Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment.

BACKGROUND: The management of unruptured intracranial aneurysms is controversial. Investigators from the International Study of Unruptured Intracranial Aneurysms aimed to assess the natural history of unruptured intracranial aneurysms and to measure the risk associated with their repair. METHODS: Centres in the USA, Canada, and Europe enrolled patients for prospective assessment of unruptured aneurysms. Investigators recorded the natural history in patients who did not have surgery, and assessed morbidity and mortality associated with repair of unruptured aneurysms by either open surgery or endovascular procedures. FINDINGS: 4060 patients were assessed-1692 did not have aneurysmal repair, 1917 had open surgery, and 451 had endovascular procedures. 5-year cumulative rupture rates for patients who did not have a history of subarachnoid haemorrhage with aneurysms located in internal carotid artery, anterior communicating or anterior cerebral artery, or middle cerebral artery were 0%, 2. 6%, 14 5%, and 40% for aneurysms less than 7 mm, 7-12 mm, 13-24 mm, and 25 mm or greater, respectively, compared with rates of 2 5%, 14 5%, 18 4%, and 50%, respectively, for the same size categories involving posterior circulation and posterior communicating artery aneurysms. These rates were often equalled or exceeded by the risks associated with surgical or endovascular repair of comparable lesions. Patients' age was a strong predictor of surgical outcome, and the size and location of an aneurysm predict both surgical and endovascular outcomes. INTERPRETATION: Many factors are involved in management of patients with unruptured intracranial aneurysms. Site, size, and group specific risks of the natural history should be compared with site, size, and age-specific risks of repair for each patient.

Persistent chorea triggered by hyperglycemic crisis in diabetics.

Five female patients developed chorea concurrent with, or shortly after a hyperglycemic episode (admission glucose values 500-1,000 mg/dL). In four of these five patients, there was no prior history of diabetes mellitus. The chorea continued despite correction of blood glucose and persisted to the time of last follow-up, 6 months to 5 years later. The chorea developed subacutely over 2 days to 1 month and was generalized in one, unilateral in three, and involved right > left lower extremity in the other; the severity initially reached ballistic proportions in two. Associated clinical features were nil in four of these patients, but cognitive impairment and personality change occurred in one. The histories and laboratory studies identified no predisposing factors other than the hyperglycemia. The chorea was sufficiently troublesome to require administration of neuroleptic medication in all five cases. Four of the five cases had high signal intensity within basal ganglia on T1-weighted magnetic resonance (MR) imaging, as has previously been described; however, this was not seen in one case (who had the most severe clinical condition). Most previously described cases have involved a reversible clinical syndrome, in contrast to our patients. The pathogenic mechanisms remain uncertain.

A prospective, randomized, double-blind, placebo-controlled study of orbital radiotherapy for Graves' ophthalmopathy.

CONTEXT: Although widely used for more than 85 years, the efficacy of radiotherapy for Graves' ophthalmopathy (GO) has not been established convincingly. OBJECTIVE: To evaluate the efficacy of radiotherapy for GO. DESIGN: Prospective, randomized, internally controlled, double-blind clinical trial in a tertiary care academic medical center. PARTICIPANTS: The patients were ethnically diverse males and females over age 30 seen in a referral practice. The patients had moderate, symptomatic Graves' ophthalmopathy (mean clinical activity score, 6.2) but no optic neuropathy, diabetes, recent steroid treatment, previous decompression, or muscle surgery. Forty-two of 53 consecutive patients were enrolled after giving informed consent and fulfilling study entry criteria. Eleven eligible patients declined to participate because of inconvenience, desire for alternative therapy, or concern about radiation. INTERVENTION: One randomly selected orbit was treated with 20 Gy of external beam therapy; sham therapy was given to the other side. Six months later, the therapies were reversed. MAIN OUTCOME MEASURES: Every 3 months for 1 year, we measured the volume of extraocular muscle and fat, proptosis, range of extraocular muscle motion, area of diplopia fields, and lid fissure width. Effective treatment for GO will modify one or more of these parameters. RESULTS: No clinically or statistically significant difference between the treated and untreated orbit was observed in any of the main outcome measures at 6 months. At 12 months, muscle volume and proptosis improved slightly more in the orbit that was treated first. CONCLUSIONS: In this group of patients, representative of those for whom radiotherapy is frequently recommended, we were unable to demonstrate any beneficial therapeutic effect. The slight improvement noted in both orbits at 12 months may be the result of natural remission or of radiotherapy, but the changes are of marginal clinical significance.

Spontaneous involution of pilocytic astrocytoma in a patient without neurofibromatosis type 1: case report.

Serial magnetic resonance imaging findings are described in a patient with a sporadically occurring pilocytic astrocytoma that underwent spontaneous regression over 6 years. To the authors' knowledge, this is the first report in which spontaneous involution of a pilocytic astrocytoma not associated with neurofibromatosis type 1 has been described. A literature review regarding sporadic and syndrome-associated pilocytic astrocytoma was undertaken, with particular reference to treatment and natural history.

Electronic imaging impact on image and report turnaround times.

We prospectively compared image and report delivery times in our Urgent Care Center (UCC) during a film-based practice (1995) and after complete implementation of an electronic imaging practice in 1997. Before switching to a totally electronic and filmless practice, multiple time periods were consistently measured during a 1-week period in May 1995 and then again in a similar week in May 1997 after implementation of electronic imaging. All practice patterns were the same except for a film-based practice in 1995 versus a filmless practice in 1997. The following times were measured: (1) waiting room time, (2) technologist's time of examination, (3) time to quality control, (4) radiology interpretation times, (5) radiology image and report delivery time, (6) total radiology turn-around time, (7) time to room the patient back in the UCC, and (8) time until the ordering physician views the film. Waiting room time was longer in 1997 (average time, 26:47) versus 1995 (average time, 15:54). The technologist's examination completion time was approximately the same (1995 average time, 06:12; 1997 average time, 05:41). There was also a slight increase in the time of the technologist's electronic verification or quality control in 1997 (average time, 7:17) versus the film-based practice in 1995 (average time, 2:35). However, radiology interpretation times dramatically improved (average time, 49:38 in 1995 versus average time 13:50 in 1997). There was also a decrease in image delivery times to the clinicians in 1997 (median, 53 minutes) versus the film based practice of 1995 (1 hour and 40 minutes). Reports were available with the images immediately upon completion by the radiologist in 1997, compared with a median time of 27 minutes in 1995. Importantly, patients were roomed back into the UCC examination rooms faster after the radiologic procedure in 1997 (average time, 13:36) than they were in 1995 (29:38). Finally, the ordering physicians viewed the diagnostic images and reports in dramatically less time in 1997 (median, 26 minutes) versus 1995 (median, 1 hour and 5 minutes). In conclusion, a filmless electronic imaging practice within our UCC greatly improved radiology image and report delivery times, as well as improved clinical efficiency.

Rathke cleft cyst: CT, MR, and pathology of 23 cases.

OBJECTIVE: We report the radiologic findings in 23 cases of Rathke cleft cyst (RCC) and correlate them with the histopathology. MATERIALS AND METHODS: We reviewed the radiology and pathology of 23 cases of surgically treated RCC operated upon at our institution or referred in consultation. RESULTS: There appears to be a correlation between the MR and CT appearance of the cyst, the gross appearance of the cyst contents, and the histopathologic characteristics of the cyst lining. Some of the lesions demonstrated peripheral enhancement, which in two cases was clearly due to a peripherally displaced rim of pituitary tissue. CONCLUSION: The appearance of RCC with CT and MRI is variable, and radiologic diagnosis can be difficult. Imaging features such as a sellar epicenter, smooth contour, absence of calcification, absence of internal enhancement, and homogeneous attenuation or signal intensity within the lesion suggest the diagnosis of RCC. Rim enhancement does not correlate with the presence of squamous metaplasia, hemosiderin, or cholesterol within the cyst wall and is not consistently seen in cases with changes of mild, chronic inflammation. In some cases, rim enhancement is due to a peripherally displaced rim of pituitary tissue.

Use of a neural network and a multiple regression model to predict histologic grade of astrocytoma from MRI appearances.

Several MRI features of supratentorial astrocytomas are associated with high histologic grade by statistically significant p values. We sought to apply this information prospectively to a group of astrocytomas in the prediction of tumor grade. We used 10 MRI features of fibrillary astrocytomas from 52 patient studies to develop neural network and multiple linear regression models for practical use in predicting tumor grade. The models were tested prospectively on MR images from 29 patients studies. The performance of the models was compared against that of a radiologist. Neural network accuracy was 61% in distinguishing between low and high grade tumors. Multiple linear regression achieved an accuracy of 59%. Assessment of the images by a radiologist yielded 57% accuracy. We conclude that while certain MRI parameters may be statistically related to astrocytoma histologic grade, neural network and linear regression models cannot reliably use them to predict tumor grade.

Magnetic resonance imaging of facial vascular anomalies.

In 36 patients, facial vascular anomalies were studied with 46 magnetic resonance (MR) examinations, 9 angiograms, and 5 computed tomographic scans. All lesions were categorized into classic pathologic groups on the basis of radiologic and pathologic studies, clinical examination, and behavior. Overall, 2 juvenile hemangiomas, 3 capillary malformations (port-wine stains), 18 venous malformations, 9 lymphatic malformations, and 4 arteriovenous malformations were found. MR imaging was superior to computed tomography and angiography for demonstrating the precise anatomic extent of the facial vascular anomalies and their relationship to the adjacent soft tissues but was inferior to computed tomography for demonstrating radiopaque structures such as trophic bone changes and phleboliths. MR imaging was also inferior to angiography in determining the nidus and the exact nature of collateral vascular structures in arteriovenous malformations. MR studies confirmed the clinically suspected diagnosis of facial vascular anomalies and demonstrated typical characteristics for each type of lesion. MR imaging is an ideal initial technique to triage patients with facial vascular anomalies for appropriate management, including observation, endovascular therapy, or surgical excision.

Embolization of spinal dural arteriovenous fistula with polyvinyl alcohol particles: experience in 14 patients.

PURPOSE: To assess the effectiveness, initial and long term, of embolization using polyvinyl alcohol particles (PVA) particles in patients with spinal dural arteriovenous fistulas (SDAVF). METHODS: The initial treatment in 14 patients with SDAVF was embolization with PVA particles. Postembolization occlusion of the SDAVF was documented angiographically in all cases. RESULTS: All 14 patients initially showed an improvement in clinical symptoms, but the neurologic status of 11 patients subsequently deteriorated. Of the 11 patients who had recurrence of symptoms, nine had angiographically proven recurrences of their SDAVF while two had negative follow-up of spinal angiography. PVA embolization is ineffective for the treatment of SDAVF and is not without risk. CONCLUSION: On the basis of a literature review of the other two alternative treatments (operation or liquid adhesive embolization) available for SDAVF and our preliminary experience with N-butyl cyanoacylate (NBCA) embolization, we suggest that NBCA embolization be the initial treatment of choice for SDAVF; if that procedure is not possible, or is unsuccessful, then operation is recommended.

Symptomatic subependymoma: a clinicopathological and flow cytometric study.

Twenty-one intracranial subependymomas were reviewed with regard to presentation, diagnosis, operative findings, and long-term follow-up data. The histopathological features were critically reviewed, and deoxyribunucleic acid analysis was performed by flow cytometry. The patients' mean age was 48.5 years (range 32 to 72 years). In 14 cases the tumor was located in the fourth ventricle, in six within a lateral ventricle, and in one in the third ventricle with extension into the lateral ventricle. Radiographic characteristics included isodensity with minimal enhancement on computerized tomography, frequent dystrophic calcification, and isointensity on T1-weighted or slight hyperintensity on T2-weighted magnetic resonance images. The predominant histological features in all cases were those of classic subependymoma. Nonetheless, pathological examination showed a minor (less than 20%) ependymoma component in five cases, significant cytological atypia in seven, mitoses in 11, endothelial prominence in four, and focal hemorrhage-associated necrosis in two. Flow cytometry revealed a diploid pattern in 12 patients, tetraploidy in two, and aneuploidy in one. Two patients died in the perioperative period. Of the remaining 19, 12 underwent gross total resection (two of whom received postoperative irradiation) and seven underwent subtotal resection (five of whom received irradiation). None of the 12 non-irradiated patients developed tumor progression or died of direct tumor-related causes. Of the seven irradiated patients, follow-up imaging studies demonstrated their tumors to be radioresponsive, particularly with doses of 5000 cGy or greater. Despite the presence of cytological atypia and mitotic activity in the majority of cases, the prognostic effects of such factors as tumor location and the extent of surgical resection outweighed those of the standard histopathological parameters. Routine postoperative irradiation is not recommended, but should be reserved for cases with a symptomatic residual or recurrent subependymomas following surgery.

"Angioglioma" and the arteriovenous malformation-glioma association.

The term "angioglioma" denotes a highly vascular glioma, most of which are low-grade lesions associated with a favorable prognosis. The authors encountered an example of this pathology, a cystic oligodendroglioma associated with prominent vasculature which both clinically and histologically mimicked an occult arteriovenous malformation (AVM). This case and reports of the association of AVM and glioma prompted a histological review of 1034 surgically resected AVM's, both angiographically occult and visible, among which no oligodendroglial or astrocytic forms of "angioglioma" were found. Eight cases were observed, however, wherein oligodendroglial cells were increased in number within or about the malformation. Two basic histological patterns of oligodendroglial cell excess were seen; one appeared to be malformative in nature with abnormal disposition of oligodendroglial cells being an integral part of the AVM, whereas in the other an apparent increase in cellularity seemed the result of chronic ischemia with condensation of white matter. It appeared that the areas of increased oligodendrocyte content seen in association with AVM are non-neoplastic lesions that exhibit two rather distinct histological patterns of differing origin. In an effort to determine the frequency of "angioglioma," the authors examined Tissue Registry data for several glioma groups in which highly vascular examples are prone to occur. Tumors selected for study included 104 cerebellar-type (pilocytic) astrocytomas, 82 oligodendrogliomas, and 51 supratentorial pilocytic astrocytomas. Histological hypervascularity mimicking a vascular malformation (that is, an "angioglioma") was encountered in 5%, 4%, and 12% of the cases, respectively. Based upon clinical, radiological, and pathological reviews of these cases, as well as a careful review of the literature, it was concluded that 1) "angiogliomas" are neither rare nor represent a distinct clinicopathological entity; 2) in histological but not necessarily angiographic surgical terms, they represent simply highly vascular gliomas, usually of low grade; and 3) the clinicopathological and angiographic features as well as the prognosis of such lesions do not differ from those of similar gliomas without angioma-like vasculature. Finally, "angiogliomas" must not be confused with gliomas of high-grade malignancy which, due to neovascularity, may be highly vascular at angiography and at surgery.

Multiple sclerosis: histopathologic and MR and/or CT correlation in 37 cases at biopsy and three cases at autopsy.

The authors retrospectively reviewed the cases of 40 patients with biopsy- or autopsy-proved inflammatory demyelination consistent with multiple sclerosis (MS); each patient underwent magnetic resonance (MR) and/or computed tomographic (CT) imaging. In review of the 32 MR and 30 CT examinations, three predominant radiologic patterns were apparent: (a) relatively small homogeneous lesions with no or minimal diffuse enhancement; (b) hypoattenuating lesions on CT scans and hypointense lesions on short-TR MR images, with a contrast material-enhanced, isoattenuating or isointense ring; and (c) lesions that were more infiltrative and ill defined, with mixed attenuation and signal intensity and scattered enhancement. A common radiologic feature was lack of mass effect or edema in white matter surrounding even large lesions. In all but four patients the lesions were classified as active by using both histologic and clinical criteria. Histologically active lesions had various radiologic appearances; however, in all contrast-enhanced studies they demonstrated some form of enhancement. Inactive lesions were homogeneously hypoattenuating on CT scans, hypointense on short-TR MR images, and hyperintense on unenhanced, long-TR MR images.

Intracranial fibromatosis.

Fibromatoses are uncommon infiltrative lesions affecting musculoaponeurotic structures, most often of the limbs and trunk. Lesions involving the cranial cavity are rare and require the same aggressive surgical management as elsewhere in the body. This case illustrates their clinical and neuroradiological features and underscores the necessity for aggressive resection to avoid recurrence. The literature is reviewed.

Subependymoma with rhabdomyosarcomatous differentiation: report of a case and literature review.

A unique gliomesenchymal neoplasm, consisting of a subependymoma, a form of low-grade glioma, and a rhabdomyosarcoma, is described. It arose and recurred in the brain stem of a 52-year-old man. Immunohistochemical studies demonstrated the presence of a neuroectodermal marker S-100 protein within some sarcoma cells. The occurrence of this rare tumor supports the concept that striated muscle may derive from a neuroectodermal precursor lesion.

Unruptured intracranial aneurysms and arteriovenous malformations: frequency of intracranial hemorrhage and relationship of lesions.

Among 91 patients with unruptured intracranial arteriovenous malformations (AVM's), 16 patients had 26 unruptured intracranial saccular aneurysms. An actuarial analysis showed the risk of intracranial hemorrhage among patients with coexisting aneurysm and AVM to be 7% per year at 5 years following diagnosis compared to 1.7% for patients with AVM alone. The difference in length of survival free of hemorrhage was significant (log-rank, p less than 0.0007). Several angiographic and clinical parameters were investigated to better understand the relationship of these lesions. The aneurysms occurred in similar percentages in patients with small, medium, and large AVM's. Twenty-five aneurysms were on arteries feeding the malformation system, almost equally distributed proximally and distally. Eleven aneurysms were atypical in location, and all arose from primary or secondary branch feeders to the malformation; 24 were on enlarged feeding arteries. Eleven (16%) of the 67 patients with high-flow AVM's had associated aneurysms, compared with five (21%) of the 24 patients with low-flow AVM's. Four (16%) of 25 low-shunt malformations and 12 (18%) of 65 high-shunt malformations had associated aneurysms. All five aneurysms associated with low-shunt malformations were on a direct arterial feeder of the malformation. These data suggest that the intracranial AVM's predispose to aneurysm formation within AVM feeding systems and that the mechanism is not simply based upon the high blood flow or high arteriovenous shunt in these systems.

Cerebral sparganosis. Case report.

The tapeworm Spirometra mansonoides infects man worldwide, particularly in Asian countries. Rarely, the central nervous system is involved; such a case is presented here. In the total of 12 reported cases, including the case described, the worm presented clinically as a mass suspicious for neoplasm or chronic abscess cavity. Surgical removal was invariably curative in each case. Although infrequent, the possibility of tapeworm infection should be entertained in the evaluation of intracranial masses in patients who have visited exotic locales.

Field strength in neuro-MR imaging: a comparison of 0.5 T and 1.5 T.

A study was undertaken comparing neurological magnetic resonance imaging at high (1.5 T) and mid (0.5 T) field strengths. Twenty-eight patients (20 head and 8 spine) from our routine case load volunteered to undergo two consecutive and identical MR studies on the two systems. The two MR systems were built by the same manufacturer and were equipped with essentially identical hardware and software. Individual patient studies were performed consecutively in adjacent MR suites, and pulse sequence parameters were replicated exactly at the two field strengths. One exception to this rule was that the second echo of the long TR sequence in the head was acquired with a narrow receiver bandwidth on the 0.5 T system. The resulting axial double echo long repetition time (TR) and sagittal short TR head images and sagittal short and double echo long TR spine images were graded by two blinded observers (senior staff neuroradiologists) on two levels. First, the images were graded for image quality, i.e., conspicuousness of artifacts and clarity in depiction of normal and pathologic anatomy. Second, diagnostic accuracy of MR was assessed relative to the clinical-pathologic diagnosis in each case. The image quality of the 1.5 T system was rated superior in both the head and spine for most specific items assessed. This observer preference for 1.5 T images did not, however, translate into greater diagnostic accuracy for the 1.5 T system in the head. Although the 1.5 T system did have a slight advantage in diagnostic accuracy in the spine, a significant difference was not found.

Cranial computed tomography and magnetic resonance imaging in autosomal dominant polycystic kidney disease.

High resolution computed tomography or magnetic resonance imaging of the head with 3-mm sections through the circle of Willis was obtained in 96 patients with autosomal dominant polycystic kidney disease. These scans are well suited for the noninvasive, presymptomatic diagnosis of intracranial aneurysms at high risk of rupture, since the risk of rupture of unruptured aneurysms is heavily dependent on their size, and aneurysms equal to or larger than 6 mm in diameter are not likely to escape detection by these techniques. No definite aneurysm was observed in any of the 96 patients. Small areas (2-4 mm) of contrast enhancement or signal void were detected in 11 patients. These areas are possibly due to vascular tortuosity, but the studies were considered to be indeterminate because a small aneurysm could not be ruled out. Cerebral angiography in two of these 11 patients was normal. Arachnoid cysts were observed more frequently in the patients with polycystic kidney disease than in a control group of patients matched by gender, age, and type and date of examination.

Magnetic resonance imaging of the spine.

Magnetic resonance (MR) imaging offers the advantages of multiplanar capabilities, physiologic as well as anatomic imaging, and some tissue specificity. Since mid-1985, we have performed more than 2,800 MR examinations of the spine, and gadolinium has been used as a contrast agent in many MR studies since its release by the Food and Drug Administration last year. Although MR imaging has been promoted as the procedure of choice for all clinical indications, on the basis of our experience myelography still plays an important role in the imaging of many spinal disorders, including disk disease. Some pathologic conditions such as intraspinal vascular malformations and leptomeningeal metastatic lesions are best imaged by myelography. MR studies have been most useful in evaluating intraspinal neoplasms, spinal cord parenchymal lesions, infections, congenital abnormalities, and "postoperative failed back syndromes."