A restricted subarachnoid hemorrhage in the cortical sulcus in cerebral amyloid angiopathy: could it be a warning sign?

BACKGROUND: Cerebral amyloid angiopathy is a well-known disease that is predominantly recognized in elderly people and repeatedly causes large subcortical hemorrhages. These hemorrhages may be derived from vessel wall weakness because of Abeta depositions in the wall of the cortical and leptomeningeal arteries. Although vessel ruptures in CAA have been thought to occur in cortical arteries, it was recently demonstrated that the primary hemorrhage occurs in the subarachnoid space, particularly the cerebral sulci, as a result of multiple ruptures of meningeal arteries in some cases of subcortical hematoma caused by CAA. CASE DESCRIPTION: Case patient 1 was a 74-year-old woman who presented with epileptic seizure. A restricted SAH in the right frontal lobe was observed on MRI. Thirty-three days later, left hemiparesis occurred suddenly and a huge subcortical hematoma was observed in the right frontal lobe on CT. The hematoma was removed, and the patient was pathologically diagnosed with amyloid angiopathy. Case patient 2 was a 73-year-old man who presented with epileptic seizure. A restricted SAH in the right frontal lobe was observed on MRI. Twenty days later, left hemiparesis occurred suddenly and a huge subcortical hematoma was observed in the right frontoparietal area on CT. Hematoma removal was performed on both patients, and they were diagnosed pathologically with amyloid angiopathy. CONCLUSIONS: We report on the cases of 2 patients with CAA who presented with epileptic seizure and were found to have a restricted subarachnoid hematoma in the cerebral sulcus on MRI before their subcortical hemorrhages occurred. Both cases were diagnosed pathologically. This demonstrated that vessel ruptures in CAA can occur in the subarachnoid space, particularly the cerebral sulci, as a result of ruptures of meningeal arteries. A restricted SAH on CT/MRI could be a warning sign of a huge subcortical hemorrhage in CAA.     

Amyloid angiopathy — a rare cause of intracerebral hemorrhage

The cerebral amyloid angiopathy (CAA), morphologically characterized by amyloid deposition in the vessel walls which are altered to rigid tubes, is a chronic disease of the cortical and meningeal vessels and can cause intracerebral hemorrhages (1,5% of all intracerebral bleedings). We report the course of five surgically treated patients with lobar space-occupying intracerebral hemorrhages and CAA confirmed by histological examination. All patients were elderly (74–84 years), in good condition, and self-providing before the hemorrhage. There were no signs of dementia of the Alzheimer's type. In four cases, CT showed a hematoma in the parietooccipital, and in one case in the temporo-parietal, region. After surgical evacuation, two patients recovered, one patient remained in bad condition, and two patients died from recurrent hemorrhage within two weeks. Spontaneous intracerebral hemorrhage of lobar localisation in an elderly patient strongly suggests CAA. The prognosis seems to be poor in cases with recurrent hemorrhage, the other patients presented an uneventful course, comparable with patients operated on for intracerebral bleeding of other origin. Further investigations are necessary to elucidate the prognosis of this entity.     

Cerebral amyloid angiopathy presenting as multiple intracranial lesions on magnetic resonance imaging. Case report

Cerebral amyloid angiopathy is recognized as an important cause of spontaneous intracerebral hemorrhage in the elderly normotensive patient. Magnetic resonance (MR) imaging characteristics of this disease entity are rarely mentioned in the literature. The MR imaging findings of an elderly normotensive patient presenting with an acute spontaneous intracerebral hemorrhage secondary to amyloid angiopathy are reported and a brief review of amyloidosis is presented.     

Primary intracerebral hemorrhage due to probable cerebral amyloid angiopathy complicated by brain abscess: case report

Cerebral amyloid angiopathy (CAA), in which deposition of amyloid within the arterial media and adventitia is the remarkable feature, causes spontaneous lober cerebral hemorrhage in elderly person, and some reports show the quite high occurrence rate of this entity among intracerebral hemorrhage in patents above 70 years old. Brain abscess resulting from intracerebral hemorrhage is rare. To our knowledge, no report of such hemorrhage which is caused by CAA has been published so far. We report a case of brain abscess, from which Stenotrophomonas maltophilia was isolated, following spontaneous non-hypertensive intracerebral hemorrhage caused by probable CAA, with a review of the relevant literature.     

Recurrent intracerebral hemorrhages in cerebral amyloid angiopathy: a case report

Cerebral amyloid angiopathy frequently causes recurrent intracerebral hemorrhages in elderly patients who do not have systemic hypertension. Surgery should be reserved for conditions which cannot be controlled by medical treatment. When surgery is needed, potential complications (such as bleeding near the operation site or remote area) should be kept in mind. A case study of a 66-year-old woman with cerebral amyloid angiopathy and recurrent intracerebral hemorrhages is presented.     

Intracerebral hemorrhage induced by cerebral amyloid angiopathy in a patient during transurethral electrocoagulation]

A 68-year-old female with severe anemia due to a bleeding bladder was scheduled for emergency transurethral electrocoagulation surgery under general anesthesia. Her preoperative consciousness was clear. After preoxygenation, general anesthesia was induced with ketamine 40 mg and vecuronium 6 mg. After tracheal intubation, anesthesia was maintained with nitrous oxide, oxygen, and isoflurane (0.5-0.8%). During the operation, a large fluctuation in blood pressure was observed. The operation was completed uneventfully, but the patient did not recover smoothly from the anesthesia. A few minutes later, signs of neurological abnormality were observed, and an emergency brain CT scan was performed. Since CT images of the brain showed extensive intracerebral hemorrhage (ICH) and edema, emergency craniotomy was performed. Postoperative pathological examination showed that the cause of the ICH was cerebral amyloid angiopathy (CAA). Since the incidence of CAA is relatively high in elderly people and CAA can cause ICH due to fluctuation in blood pressure, prudent anesthetic management is needed for elderly patients undergoing emergency operations, particularly an operation leading to a decrease in blood volume.     

Cerebral amyloid angiopathy associated with hemorrhage: immunohistochemical study of 41 biopsy cases.

The relationship between cerebral amyloid angiopathy and hemorrhage was investigated by an immunohistochemical study of biopsy cases to characterize the involvement of amyloid beta-protein, apolipoprotein E, and cystatin C in cerebral amyloid angiopathy associated with hemorrhage. The amyloid-laden vessels were examined in biopsy specimens from 41 surgical cases of sporadic cerebral amyloid angiopathy (36 cases with hemorrhage and 5 cases without hemorrhage), using immunohistochemical staining with antibodies against amyloid beta-protein, apolipoprotein E, cystatin C, and alpha-smooth muscle actin. The relationship between the occurrence, recurrence, and enlargement of the hemorrhage, and the semiquantitative estimation of the cerebrovascular amyloid-related protein deposition was analyzed using Fisher's exact test. Severe amyloid beta-protein (p < 0.013) and apolipoprotein E (p < 0.013) immunoreactivity were risk factors for the occurrence of the hemorrhage. Severe cystatin C immunoreactivity was a risk factor for the occurrence (p < 0.002) and enlargement (p < 0.014) of the hemorrhage, and tended to induce recurrent hemorrhage (p < 0.103). In addition, loss of the vascular smooth muscle was observed in the intensely amyloid-laden vascular walls that showed cystatin C-immunoreactivity. The present study indicates that intense amyloid beta-protein deposition with cystatin C deposition weakens the cerebrovascular walls, and that cystatin C deposition is a strong predictor of hemorrhage in cerebral amyloid angiopathy.     

Intracerebral hemorrhage due to cerebral amyloid angiopathy. Case report

The case is presented of a 59-year-old man with cerebral amyloid angiopathy and three consecutive hemorrhages in the occipital lobes. The clinicopathological features and the relationship to Alzheimer's dementia are discussed. The correct treatment of intracerebral hemorrhage related to cerebral amyloid angiopathy is a matter of controversy.     

Recurrence and extension of lobar hemorrhage related to cerebral amyloid angiopathy: multivariate analysis of clinical risk factors

BACKGROUND: Many recent studies have analyzed clinical risk factors for the recurrence and extension of intracerebral hemorrhage. However, they have not been investigated in patients with lobar hemorrhage related to cerebral amyloid angiopathy (CAA). METHODS: We studied 40 surgically treated patients with lobar hemorrhage diagnosed histologically as being related to CAA. To determine clinical factors influencing the recurrence and hematoma size their clinical data (demographics, medical history, and radiographic and laboratory data) were examined retrospectively and subjected to multivariate analysis. RESULTS: Twelve patients (30%) had recurrent lobar hemorrhage. Twenty-one patients had a small hematoma and 19 had a large hematoma. Hypertension was the only significant clinical factor influencing the recurrence of CAA-related lobar hemorrhage. There was no significant clinical factor influencing the hematoma size of CAA-related lobar hemorrhage. CONCLUSIONS: The history of hypertension is associated with an increase in the recurrence of CAA-related lobar hemorrhage.     

Cerebral amyloid angiopathy complicated by multiple cerebral infarcts and intracerebral hemorrhages: case report

Recently, cerebral amyloid angiopathy is stressed as an unusual and infrequent cause of cerebral infarct or intracerebral hemorrhage. This report described a case of cerebral amyloid angiopathy complicated by multiple cerebral infarcts and multiple intracerebral hemorrhages. This 70-year-old man was admitted to our hospital on November 10, 1984 for evaluation of the gradual onset of dysarthria. Examination showed only slight dysarthria. There was no history of hypertension or dementia. A computed tomography (CT) showed enlarged ventricles with cortical atrophy and multiple low-density lesions, deep in the left frontal, left parietal, right parietal lobes, and in the both basal ganglias. The patient discharged from the hospital with only slight dysarthria. On November 20, 1984, he was admitted to our hospital again, because he was found unresponsive on the floor. He was somnolent but arousable. Examination showed disorientation, impairment of recent memory and impairment of calculation, A CT scan demonstrated three small intracerebral hemorrhages in the left frontal, right parietal lobes and left basal ganglia. On the fifth hospital day he deteriorated acutely, becoming semicomatose and hemiparetic on the right side. A repeated CT scan showed two new intracerebral hemorrhages in the left frontal lobe. Cerebral angiograms showed only minimal changes due to the occupying lesions in the above mentioned area. The hematomas was evacuated via left fronto-parietal craniotomy. The specimens removed with hematoma (stained with hematoxylin-eosin, Congo red and thioflavin T) showed extensive amyloid angiopathy. Postoperatively he made a good recovery, although he had residual mental dysfunctions. He expired by pneumonia on March 30, 1985.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intraoperative use of real-time ultrasonography in neurosurgery: with special reference to head injury and intracerebral hemorrhage]

We used B mode ultrasonography during 61 craniotomies performed in the acute stage after head injury, intracerebral hemorrhage, ruptured cerebral aneurysm and so on. We examined intracerebral lesions, new hemorrhages near the operative field, and contralateral hemorrhages appearing simultaneously intraoperatively. The resolution with ultrasonography was similar to that of CT and had few obstructing artifacts. It was easy to use and very useful for diagnosing abnormal intracranial mass lesions during craniotomy, mainly for the acute stage of head injury or intracerebral hemorrhage.     

Surgical considerations in cerebral amyloid angiopathy

In cerebral amyloid angiopathy, the contractile elements of the leptomeningeal and cortical arteries are replaced by noncontractile amyloid beta protein. The incidence of amyloid angiopathy increases with advancing age. It is associated with Alzheimer's disease and spontaneous cerebral hemorrhage. The latter can have the characteristic acute computed tomographic appearance of a hematoma at the cortex-white matter junction with extension of blood into the subarachnoid, subdural, and intraventricular spaces. Multiple hemorrhages are frequent. Additional bleeding can occur after evacuation of the hematoma, and postoperative hemorrhage can occur after cortical biopsy. To elucidate the role of surgery in this condition, we have reviewed 20 consecutive operated cases of cerebral amyloid angiopathy. A first group of 8 patients with senila dementia underwent cortical biopsy without resultant hemorrhage. A second group of 6 patients in good clinical condition had delayed evacuation of a spontaneous cerebral hematoma from cerebral amyloid angiopathy because of the radiological misdiagnosis of a hemorrhage within a tumor. One patient died of a pulmonary embolism, and another had subsequent multiple hemorrhages that were ultimately fatal. A third group of 6 patients in poor neurological condition had the acute evacuation of a spontaneous cerebral hematoma to relieve intracranial hypertension. All died or were severely disabled. One had repeated hemorrhages which added a progressively more severe organic dementia onto an initial hemiplegia.(ABSTRACT TRUNCATED AT 250 WORDS)     

Outcome of cerebral amyloid angiopathic brain haemorrhage

Background  Abnormal amyloid protein can be deposited in the wall of cerebral arteries leading to fragility and intracerebral haematoma in patients with cerebral amyloid angiopathy. Diagnosis can be done only histologically. The indication of surgically treating intracerebral haemorrhage caused by amyloid angiopathy is controversial. There are studies showing a high mortality and a high rate of recurrent bleeding. Others show almost no recurrent bleeding and a very low mortality and a third party states that even when recurrent intracerebral haemorrhage occurs, re-evacuation should be performed. In the present retrospective study a population of 99 patients suffering from cerebral amyloid angiopathy-related cerebral haemorrhage has been studied, to investigate the surgical outcome. Method  Ninety-nine patients were histologically diagnosed with cerebral amyloid angiopathy in our department from 1991–2004. The outcome has been established by the Glascow Outcome Score. Findings  It could be shown that intraventricular bleeding and age >75 years increased the mortality after operative evacuation. Recurrent bleeding occurred in 22% of patients. After re-evacuation at least half of the patients survived leading to the suggestion to re-operate a recurrent bleeding since patients have a chance to survive even when the Glascow Outcome Score is 3. The overall mortality in the observed population was 16% and 11% had a very good neurological recovery based on a Glascow Outcome Score of 4–5. The operative outcome in amyloid angiopathy related intracerebral haemorrhage is similar to this of intracerebral haemorrhage induced by other causes like hypertensive bleeding. Conclusions  Possible cerebral amyloid angiopathy is no contraindication for evacuation of brain-haematoma, and especially not in patients younger than 75 years old without an intraventricular haemorrhage.     

Clinical and cerebral blood flow studies in patients with intracranial hemorrhage and amyloid angiopathy typical of Alzheimer's disease

Spontaneous intracerebral hemorrhages can occur in patients with severe amyloid angiopathy and other morphological signs of Alzheimer's disease (AD). We observed 15 patients in whom histological examination of brain tissue specimens obtained at surgery revealed characteristic congophilic amyloid deposits in subcortical arteries and/or nerve cells. Clinical follow-up examinations were carried out up to 9 years after diagnosis. In addition, three survivors from the operated group were investigated by neuropsychological testing and single photon emission computer tomography (SPECT) using Tc-99m-HMPAO for determination of regional cerebral blood flow (rCBF).SPECT could not differentiate between the typical Alzheimer disease pattern of bilateral temporo-parietal rCBF reduction and flow deficits resulting from previous hemorrhage. Intellectual functioning was found to be impaired to various degrees ranging from normal function to severe dementia (MMS test scores varied between 15 and 26 points); again, it was difficult to differentiate clinically between the nosologic entities mentioned above.On the basis of our present experience we cannot distinguish between brain dysfunction due to Alzheimer's disease and intracranial hemorrhage from amyloid angiopathy. This supports the idea that intracranial hemorrhage may only be one clinical manifestation of amyloid deposits, another one being Alzheimer's disease with varying preponderance.     

Thrombosis of cortical vein: a case report

A 39-year-old woman presented with disturbed consciousness, left hemiparesis and headache. CT scan revealed subcortical hemorrhage in the right parietal region, which had lower density than usual intracerebral hemorrhage and was associated with irregular perifocal edema. Cerebral angiograms revealed narrowed cortical vein with irregular wall in the right parietal region, where delayed local blood flow into the deep cerebral veins through collateral vessels was noted. The diagnosis was subcortical hemorrhage due to thrombosis of cortical vein. Her symptoms and brain edema were progressive. To reduce intracranial pressure, evacuation of the hematoma was performed. The hematoma which existed 1cm under the right parietal cortex was about 20g in weight. She remarkably improved soon after operation. Frequency of sinovenous thrombosis has been reported to be about 10% of all cerebral ischemic diseases, however, localized venous thrombosis is rare among them. This is because it is not always symptomatic and it is difficult to make accurate diagnosis. Six cases of localized venous thrombosis were reviewed, in which accurate diagnosis were established by cerebral angiograms, CT scan and/or autopsy. Clinical and radiological features of this case were presented.     

Cerebral amyloid angiopathy presenting as transient ischemic attacks. Case report

Cerebral amyloid angiography can cause ischemic stroke and transient ischemic attacks (TIA's), as illustrated by this case report and literature review. It is possible that the use of anticoagulant or platelet-antiaggregant drugs in elderly patients with TIA's but no angiographic abnormalities may increase the risk of hemorrhage from unsuspected amyloid angiopathy.     

Cerebral amyloid angiopathy is difficult to diagnose in the intensive care unit

Cerebral amyloid angiopathy is a common cause of intracerebral haemorrhage in elderly patients. The diagnosis of cerebral amyloid angiopathy is based on the Boston criteria combining clinical and radiological criteria with no other cause of intracerebral haemorrhage. We describe the case of a 60-year-old female admitted to the intensive care unit for agitation and spatial disorientation. She had multiple intracerebral haematomas on brain CT scan. Typical cerebral microbleeds using MRI and the absence of other cause of intracerebral haemorrhage argued in favour of the diagnosis of cerebral amyloid angiopathy. The patient outcome was favourable with a discharge from the intensive care unit on day 16.     

Cerebral amyloid angiopathies

PATHOLOGY FINDINGS: Cerebral amyloid angiopathies are defined by the presence of amyloid deposits on the walls of cerebral vessels. These amyloid deposits are found in the media of arterioles of the leptomeninges and the cortex. They are sometimes associated with Alzheimer-type lesions. Overt amyloid vasculopathy characterized by lesions of the vascular wall with a media totally replaced by amyloid substance may be observed. CLINICAL EXPRESSION: Lobular hemorrhage, often with recurrent episodes is the most frequent manifestation of cerebral amyloid angiopathy. Cerebral infarcts or leukoencephalopathy may also be observed. An association with cerebral angiitis has been reported in a few cases. SPORADIC OR FAMILIAL DISEASE: Several types can be distinguished depending on the sporadic or familiar nature of the disease pattern as well as the chemical make-up of the protein deposit. In sporadic cerebral amyloid angiopathy the deposit is composed of protein A beta, and in familial cases, of protein A beta, cystatin C, gelsosine or transthyretin. Mutation of the genes coding for amyloid substance constituents have been identified in diverse forms of familial cerebral amyloid angiopathy.     

Incidence and etiology of intracerebral hemorrhage following carotid endarterectomy

In a consecutive series of 1930 carotid endarterectomies there were eight cases of postoperative intracerebral hemorrhage. One of these patients was operated on 2 weeks following cerebral infarction and had severe uncontrollable hypertension after surgery. A second patient had an intraoperative embolus and bled while fully heparinized on the 3rd postoperative day. Only one patient in the series bled into an area of documented cerebral infarction. The remainder of the cases represented hemorrhage into essentially normal brain. Seven of the eight patients with intracerebral hemorrhage had high-grade internal carotid artery stenosis preoperatively. Although several factors have contributed to the brain hemorrhages in this series of patients, postoperative cerebral hyperperfusion which often follows endarterectomy may have played an important role. Defective cerebrovascular autoregulation in chronically ischemic brain regions may predispose patients to intracerebral hemorrhage after removal of a high-grade stenosis of the internal carotid artery.     

Cerebral hemorrhage from amyloid angiopathy and coronary thrombolysis.

Coronary thrombolysis with streptokinase or tissue plasminogen activator is useful for the treatment of acute myocardial infarction in selected patients. This treatment is associated with local hemorrhagic complications and age-related cerebral hemorrhage. Coronary thrombolysis is contraindicated in patients with transient cerebral ischemia and stroke, arterial hypertension, cerebral trauma, cerebral aneurysms, and arteriovenous malformations, because of the risk of cerebral hemorrhage. We report the occurrence of a cerebral hemorrhage related to cerebral amyloid angiopathy in a patient who underwent thrombolysis and treatment with heparin for acute myocardial infarction. Despite normal coagulation parameters, the cerebral hematoma enlarged over 36 hours, as documented by sequential computed tomographic scans, to produce significant mass effect, which prompted surgical evacuation. Histological examination of the resected specimen demonstrated the strong affinity for Congo red and yellow-green birefringence that are characteristic of cerebral amyloid angiopathy. Hemostasis was difficult to achieve, as the divided or disrupted amyloid-laden cortical vessels failed to vasoconstrict, their contractile elements replaced by amyloid beta protein. The patient died of recurrent myocardial ischemia 3 days postoperatively. The incidence of cerebral amyloid angiopathy increases with advancing age. It must be considered as a potential source of cerebral hemorrhage in elderly patients undergoing thrombolysis for cardiac ischemia. Such an occurrence presents a difficult challenge because cardiac function is compromised, the coagulation profile may be altered, the cerebral hematoma is life threatening, and intracranial hemostasis is difficult to achieve.