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.     

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.     

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.     

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)     

Surgical experience with massive lobar haemorrhage caused by cerebral amyloid angiopathy

Summary Nineteen patients with massive lobar haemorrhage without angiographic lesions received direct or stereotactic surgery, and biopsy specimens were examined histologically. Ten patients (53%) were found to have vessels positive for Congo-red staining, and demonstrating amyloid angiopathy. In the patients with amyloid angiopathy, CT scan and surgical findings were investigated. Subarachnoid haemorrhage (9/10), irregularly shaped haematoma (9/10) and fluid-blood density level in the haematoma cavity (7/10) were frequently found on CT scan. The characteristic surgical findings in patients treated by direct surgery were subarachnoid haemorrhage adjacent to intracerebral haematoma (8/8) and the existence of a tangle of vessels in the haematoma cavity (4/8). Evacuation of haematomas was relatively easy, and difficulty of haemostasis was not encountered during surgery.     

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.     

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.     

Fatal Intracerebral Haemorrhage Following Carotid Endarterectomy in a Patient with Cerebral Amyloid Angiopathy

A 68-year-old man underwent carotid endarterectomy for symptomatic carotid artery stenosis. Immediately after surgery the patient suffered dramatic neurological deterioration, due to massive cerebral bleeding. Pathological examination revealed cerebral amyloid angiopathy. This condition is known to predispose to spontaneous, as well as anticoagulation induced, cerebral haemorrhage. Surgical intervention needing anticoagulation in elderly patients at risk for congophilic angiopathy should be performed with extreme caution.     

Influence of intraventricular hemorrhage on outcome after rupture of intracranial aneurysm

This study was performed to analyze the effect of intraventricular hemorrhage (IVH) on 14-day mortality, outcome at 6 months, and the occurrence of chronic hydrocephalus in patients with aneurysmal subarachnoid hemorrhage. Clinical grade of subarachnoid hemorrhage and the distribution of extravasated blood were evaluated in 219 patients with ruptured aneurysms. Computed tomographic scans performed within 72 h of hemorrhage were analyzed to determine the severity of intraventricular and subarachnoid hemorrhage and the volume of intracerebral hematomas. Outcome at 6 months was assessed using the Glasgow Outcome Scale. Intraventricular hemorrhage extension occurred in 109 of the 219 patients studied. Fourteen-day mortality increased from 7.3% in patients without IVH to 14.1% in those with moderate IVH (IVH score 1–6) and to 41.7% in those with more severe IVH (IVH score >6). The corresponding figures for unfavorable outcome at 6 months are 19.8%, 30.5%, and 66.7%, respectively. According to logistic regression analyses, the severity of IVH was an independent predictor of mortality and functional outcome. The clinical outcome after aneurysm rupture is at least in part determined by the severity of IVH. Knowledge of the effect of IVH may help guide physicians in the care of patients with aneurysmal bleeding. Electronic Publication     

Outcome after emergency surgery without angiography in patients with intracerebral haemorrhage after aneurysm rupture

Background  Patients with large intracerebral haematomas (ICH) from aneurysm rupture often present in a poor clinical condition and have a poor prognosis. Time delay for preoperative angiography might in some cases be unappealing. We evaluated the outcome after immediate haematoma removal and aneurysm occlusion without preoperative angiography. Methods  We retrospectively identified 13 consecutive patients. We recorded clinical data and evaluated mortality and morbidity with the Glasgow Outcome Score (GOS) and Telephone Interview of Cognitive Status (TICS). Findings  At follow up seven of thirteen patients had favourable outcome assessed by GOS. Three patients had severe disability and three patients died. None of the survived patients interviewed had impaired cognition. Conclusions  In patients presented in a critical state with aneurysmal ICH, emergency haematoma removal and aneurysm clipping without the delay for diagnostic angiography may be life saving and a satisfactory outcome can be accomplished.     

Influence of intraventricular hemorrhage on outcome after rupture of intracranial aneurysm.

This study was performed to analyze the effect of intraventricular hemorrhage (IVH) on 14-day mortality, outcome at 6 months, and the occurrence of chronic hydrocephalus in patients with aneurysmal subarachnoid hemorrhage. Clinical grade of subarachnoid hemorrhage and the distribution of extravasated blood were evaluated in 219 patients with ruptured aneurysms. Computed tomographic scans performed within 72 h of hemorrhage were analyzed to determine the severity of intraventricular and subarachnoid hemorrhage and the volume of intracerebral hematomas. Outcome at 6 months was assessed using the Glasgow Outcome Scale. Intraventricular hemorrhage extension occurred in 109 of the 219 patients studied. Fourteen-day mortality increased from 7.3% in patients without IVH to 14.1% in those with moderate IVH (IVH score 1-6) and to 41.7% in those with more severe IVH (IVH score > 6). The corresponding figures for unfavorable outcome at 6 months are 19.8%, 30.5%, and 66.7%, respectively. According to logistic regression analyses, the severity of IVH was an independent predictor of mortality and functional outcome. The clinical outcome after aneurysm rupture is at least in part determined by the severity of IVH. Knowledge of the effect of IVH may help guide physicians in the care of patients with aneurysmal bleeding.     

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.     

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.     

Aneurysms with severe clinical manifestations (Hunt and Hess grade IV and V). A series of 66 cases

Among 250 patients consecutively admitted in our center with a ruptured intracranial aneurysm, 66 patients (24.4%) were initially classified in Hunt and Hess clinical grade IV (37 cases) or grade V (29 cases). All patients were studied as following: --The severity of subarachnoid haemorrhage was evaluated on the pre-operative C.T. scan using Fisher's criteria. Quantification of the intracerebral haematoma or the intraventricular associated haemorrhage or a subdural haematoma was estimated as well. --The arterial diameter, aneurysm size and location, and the eventual presence of intra-arterial embolus were noted on the pre-operative angiography. Aneurysm location was: anterior artery 27 cases, internal carotid artery 9 cases, middle cerebral artery 27 cases, posterior cerebral artery 2 cases, and 1 case on the basilar artery. In this series, a poor clinical condition on admission was related to the direct effect of the initial haemorrhage in 84.9% of the patients. Early seizures (7.6%) acute hydrocephalus (1.5%), multiple emboli (3%) and apparently early diffuse vasospasm (1.5%) were the other documented causes explaining the initial poor clinical condition. One case remained completely unexplained. Sixteen patients admitted with bilateral fixed dilated pupils or a major intracerebral haematoma from a ruptured anterior artery aneurysm were not operated on and subsequently died. Operative treatment (aneurysm clipping in all cases, and haematoma evacuation on demand) was performed in the remaining 50 cases within 12 hours after their admission. Thus, these patients underwent surgery on Day 0 in 31 cases, on Day 1 in 11 cases, and on Day 2 in 8 cases. A post-operative C.T. scan was performed in 46 cases. Post-operative angiographic control was only performed in 29 cases. Changes on the post-operative C.T. scan or the angiographic control were strictly compared to the neuroradiological information previously available. Final outcome was assessed at least two months after the onset. According to the Glasgow Outcome Score, the results were: good recovery 12 patients (18.2%); moderate disability 1 patient; severe disability 4 patients (6%); vegetative state 4 patients; death 45 patients (68.2%). Excluding the patients admitted in poor clinical grade but presenting with early seizures or minor initial haemorrhage, the mortality rate was 74.2%. According to the initial clinical grade, the initial C.T. scan findings, the eventual post-operative angiographic presence of an arterial thrombosis or vasospasm, it was obvious that the final bad outcome was mainly related to the severity of the initial haemorrhage. However, in 7 patients, post-operative disability or death can be explained by other complications, principally an arterial thrombosis.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

Modern treatment options for intracerebral hemorrhage

Opinion statement Spontaneous intracerebral hemorrhage (ICH) is a devastating neurological event with a 30-day mortality of approximately 40%. Recent research provides new insights into the pathophysiology of ICH-associated edema, with potential molecular and cellular targets for future therapy. Neuroimaging techniques such as gradient echo MRI are yielding insights into cerebral microbleeds and the microangiopathies associated with hypertension and cerebral amyloid angiopathy. Recent literature provides new medical treatment strategies for fever, acute hypertension, and perihematomal edema, and methods of reducing intracranial pressure. Two randomized controlled trials have provided crucial evidence regarding surgical and medical intervention for acute ICH intervention. Recombinant factor VIIa appears to lessen growth of ICH when administered within 4 hours of ictus. Further study of potential efficacy and safety is underway in an international phase III trial. In addition, the Surgical Trial in Intracerebral Hemorrhage reported results from an international randomized trial of 1033 patients who did not show benefit for surgical evacuation of ICH, compared with medical therapy alone. Less invasive surgical methods for hematoma evacuation, studied previously over the past decade, continue to be investigated.     

Fibrinolytic treatment of intraventricular haemorrhage preceding surgical repair of ruptured aneurysms and arteriovenous malformations

Previous studies have indicated that intraventricular administration of tissue-type plasminogen activator (TPA) might improve the prognosis of patients with intraventricular haemorrhage (IVH). In aneurysmal IVH, fibrinolytic treatment was always preceded by surgical repair of the aneurysm, since the risk of recurrent haemorrhage from a non-occluded aneurysm was estimated to be high. We reviewed a series of patients with IVH secondary to ruptured aneurysms (n = 4) or arteriovenous malformation (AVM; n = 1) who underwent emergency intraventricular administration of TPA before repair of the bleeding source. Fibrinolysis resulted in rapid decrease of haematoma volume and of ventricular dilatation, and prevented ventricular catheters from becoming obstructed. No intracranial haemorrhages or other complications occurred. The results suggest that the presence of recently ruptured aneurysms or AVM is not necessarily a contraindication for intraventricular administration of TPA. The potentially life saving benefits might outweigh the inherent risks of recurrent haemorrhage in carefully selected patients with massive IVH, in whom ventricular distension, periventricular brain compression, obstruction of CSF flow, and elevated ICP appear to be major determinants for the outcome.     

Profuse Hemorrhage from Cerebral Vessels in Tangential Missile Injuries

Summary Tangential missile injuries are recognized as a cause of cerebral damage by indriven bone fragments, parenchymal lacerations and intracerebral haematomas. Severe blood loss from this type of injury was not reported. Four patients are described in whom high kinetic energy missiles caused severe tangential craniocerebral injuries. Their condition was aggravated by life threatening haemorrhage. The bleeding vessels were cerebral arteries or major veins entrapped in the fracture line and bleeding extracranially. This blood loss cannot be controlled by dressing and salvage could be achieved only by immediate resuscitation, vigorous fluid replacement and very rapid evacuation to a neurosurgical center.     

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.     

Computed tomography of the brain in predicting outcome of traumatic intracranial haemorrhage in Malaysian patients

Summary  Head injury is a significant economic, social and medical problem all over the world. Road accidents are the most frequent cause of head injury in Malaysia with highest risk in the young (15 to 24 years old). The associated outcomes include good recovery, possibility of death for the severely injured, which may cause disruption of the lives of their family members. It is important to predict the outcome as it will provide sound information to assist clinicians in Malaysia in providing prognostic information to patients and their families, to assess the effectiveness of different modes of treatment in promoting recovery and to document the significance of head injury as a public health problem.  Results. A total of 103 cases with intracranial haemorrhage i.e. intracerebral haemorrhage, extradural haemorrhage, subdural haemorrhage, intraventricular haemorrhage, haemorrhagic contusion and subarachnoid haemorrhage, following motor vehicle accidents was undertaken to study factors contributing to either good or poor outcome according to the Glasgow Outcome Scale. Patients below 12 years of age were excluded. The end point of the study was taken at 24 months post injury.  The selected variables were incorporated into models generated by logistic regression techniques of multivariate analysis to see the significant predictors of outcome as well as the correlation between the CT findings with GCS.  Conclusion. Significant predictors of outcome were GCS on arrival in the accident emergency department, pupillary reflex and the CT scan findings. The CT predictors of outcome include ICH, EDH, IVH, present of SAH, site of ICH, volumes of EDH and SDH as well as midline shift.