<PRE> INTRACEREBRAL ANEURYSMS Frequently asked questions/answers prepared by: Gary L. Bernardini, M.D., Ph.D. Department of Neurology and Neuroscience The New York Hospital/Cornell University Medical Center 1300 York Avenue NY, NY 10021 1. What is an intracerebral aneursym? An intracerebral aneurysm is a small, thin walled outpouching or dilatation of one of the large blood vessels that supply the brain. Aneurysms pose a risk to health from the potential for rupture and subsequent bleeding into the substance of the brain and/or the fluid-filled spaces that surround the brain (the subarachnoid space). These so-called saccular or berry aneurysms occur at the bifurcation of the large blood vessels at the base of the brain. 2. What causes aneuryms? Intracerebral aneurysms can result from trauma, infection, or neoplastic disease. Most aneurysms, however, result from a developmental abnormality of the inside lining or intima of an artery with abnormal thinning of the vessel at the site of origin. It appears there may be a genetic predisposition to the development of intracerebral aneurysms; the existence in some families runs as high as 10%, approximately 10 times higher than that found in the general population. There are several other causes of intracerebral aneurysms. For example, they can result from infected embolic material from a bacterial infection on one of the heart valves being deposited on one of the arteries in the brain (mycotic aneuryms). 3. Who is at greatest risk for aneurymal rupture? Aneurysmal rupture leads to subarachnoid hemorrhage (SAH) and occurs most often in patients between 40 and 60 years of age with approximately equal sex distribution. Cigarette smoking and excess alcohol use have been shown to increase the risk of rupture. Likewise, the existence of intracerebral aneurysms is associated with other diseases such as polycystic kidney disease, coarctation of the aorta, and fibromuscular hyperplasia. Other factors such as high blood pressure seem to be less important since aneurysms often occur in persons with normal blood pressure. Pregnancy has not been associated with an increased incidence of aneurysmal rupture. 4. What are the symptoms of intracerebral aneurysmal rupture? Prior to rupture, saccular aneurysms are usually asymptomatic. However, an expanding aneurysm can have a "mass" effect causing problems with double vision, loss of vision, numbness in the face, an enlarged pupil size, or a drooping eyelid. Usually patients who have an aneurysm rupture experience sudden onset of a severe headache, often described as "the worst headache of my life", frequently accompanied by transient loss of consciousness and sometimes vomiting. A stiff neck often follows. Rupture of an aneurysm usually occur while the person is active rather than during sleep. Occasionally, patients experience a warning or "sentinel" headache which is attributed to a smaller leakage of blood usually preceding a major bleed by several hours to days later. These milder headaches are often associated with nausea and vomiting and are often mistaken for migraine headaches. 5. What kind of tests do I need to determine if I have an aneurysm? Carotid and vertebral angiography is the only definitve means of demonstrating an intracerebral aneurysm, while a CT scan of the head will confirm the presence of blood within the brain or subarachnoid space if an aneurysm has ruptured. Lumbar puncture is sometimes used to evaluate for the presence of blood in the cerebrospinal fluid if the results of the CT scan are equivocal. More recently, non-invasive studies using magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) have shown promise in detection of aneurysms. However, the intracerebral angiogram remains the test of choice. 6. I have an intracerebral aneurysm. What is the risk of death from rupture? If rupture occurs, only approximately half of the patients survive. The best predictor of risk of rupture is the size of the aneurysm. Most aneurysms that rupture have a diameter equal to or greater than 10mm (about half an inch) but rupture also occurs with aneurysms of smaller size. A guide to prognosis is provided by the neurologic grade (Hunt and Hess Grades I-V) of the patient determined by his/her level of consciousness and neurologic deficits when first examined upon arrival to the hospital. In a large study of survival of patients from aneurysm rupture, a Grade of I-II (awake with slight to moderately severe headache and neck stiffness) predicted a low mortality (4%) and an independent life (up to 90%) at follow-up whereas Grades IV-V (stupor with neurological deficits to deep coma) predicted increasingly higher mortality rates (up to 46%) and decreased independent functioning (only about 30%). 7. What are the most serious complications associated with aneurysmal rupture? The developement of cerebral vasospasm, rebleeding from the aneurysm, swelling of the ventricles in the brain (hydrocephalus), and seizures may occur after rupture of an intracerebral aneurysm. Cerebral vasospasm after aneurysmal subarachnoid hemorrhage usually occurd within the first 14 days of rupture and is a major cause of morbidity and mortality in survivors of the bleed. Its incidence has varied in different studies between approximately 20 to 80% of all patients with SAH and its occurence is related to the amount of subarachnoid blood in the brain. Other complications including rebleeding from an aneurysm and hydrocephalus also contribute to the overall morbidity and mortality. In addition, dangerous cardiac arrhythmias may develop in the acute period following a bleed. 8. What are the risks of surgical repair? The timing of surgery is now recognized as an important factor in the prevention of complications associated with aneurysmal rupture. Successful early surgical clipping of a ruptured aneurysm (within the first 5 days of a bleed) helps to prevent the occurence of rebleeding, likely to be an even more catastrophic event when it occurs, and permits the safe treatment of cerebral ischemia due to vasospasm. High morbidity and mortality may occur even in low-risk patients treated with delayed operation because preoperative complications have time to develop. However, such patients operated on within the first 5 days of a bleed usually recover with no or mild neurological deficit and mortality is less than 5%. 9. What are my chances of recovery? As stated above, patients in Grades I-III can be operated on safely within 72 hours with good results. In one study of 145 patients with Hunt & Hess Grades I-III, 81% (117 patients) made a good recovery. The morbidity was 12% (17patients) and the mortality 7% (11 patients). The most common cause of unfavorable outcome was surgical complications. Other factors contributing to a worse outcome correlated with a higher age, worse Grade, and more severe SAH on CT scan. 10. What is the treatment for unruptured aneurysms? The management of asymptomatic aneurysms discovered incidentally remains controversial. A recent study followed 142 patients with unruptured aneurysms for a period of 14 years and found an average annual rupture incidence of 1.4%. The cummulative rate of bleeding from the aneurysms was 10% at 10 years, 26% at 20 years, and 32% at 30 years after the diagnosis. The surgical clipping of an intact and accesible aneurysm is usually a procedure of low risks without the future development of postoperative ischemia or vasospasm, which are often encountered with the clipping of ruptured aneurysms (leading to risk of death or disability). Surgery is usually recommended for large accessible aneurysms; but with small ones (with a proportionally lower chance of rupture) you should discuss the ratio of risk to benefit with your physicians. 11. How can I get more information on this subject? Your neurologist or neurosurgeon should be your primary resource. A FAQ like this one is very general in nature, and details about your own situation may result in the possibility that the general guidelines do not apply. For additional reading, large bookstores carry rather comprehensive reference works (like the one from the Mayo clinic). If you have access to MedLine, you can download abstracts from the primary medical literature. You will find additional pointers on the Aneurysm Information Project Home Page that will guide you to other useful resources like the Aneurysm Victims' Support Group.