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)     

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.     

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.     

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 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.     

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)     

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.     

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.     

Neurosurgical multicenter study of cerebral metastases (Neurosurgical departments of Paris Beaujon, Paris Sainte-Anne, Strasbourg)

We report a multicentric outcome study of cerebral metastases in 174 patients collected consecutively in three neurosurgical centers in France. Age and condition of patients allowed surgical operation; the sex ratio was 1.67. The revelation mode was usual. However tumoral hemorrhage frequency was 3.4%. Primitive cancers were usual cancers as in oncological series with a higher proportion of radioresistant metastases. Mean dimension was 30 mm with mean of 1. 25 metastases per patient. Supratentorial localization was more frequent and the metastasis revealed cancer in 40% of cases. Global median survival was 12.1 months. Factors correlated with the survival in our series of patients with cerebral metastases were: solitary cerebral metastasis, extracerebral extension of cancer, treatment of primitive cancer, complete excision and post operative cerebral radiotherapy.     

The incidence of cerebral amyloid angiopathy in surgically treated intracranial hemorrhage in the Chinese population

Despite being widely accepted as an important cause of spontaneous intracranial hemorrhage (ICH), cerebral amyloid angiopathy (CAA) has seldom been studied in the Chinese population. The current study aims to investigate the incidence and features of CAA in surgically treated ICH patients in China. From May 2006 to April 2011, 974 patients admitted to 71 hospitals throughout China for acute spontaneous ICH were studied. Craniotomy for hematoma evacuation was performed. Brain tissue from the superficial side of the suspected residual hematoma cavity, as well as from the cortex and subcortex, was obtained. Congo Red stain and β-amyloid immunohistochemistry were used for the diagnosis. Each case was assigned a pathological severity score. Of the 974 involved patients, 37.7 % were identified with CAA of different degrees. CAA had positive correlation with age and was independent of sex. Most patients had mild CAA with only the superficial vessels involved in lobes instead of the basal ganglia; the patients ≥65 years had more severe pathological score of CAA than those <65 years and had more lobes and cerebellum involved than the latter. More than one third of the surgically treated Chinese ICH patients may have CAA of different degrees.     

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.     

Neurosurgical implications of Carney complex

OBJECT: The authors present their neurosurgical experience with Carney complex. Carney complex, characterized by spotty skin pigmentation, cardiac myxomas, primary pigmented nodular adrenocortical disease, pituitary tumors, and nerve sheath tumors (NSTs), is a recently described, rare, autosomal-dominant familial syndrome that is relatively unknown to neurosurgeons. Neurosurgery is required to treat pituitary adenomas and a rare NST, the psammomatous melanotic schwannoma (PMS), in patients with Carney complex. Cushing's syndrome, a common component of the complex, is caused by primary pigmented nodular adrenocortical disease and is not secondary to an adrenocorticotropic hormone-secreting pituitary adenoma. METHODS: The authors reviewed 14 cases of Carney complex, five from the literature and nine from their own experience. Of the 14 pituitary adenomas recognized in association with Carney complex, 12 developed growth hormone (GH) hypersecretion (producing gigantism in two patients and acromegaly in 10), and results of immunohistochemical studies in one of the other two were positive for GH. The association of PMSs with Carney complex was established in 1990. Of the reported tumors, 28% were associated with spinal nerve sheaths. The spinal tumors occurred in adults (mean age 32 years, range 18-49 years) who presented with pain and radiculopathy. These NSTs may be malignant (10%) and, as with the cardiac myxomas, are associated with significant rates of morbidity and mortality. CONCLUSIONS: Because of the surgical comorbidity associated with cardiac myxoma and/or Cushing's syndrome, recognition of Carney complex has important implications for perisurgical patient management and family screening. Study of the genetics of Carney complex and of the biological abnormalities associated with the tumors may provide insight into the general pathobiological abnormalities associated with the tumors may provide insight into the general pathobiological features of pituitary adenomas and NSTs.     

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.     

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.     

Neurosurgical aspects of cerebral cryptococcosis

Eleven patients with raised intracranial pressure caused by cerebral cryptococcosis developed complications requiring neurosurgical operations. Two patients were fully conscious on admission, and 9 had impaired consciousness, four of whom were comatose. Seven patients were found to have hydrocephalus only. Two patients had cerebral edema initially, and 2 had cystic lesions. One of the latter developed subdural effusion. All patients eventually developed progressive hydrocephalus requiring placement of a ventriculoperitoneal shunt. Four patients were initially treated by external ventricular drainage. When external ventricular drainage was used initially, there were no associated complications; however, there was a high incidence of complications (4 of 7 patients) when a ventriculoperitoneal shunt was placed as the initial treatment. Of the 11 patients, 10 (91%) survived; of these 9 (82%) made an excellent recovery and 7 (64%) returned to their original work. The patient who died had been receiving steroid therapy for hypopituitarism.     

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.     

Neurosurgical implications of allergic fungal sinusitis

OBJECT: Allergic fungal sinusitis (AFS) is a form of paranasal mycosis that often involves bone destruction and extension into the orbit and anterior skull base. Treatment consists of surgical extirpation and a course of corticosteroids. Despite frequent intracranial involvement, AFS is rarely reported in the neurosurgical literature. METHODS: The records of 21 patients with the histological diagnosis of AFS were reviewed. The histological diagnosis was based on findings of branching septated fungi interspersed with eosinophilic mucin and Charcot-Leyden crystals without fungal invasion of soft tissue. The average age of the 21 patients in this study was 25 years (range 9-46) and the male/female ratio was 3.75:1. All patients were immunocompetent. All had a history of chronic sinusitis and imaging findings of expansile disease involving multiple sinuses. Fifteen patients had nasal polyposis, eight had erosion of bone, which was observed on computerized tomography (CT) scans, eight had disease extending intracranially, and six had disease that involved the lamina papyracea. All patients underwent transnasal and/or transmaxillary endoscopic approaches for debridement and irrigation, six underwent orbital decompression, and three underwent a bifrontal craniotomy for removal of intracranial extradural disease. No patient had a cerebrospinal fluid leak. Postoperatively, one patient was treated with amphotericin B and the other 20 were treated with a short course of corticosteroids. The follow-up period ranged from 2 to 19 years. CONCLUSIONS: Allergic fungal sinusitis is a unique form of fungal disease that may mimic anterior skull base and paranasal sinus tumors. A cranial base team approach of neurosurgeons and otolaryngologists is recommended. Most cases can be successfully managed with transnasal and/or transmaxillary endoscopic techniques. A craniotomy is rarely indicated unless there is the suspicion of dural invasion or extensive intracranial and/or intraorbital involvement that is inaccessible from below.