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• W2164656552 abstract "A 50-year-old man with a medical history notable only for hypercholesterolemia developed a generalized tonic-clonic seizure while at work. Family history was significant for a father who had onset of Alzheimer disease at age 71 years. Computed tomography of the head initially suggested a right frontal hemorrhage, and magnetic resonance imaging showed a well-circumscribed right frontal lesion (Figure, A) with features suggestive of a brain tumor. On computed tomography, it was 4 to 5 cm in size, with subtle ring enhancement, mild edema but negligible mass effect, and signs of calcification. A multidisciplinary team concluded that the patient's presentation was most consistent with a brain tumor such as oligodendroglioma. He underwent a right frontal craniotomy for resection. Preliminary intraoperative frozen sections of the tissue revealed gliosis and macrophages but no neoplasm. The surgical specimen consisted of multiple fragments of pale-tan gelatinous friable neural tissue, measuring 1.3 × 0.4 × 0.2 cm in aggregate. Granulomatous giant cells were present in and around vessels (Figure, B and C, hematoxylin-eosin, original magnification ×160). It also was rich in macrophages, visible gliosis, and cortical vessels that contained abundant amyloid, highlighted by Congo red stains and positive for β-amyloid (Figure, D, immunohistochemical stain for β-amyloid, original magnification ×160). Hemorrhages and reactive changes, including pronounced vascular reaction, were noticeable on the cortical surface and in the leptomeninges. There were moderate numbers of neuritic plaques and occasional neurofibrillary tangles detected on Hirano silver stain, consistent with Alzheimer disease pathology. After surgery, the patient did well and was discharged home after a short course of physical therapy, without any neurological deficit and with fully independent capabilities.What is your diagnosis?We present a case of a 50-year-old man with a generalized tonic-clonic seizure. Magnetic resonance imaging findings were suggestive of a brain tumor. Microscopic examination revealed cortex with vessels that contained abundant amyloid, associated with prominent giant cell reaction. The differential diagnosis included hemorrhage related to cerebral amyloid angiopathy (CAA), hemorrhagic tumor, hemorrhagic stroke, bleeding into an abscess, and inflammatory or autoimmune vasculitis. The clues pointing to CAA were lobar white matter location, absence of signs or symptoms of infection or autoimmune diseases, and minimal contrast enhancement. Cerebral amyloid angiopathy is commonly associated with normal aging. It is characterized by cerebrovascular amyloid deposition, associated with superficial lobar hemorrhage, ischemic lesions, and dementia. Hereditary forms of CAA can be classified according to the amyloid protein deposited. Amyloid angiopathy and associated giant cell reaction raise the suspicion of inflammatory CAA. The clinical spectrum of noninflammatory CAA is different from that of inflammatory CAA, which may respond to immunosuppressive treatment.The differential diagnosis for the clinical presentation and imaging findings included hemorrhage related to cerebral amyloid angiopathy (CAA), hemorrhagic tumor, hemorrhagic stroke, bleeding into an abscess, and inflammatory or autoimmune vasculitis. The indications suggestive of CAA were lobar white matter location, absence of signs or symptoms of infection or autoimmune diseases, and minimal contrast enhancement. The presence of amyloid angiopathy and associated giant cell reaction suggest an amyloid β-related angiitis (ABRA) or a destructive inflammatory or granulomatous reaction to amyloid. A clear-cut distinction between the 2 was difficult with the available data.Cerebral amyloid angiopathy is characterized by amyloid deposition in the walls of arteries, arterioles, and, less often, capillaries and veins of the central nervous system. It occurs in approximately 30% of healthy persons older than 60 years.1 In one study, CAA was found in 57% of 123 brains from autopsies of patients aged 59 to 101 years.2 By contrast, 80% to 90% of autopsy-confirmed cases of Alzheimer disease (AD) show at least a mild degree of CAA, and about 25% show moderate to severe CAA.3 Superficial lobar hemorrhage is the main clinical manifestation of CAA, which otherwise usually remains clinically silent. Cerebral amyloid angiopathy–associated hemorrhage accounts for approximately 11% of all cerebral hemorrhages among older persons.4 It can also cause infarction and ischemic leucoencephalopathy. The presence of the apolipoprotein ɛ4 genotype has been identified as an important risk factor for sporadic and AD-related CAA.56In 1938, more than a decade after the first description by Divry7 of amyloid deposition in senile plaques in AD, it was recognized by Scholz8 in Germany that amyloid can also be found in cerebral blood vessels in the brains of older patients. Interest in CAA increased in the 1970s, when it was recognized that CAA is often the cause of cerebral hemorrhage among older nonhypertensive individuals.9From a clinical perspective, it is important to determine whether vascular β-amyloid has induced or is associated with inflammation, because vasculitis warrants immunosuppressive therapy, while CAA is resistant to such treatment. No less important are the immunological and pathogenic implications of a destructive inflammatory reaction to cerebral or cerebrovascular β-amyloid. Clinical investigations exploring the potential of β-amyloid immunization among patients with Alzheimer disease required premature discontinuation following the development of subacute meningoencephalitis in a small number of patients.10Scolding et al11 described the clinical and pathological findings in a substantial series of patients with ABRA. They propose that ABRA is a distinct clinicopathological entity, a specific form of primary central nervous system angiitis, which is triggered by vascular deposition of β-amyloid in susceptible patients and is part of a more widespread immune reaction to β-amyloid within the central nervous system. In their patients with ABRA, the most common clinical features were mental status changes, often leading to dementia, headaches, hallucinations, and seizures. The earliest manifestations also included cerebellar features. Cerebral hemorrhage was found in approximately one fifth of the patients in the series. The authors suggest that ABRA behaves less like CAA and more like well-recognized idiopathic primary angiitis of the central nervous system without CAA.Eng et al12 explored the clinical effects of inflammation associated with vascular deposits of β-amyloid. They concluded that the clinical spectrum of inflammatory CAA is different from that of noninflammatory CAA and includes subacute cognitive decline, seizures, and leucoencephalopathy.Recent studies indicate that CAA is associated with white matter lesions and cognitive impairment.1314 Inflammatory response to CAA can cause clinically significant vascular dysfunction. Possible mechanisms for CAA-related white matter damage include vessel stenosis, loss of vasoreactivity caused by smooth muscle cell necrosis, or vasoactive effects of β-amyloid. White matter damage could cause symptoms characteristic of inflammatory CAA by interrupting white matter fibers linking areas of association cortex. Cerebral amyloid angiopathy is known to exist in several neurological conditions, including AD and Down syndrome, and can be categorized according to the type of amyloid-forming protein. Several cerebrovascular amyloid proteins (β-amyloid protein, prion protein, cystatin C, transthyretin, gelsolin, ABri, and ADan) have been implicated in familial CAAs. In AD, this is the 4-kd β-amyloid protein.15Icelandic CAA, or hereditary cystatin C amyloid angiopathy, is inherited as an autosomal dominant trait.16 It is associated with brain hemorrhage in young adult or middle-aged patients. Hereditary cystatin C amyloid angiopathy is characterized by vascular deposition of a mutant form of cystatin C γ trace (a cystein proteinase inhibitor), in which glutamine is replaced by leucine as a result of a single nucleotide substitution in codon 68. The amyloid implicated in this type of CAA is biochemically distinct from β-amyloid CAA. Dutch CAA, or hereditary cerebral hemorrhage with amyloidosis (Dutch), is also inherited as an autosomal dominant trait.16 In contrast to hereditary cystatin C amyloid angiopathy, it leads to brain hemorrhage in middle-aged and older patients. The vascular amyloid is related to β-amyloid protein. The condition results from a mutation at codon 693 of the amyloid precursor protein.Finally, central nervous system involvement in systemic AA and AL amyloidosis has been reported.17 In that study, the distribution of amyloid in the cases was indicative of a hematogenic pattern of amyloid deposition in systemic amyloidosis and was different from that in Alzheimer disease, prion disease, ATTR amyloidosis, and cystatin C disease. The authors found that the distribution corresponded to areas of the brain with a “leaky” blood-brain barrier." @default.
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• W2164656552 title "A 50-Year-Old Man With Acute-Onset Generalized Seizure" @default.
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