Multiple intracerebral hemorrhages due to cerebral amyloid angiopathy after head trauma

Cerebral amyloid angiopathy (CAA) is an important cause of intracerebral hemorrhage and ischemic cerebrovascular disease in some normotensive elderly patients. The diagnosis is made by proof of amyloid deposition in the vessel wall. A case of recurrent and multiple intracerebral hemorrhages due to cerebral amyloid angiopathy after head injury is reported. A 74-year-old female was referred to our hospital because of head injury. CT scan showed traumatic subarachnoid hemorrhage and intraventricular hematoma. Her consciousness was clear but slight disorientation was recognized. Conservative therapy was performed. During the course subcortical hemorrhages occurred five times and during the second one, right frontal and right parietal hemorrhages occurred simultaneously. Her consciousness deteriorated. The second subcortical hemorrhage was especially complicated by a ventriculoperitoneal shunt operation. A biopsy of the cortex was performed and pathological examination revealed amyloid deposition in the walls of small pial and cortical vessels. Occasional duplicated wall, obliterative intimal proliferation and disappearance of elastic lamina were recognized. The patient sank into a vegetative state due to recurrent and multiple hemorrhages. CAA results in two possibilities, hemorrhage and ischemic cerebrovascular disease. When lobar or subcortical hemorrhage is encountered in a normotensive elderly patient, the possibility of a CAA-related hemorrhage should be considered. The author carefully emphasizes that there is indication for neurosurgical treatment in CAA patients and proposes that therapy for ischemic cerebrovascular disease should be given special attention.     

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.     

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.     

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.     

Influence of apolipoprotein E and its receptors on cerebral amyloid precursor protein metabolism following traumatic brain injury

Traumatic brain injury (TBI) is the leading cause of mortality and disability among young individuals in our society,and globally the incidence of TBI is rising sharply.Mounting evidence has indicated ...     

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)     

Lobar intracerebral hemorrhage. A clinical, radiographic, and pathological study of 29 consecutive operated cases with negative angiography.

The authors operated consecutively on 50 patients with lobar intracerebral hemorrhage during a prospectively designed study period from January, 1986, to March, 1990. They investigated the correlations between the underlying causes and the clinicoradiographic features in 29 patients who showed no angiographic vascular abnormalities, in order to elucidate the operative indication for such cases. Patients with ruptured saccular aneurysm or trauma were not included in this study. There were 15 males and 14 females, ranging in age from 7 to 76 years (mean 52.4 years). Histological diagnoses of the surgical specimens were as follows: vascular malformation in nine cases (arteriovenous malformation (AVM) in six and cavernous malformation in three), microaneurysm in 11, cerebral amyloid angiopathy in six, and brain tumor in two; in the remaining case the cause was not verified histologically. The underlying cause was determined in 96.5% of cases. The mean patient age was lowest in the cavernous malformation group (27.0 years), followed by the AVM (45.8 years), microaneurysm (59.8 years), and cerebral amyloid angiopathy (70.0 years) groups. Four patients with vascular malformation (three AVM's and one cavernous malformation) had previous episodes of bleeding at the same site, whereas none of those with microaneurysms or cerebral amyloid angiopathy had such episodes. On computerized tomography (CT) scans, the round to oval hematoma was related to the presence of an AVM or cavernous malformation in contrast to microaneurysms and cerebral amyloid angiopathy. Upon infusion of contrast material, variable enhancement was seen in five (two AVM's and three cavernous malformations) of the nine vascular malformations while no enhancement was noted in any patient with microaneurysm or cerebral amyloid angiopathy at the acute stage. Subarachnoid extension of the hematoma was associated with cerebral amyloid angiopathy significantly more frequently than with AVM's (p less than 0.05) and microaneurysms (p less than 0.01). The results suggest that clinicoradiographic pictures in cases with negative angiography are quite different among the three major pathological categories; namely, vascular malformation (AVM and cavernous malformation), microaneurysm, and cerebral amyloid angiopathy. It is suggested that the underlying etiology of a given lobar intracerebral hemorrhage with negative angiography may be predicted by a combination of patient age, history of previous bleeding at the same site, hematoma shape, and subarachnoid extension of the hematoma on CT scans. Based upon these findings, the authors discuss operative indications for such cases.     

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.     

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 and moyamoya disease.

The incidence, clinical manifestations, diagnosis, and management of cerebral hemorrhage from amyloid angiopathy and moyamoya disease are reviewed. Particular attention is given to pathophysiologic mechanisms producing cerebral hemorrhage in these conditions, the diagnosis based on characteristic CT and angiographic appearances, and the role of surgery in comprehensive management.     

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.     

腹部手术对老年心脑血管病患者的影响

目的探讨老年患者腹部手术后心脑血管意外的发病特点及防治体会。 方法回顾性分析2011年1月至2013年12月本科室诊治的64例腹腔手术后发生心脑血管意外的老年患者临床资料,其中32例伴有心脑血管疾病为A组,同期无心脑血管疾病的32例行腹腔手术的患者为B组,探讨其发病的原因及防治方法。 结果A组术后11例发生了脑梗死,其中6例为脑栓塞,4例发生脑出血,心肌梗死6例,心电图有心肌缺血5例,ST段改变9例,急性心力衰竭2例,心律失常5例; B组术后3例发生了脑梗死,其中1例为脑栓塞,1例发生脑出血,心肌梗死1例,心电图有心肌缺血2例,ST段改变2例,心律失常1例。A组术后心脑血管疾病的发生率明显高于B组,组间差异有统计学意义(P < 0.05)。 结论患有腔隙性脑梗死、陈旧性脑梗死、心肌梗死、心肌缺血、ST段改变等心脑血管疾病的患者手术风险高,易出现猝死及心脑血管意外,必须引起临床医师的注意和重视。     

Neurosurgical and immunological implications of primary cerebral amyloid (congophilic) angiopathy

Two cases of histologically documented primary cerebral amyloid complicated by spontaneous intracerebral hematoma are reported. The neurosurgical implications of this disease are reviewed briefly. Immunological studies in one patient, believed to be a rare survivor of an intracerebral hemorrhage from nonfamilial primary cerebral amyloid angiopathy, demonstrated an increased percentage of B cells within both the cerebrospinal fluid (CSF) and the blood compartments. Hypofunction of CSF concanavalin A-activated suppressor cells with respect to B and T cell targets was shown. The blood suppressor cells from the patient were similar to those of an age-matched control. Compartmental central nervous system suppressor cell dysfunction resulting in B cell activation could play a role in facilitating amyloid deposits in the brain. The mechanisms responsible for the compartmental and limited suppressor cell dysfunction are unknown.     

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.     

Relationship of serum cystatin C with C-reactive protein and apolipoprotein A1 in patients on hemodialysis

Cystatin C is considered an indicator of acute renal failure and also a risk factor of cardiovascular disease. This study was undertaken to examine the relationship of serum cystatin C with C-reactive protein (CRP), lipids, and lipid-related compounds in patients on hemodialysis (HD). Cystatin C, CRP, total cholesterol, triglycerides, HDL cholesterol, LDL cholesterol, and apolipoprotein A1 and B were analyzed in serum of 30 patients on HD for 118 +/- 18 months with low-flux dialyzers, before and after HD. The results were compared with those obtained by 21 healthy individuals (NC). Multiple regression analysis was performed to evaluate the association of cystatin C concentration before HD with clinical and laboratory parameters. The results showed that cystatin C before HD was not associated with age, body mass index (BMI), or duration of HD. However, it was significantly correlated with creatinine (r = 0.435, p = 0.021) and albumin (r = 0.483, p = 0.009) concentrations. Moreover, a highly significant association was shown with logCRP (r = 0.692, p < 0.0001). Among the lipid and lipid-related compounds studied, a significant correlation was found between cystatin C and apolipoprotein A1 concentrations (r = 0.402, p = 0.034). None of those correlations were observed in the NC group. In conclusion, it seems that cystatin C levels before HD are related with CRP, an important inflammatory factor, and also with apolipoprotein A1, which has been proved to accelerate the atherosclerosis process. However more studies are needed to confirm these findings.     

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)     

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.     

Alterations in cerebrospinal fluid apolipoprotein E and amyloid beta-protein after traumatic brain injury

There is evidence that apolipoprotein E (apoE) and amyloid beta-protein (Abeta), which are implicated in the pathology of chronic neurodegenerative disorders, are involved in the response of the brain to acute injury; however, human in vivo evidence is sparse. We conducted a prospective observational study to determine the magnitude and time-course of alterations in cerebrospinal fluid (CSF) apoE and Abeta concentrations after traumatic brain injury (TBI), and the relationship of these changes to severity of injury and clinical outcome. Enzyme linked immunosorbant assay (ELISA) was used to assay apoE, Abeta(1-40) and Abeta(1-42) in serial CSF samples from 13 patients with TBI and 13 controls. CSF S100B and tau were assayed as surrogate markers of brain injury. There was a significant decrease in CSF apoE (p < 0.001) and Abeta (p< 0.001) after TBI contrasting the observed elevation in CSF S100B (p < 0.001) and tau (p < 0.001) concentration. There was significant correlation (r = 0.67, p = 0.01) between injury severity and the decrease in Abeta(1-40) concentration after TBI. In vivo, changes in apoE and Abeta concentration occur after TBI and may be important in the response of the human brain to injury.     

Presence of apolipoprotein E epsilon4 allele in cerebral palsy

The apolipoprotein E gene, which is located on chromosome 19, has three alleles (epsilon2, epsilon3, and epsilon4). Several recent publications associate the presence of the apolipoprotein E epsilon4 allele with the occurrence of neurologic diseases, and consider it a risk factor for the development of central nervous system affections. A group of 40 patients with cerebral palsy was studied and compared to a control group of 40 subjects, and higher occurrence of the allele epsilon4 in the group of subjects with cerebral palsy was observed. A significantly higher risk of developing cerebral palsy was demonstrated among those subjects with the apolipoprotein E epsilon4 allele.