recall memory and another had mild impairment of delayed visual recall. All affected individuals had an abnormal MRI of the brain, consisting of deep white-matter hyperintensity (T(2)-weighted scans) and lacunar infarcts, but no intracerebral haemorrhage. The corpus callosum was affected particularly, and in one patient it was severely atrophic.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
3/12. cerebral amyloid angiopathy presenting as nonhemorrhagic diffuse encephalopathy: neuropathologic and neuroradiologic manifestations in one case.A case of cerebral amyloid angiopathy is presented with MR imaging findings of high intense signal on T2-weighted sequences at the level of the white and gray matter of both hemispheres in the absence of neuroradiologic signs of cerebral hemorrhage. The biopsy specimen revealed deposition of amyloid in the walls of the intracranial arterial branches and focal ischemic changes and gliosis in the gray and white matter. We consider this presentation to be very unusual in patients affected by cerebral amyloid angiopathy.- - - - - - - - - - ranking = 2keywords = white (Clic here for more details about this article) |
4/12. Reversible leukoencephalopathy in cerebral amyloid angiopathy presenting as subacute dementia.This report concerns a 71-year-old woman who had rapid progressive dementia along with myocloni and increased blood pressure. Cranial computed tomography and magnetic resonance imaging scans showed bilateral widespread white matter changes with mass effect. A brain biopsy revealed an amyloid angiopathy in leptomeningeal as well as cerebral cortex arteries. After 2 months of antihypertensive treatment, a dramatic improvement of cognitive functions and a spectacular regression of leukoencephalopathy were observed. We suggest that hypertensive encephalopathy may worsen or reveal cerebral amyloid angiopathy.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
5/12. cerebral amyloid angiopathy with granulomatous angiitis ameliorated by steroid-cytoxan treatment.We report a case of a 62-year-old black woman who, 8 months prior to death, developed confusion, apraxias, disorientation, and difficulties with her vision. There was no dementia. Computed tomography (CT) scan and magnetic resonance imaging (MRI) suggested a tumor in the right posterior parietal white matter. A biopsy of the lesion displayed granulomatous angiitis and severe cerebrovascular amyloidosis, but no tumor was identified. Chronic inflammation with an occasional multinucleated giant cell was seen about the amyloid-infiltrated vessels. The cortex demonstrated gliosis but no plaques or tangles. Subsequently, the patient was treated with steroids and Cytoxan, with an improvement in her neurologic status. She died of opportunistic bronchopneumonia 8 months after the initial onset of her symptoms. On postmortem examination, the biopsied area of the brain showed atrophy with gliosis. Amyloid angiopathy was present but in much lesser degree than in the biopsy. Scant perivascular inflammatory infiltrates were seen only focally, and no giant cells were observed. The amyloid, both in the biopsy and autopsy material, was of the Alzheimer A4 type. This case suggests that steroid and cytoxan treatment ameliorated the angiitis and the amyloid angiopathy as well. The pertinent literature is discussed.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
6/12. Reversible leukoencephalopathy associated with cerebral amyloid angiopathy.The authors describe three patients with reversible leukoencephalopathy associated with cerebral amyloid angiopathy (CAA). Rapid progression of neurologic symptoms was followed by dramatic clinical and radiographic improvement. Pathologically, CAA was associated with varying degrees of inflammation ranging from none to transmural granulomatous infiltration. In the appropriate clinical context, the MRI finding of lobar white matter edema with evidence of prior hemosiderin deposition may indicate the presence of a reversible CAA leukoencephalopathy.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/12. cerebral amyloid angiopathy presenting as a posterior leukoencephalopathy: a case report and review of the literature.cerebral amyloid angiopathy (CAA) is well known to present with lobar intracerebral hemorrhage, dementia or transient neurological events. White matter changes with CAA have only been recently described and can be seen with either sporadic or familial CAA. We present a 50-year-old man with rapidly progressive dementia in whom MRI brain showed symmetrical white matter changes in the parieto-occipital regions. brain biopsy revealed changes of CAA along with features of Alzheimer's disease. immunohistochemistry revealed amyloid beta protein. The subcortical lesions were thought to occur from hypoperfusion of the distal white matter. The role of amyloid in the pathogenesis of CAA and the mechanism of leukoencephalopathy are discussed.- - - - - - - - - - ranking = 2keywords = white (Clic here for more details about this article) |
8/12. Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy.Idiopathic or primary angiitis of the CNS (PACNS) and cerebral amyloid angiopathy (CAA) are unusual vasculopathies generally regarded as unrelated disorders. A few case reports have, however, described granulomatous angiitis in patients with sporadic, amyloid beta peptide (Abeta)-related CAA. Here we describe the clinical, neuroradiological and neuropathological features of nine patients with Abeta-related angiitis (ABRA). Combining these with the individual case reports drawn from the literature has allowed us to define ABRA as a clinical entity and to compare its features with those of PACNS. The mean age of presentation of ABRA (67 years) is higher than that of PACNS but lower than that of sporadic non-inflammatory Abeta-related CAA. Alterations in mental status (59%), headaches (35%), seizures and focal neurological deficits (24%) are common. hallucinations are a presenting manifestation in 12% of cases. Most patients have white matter hyperintensities on MRI but these are of similar appearance to those in PACNS. cerebrospinal fluid usually shows modest elevation of protein and pleocytosis. Neuropathology reveals angiodestructive inflammation, often granulomatous, and meningeal lymphocytosis. Abeta is consistently present in abundance in affected blood vessels but usually scanty within the parenchyma of the cerebral cortex. However, the cortex includes numerous activated microglia, occasionally in a plaque-like distribution and containing cytoplasmic Abeta. The cerebral white matter shows patchy gliosis and rarefaction, in some cases marked. Our findings (i) help to dissect one separate clinicopathological entity from what is likely to be a spectrum of primary angiitides of the CNS; (ii) have important therapeutic implications for one category of patients with amyloid-related vasculopathy; and (iii) may provide valuable insights into the development of amyloid-associated inflammation, of relevance not only to ABRA but also to Abeta-immunization-related encephalitis and to Alzheimer's disease.- - - - - - - - - - ranking = 2keywords = white (Clic here for more details about this article) |
9/12. Multifactorial genesis of neuroradiological "leucoencephalopathies.".Three cases of neuroradiologically diagnosed "leucoencephalopathy" are reported. Histopathological examination disclosed Binswanger's encephalopathy in Case 1, congophilic angiopathy with secondary leucoencephalopathy in Case 2, and hiv encephalopathy with secondary white matter changes in Case 3. These three cases demonstrate the unspecificity of neuroradiological findings in "leucoencephalopathy".- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
10/12. The incredible shrinking brain.A 65-year-old white woman developed progressive visuospatial abnormalities over an eight-year course, secondary to Alzheimer's disease with amyloid angiopathy. Imaging studies demonstrated focal atrophy of the parietal and occipital lobes without hemorrhage. This patient manifested simultagnosia without ocular dysmetria or optic ataxia; hence, a true Balint's syndrome was not present. Her visual acuities and fields have remained stable status post-occipital lobe biopsy.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
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