The natural history of familial cavernous malformations: results of an ongoing study

1994 ◽  
Vol 80 (3) ◽  
pp. 422-432 ◽  
Author(s):  
Joseph M. Zabramski ◽  
Thomas M. Wascher ◽  
Robert F. Spetzler ◽  
Blake Johnson ◽  
John Golfinos ◽  
...  
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Ongoing Study ◽  
Mr Images ◽  
Cavernous Malformations ◽  
Content Type ◽  
Imaging Characteristics ◽  
Familial Form ◽  
Fine Print

✓ Cavernous malformations are congenital abnormalities of the cerebral vessels that affect 0.5% to 0.7% of the population. They occur in two forms: a sporadic form characterized by isolated lesions, and a familial form characterized by multiple lesions with an autosomal dominant mode of inheritance. The management of patients with cavernous malformations, particularly those with the familial form of the disease, remains a challenge because little is known regarding the natural history. The authors report the results of an ongoing study in which six families afflicted by familial cavernous malformations have been prospectively followed with serial interviews, physical examinations, and magnetic resonance (MR) imaging at 6- to 12-month intervals. A total of 59 members of these six families were screened for protocol enrollment; 31 (53%) had MR evidence of familial cavernous malformations. Nineteen (61%) of these 31 patients were symptomatic, with seizures in 12 (39%), recurrent headaches in 16 (52%), focal sensory/motor deficits in three (10%), and visual field deficits in two (6%). Twenty-one of these 31 patients underwent at least two serial clinical and MR imaging examinations. A total of 128 individual cavernous malformations (mean 6.5 ± 3.8 lesions/patient) were identified and followed radiographically. During a mean follow-up period of 2.2 years (range 1 to 5.5 years), serial MR images demonstrated 17 new lesions in six (29%) of the 21 patients; 13 lesions (10%) showed changes in signal characteristics, and five lesions (3.9%) changed significantly in size. The incidence of symptomatic hemorrhage was 1.1% per lesion per year. The results of this study demonstrate that the familial form of cavernous malformations is a dynamic disease; serial MR images revealed changes in the number, size, and imaging characteristics of lesions consistent with acute or resolving hemorrhage. It is believed that the de novo development of new lesions in this disease has not been previously reported. These findings suggest that patients with familial cavernous malformations require careful follow-up monitoring, and that significant changes in neurological symptoms warrant repeat MR imaging. Surgery should be considered only for lesions that produce repetitive or progressive symptoms. Prophylactic resection of asymptomatic lesions does not appear to be indicated.

Dynamic nature of cavernous malformations: a prospective magnetic resonance imaging study with volumetric analysis

2000 ◽  
Vol 93 (6) ◽  
pp. 981-986 ◽  
Author(s):  
Richard E. Clatterbuck ◽  
John L. Moriarity ◽  
Ilhan Elmaci ◽  
Roland R. Lee ◽  
Steven N. Breiter ◽  
...  
Keyword(s):  
Mr Imaging ◽  
De Novo ◽  
Volume Data ◽  
Mr Images ◽  
Cavernous Malformations ◽  
Content Type ◽  
Signal Characteristics ◽  
The Mean ◽  
Fine Print

Object. Although cavernous malformations (CMs) are not detected in angiographic studies, they have a characteristic appearance on magnetic resonance (MR) images. A number of reports published in the last decade have focused on the behavior of these lesions within the clinical environment. However, little has been published about the evolution of CMs over time, as observed in imaging studies. To understand imaging-documented changes in CMs over time, we analyzed MR images of 114 cavernous malformations in 68 patients who were followed prospectively.Methods. For each CM the location, volume, and MR imaging signal characteristics were recorded. Volume data were available for 107 lesions from initial images. The mean volume of these 107 CMs was 2779 mm3. The lesions ranged in size from 0.5 to 46,533 mm3 (46.5 cm3). Volume data from a second set of images were available for 76 CMs (mean interval from first imaging session 26 months), and from a third set of images for 24 lesions (mean interval from second imaging session 18 months). Over the first follow-up interval, the mean volume change was −991 mm3 (a decrease of approximately 1 cm3) and over the second interval the mean volume change was −642 mm3. Although these mean volume changes appear modest, volume changes in single lesions during follow-up intervals were more dramatic, with decreases as large as 45,629 mm3 (45.6 cm3) and increases as large as 6,074 mm3 (6 cm3). Serial examinations of the MR imaging signal characteristics of these CMs demonstrate a trend for maturation of blood products from a subacute, to a mixed, and finally to a chronic appearance. Three lesions appeared de novo during the follow-up period.Conclusions. On the basis of their analysis, the authors conclude that CMs exhibit a range of dynamic behaviors including enlargement, regression, and de novo formation, as well as progression through a series of characteristic MR imaging appearances.

Accelerated spondylotic changes adjacent to the fused segment following central cervical corpectomy: magnetic resonance imaging study evidence

2004 ◽  
Vol 100 (1) ◽  
pp. 2-6 ◽  
Author(s):  
Vaijayantee Kulkarni ◽  
Vedantam Rajshekhar ◽  
Lakshminarayan Raghuram
Keyword(s):  
Magnetic Resonance ◽  
Mr Imaging ◽  
Imaging Study ◽  
Degenerative Changes ◽  
Adjacent Segment ◽  
Mr Images ◽  
Short Term ◽  
Content Type ◽  
Fine Print

Object. The authors studied whether cervical spine motion segments adjacent to a fused segment exhibit accelerated degenerative changes on short-term follow-up magnetic resonance (MR) imaging. Methods. Preoperative and short-term follow-up (mean duration 17.5 months, range 10–48 months) cervical MR images obtained in 44 patients who had undergone one- or two-level corpectomy for cervical spondylotic myelopathy were evaluated qualitatively and quantitatively. The motion segment adjacent to the fused segment and a segment remote from the fused segment were evaluated for indentation of the thecal sac, disc height, and sagittal functional diameter of the spinal canal on midsagittal T2-weighted MR images. Thecal sac indentations were classifed as mild, moderate, and severe. New indentations of the thecal sac of varying severity (mild in 17 patients [38.6%], moderate in 10 [22.7%], and severe in six [13.6%]) had developed at the adjacent segments in 33 (75%) of 44 patients. The degenerative changes were seen at the superior level in 11 patients, inferior level in 10 patients, and at both levels in 12 patients and resulted from both anterior and posterior element degeneration in the majority (23 [69.6%]) of patients. The remote segments showed mild thecal sac indentations in seven patients and moderate indentations in two patients (nine [20.5%] of 44). Compared with the changes at the remote segment, the canal size was significantly decreased at the superior adjacent segment by 0.9 mm (p = 0.007). No patient sustained a new neurological deficit due to adjacent-segment changes. Conclusions. On short-term follow-up MR imaging, levels adjacent to the fused segment exhibited more pronounced degenerative changes (compared with remote levels) in 75% of patients who had undergone one- or two-level central corpectomy.

Magnetic resonance imaging: an accurate method to evaluate arteriovenous malformations after stereotactic radiosurgery

1996 ◽  
Vol 85 (6) ◽  
pp. 1044-1049 ◽  
Author(s):  
Bruce E. Pollock ◽  
Douglas Kondziolka ◽  
John C. Flickinger ◽  
Atul K. Patel ◽  
David J. Bissonette ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Mr Imaging ◽  
Cerebral Angiography ◽  
False Negative ◽  
Resonance Imaging ◽  
Mr Images ◽  
Content Type ◽  
Fine Print

✓ To determine the accuracy of magnetic resonance (MR) imaging in comparison to cerebral angiography after radiosurgery for an arteriovenous malformation (AVM), the authors reviewed the records of patients who underwent radiosurgery at the University of Pittsburgh Medical Center before 1992. All patients in the analysis had AVMs in which the flow-void signal was visible on preradiosurgical MR imaging. One hundred sixty-four postradiosurgical angiograms were obtained in 140 patients at a median of 2 months after postradiosurgical MR imaging (median 24 months after radiosurgery). Magnetic resonance imaging correctly predicted patency in 64 of 80 patients in whom patent AVMs were seen on follow-up angiography (sensitivity 80%) and angiographic obliteration in 84 of 84 patients (specificity 100%). Overall, 84 of 100 AVMs in which evidence of obliteration was seen on MR images displayed angiographic obliteration (negative predictive value, 84%). Ten of the 16 patients with false-negative MR images underwent follow-up angiography: in seven the lesions progressed to complete angiographic obliteration without further treatment. Exclusion of these seven patients from the false-negative MR imaging group increases the predictive value of a negative postradiosurgical MR image from 84% to 91%. No AVM hemorrhage was observed in clinical follow up of 135 patients after evidence of obliteration on MR imaging (median follow-up interval 35 months; range 2–96 months; total follow up 382 patient-years). Magnetic resonance imaging proved to be an accurate, noninvasive method for evaluating the patency of AVMs that were identifiable on MR imaging after stereotactic radiosurgery. This imaging modality is less expensive, more acceptable to patients, and does not have the potential for neurological complications that may be associated with cerebral angiography. The risk associated with follow-up cerebral angiography may no longer justify its role in the assessment of radiosurgical results in the treatment of AVMs.

Cerebral cavernous malformations: natural history and prognosis after clinical deterioration with or without hemorrhage

1997 ◽  
Vol 87 (2) ◽  
pp. 190-197 ◽  
Author(s):  
Phillip J. Porter ◽  
Robert A. Willinsky ◽  
William Harper ◽  
M. Christopher Wallace
Keyword(s):  
Natural History ◽  
Cerebellar Nuclei ◽  
Neurological Deterioration ◽  
Focal Neurological Deficit ◽  
Cavernous Malformations ◽  
Content Type ◽  
Lesion Location ◽  
Neurological Event ◽  
Fine Print

✓ Despite recent studies of the natural history of cavernous malformations, there remains significant uncertainty concerning hemorrhage rates and the importance of lesion location. Controversy arises over varying definitions of “hemorrhage.” What is ultimately important to the patient is the occurrence of a neurological event, which may or may not be associated with radiologically documented hemorrhage, as well as the chance of recovery after such an event. The purpose of this study was to determine the rates of occurrence and sequelae of neurological events in 173 patients referred to our vascular malformation clinic with cavernous malformations. All patient data were entered into a database. The mean age at presentation for the 173 patients was 37.5 years. The lesion location was deep (brainstem, cerebellar nuclei, thalamus, or basal ganglia) in 64 patients (37%) and superficial in 109 (63%). Thirty-one patients (18%) had multiple lesions. Disease presentation was due to seizures in 62 patients (36%), hemorrhage in 44 (25%), focal neurological deficit without documented hemorrhage in 35 (20%), headache alone in 11 (6%), and incidental findings in 21 patients (12%). The results obtained in the 110 patients eligible for follow-up review were used to derive information on the rates of hemorrhage and neurological events. An interval event (neurological deterioration) required both symptoms and signs. The total mean follow-up period was 46 months, the majority (65%) of which was prospective. There were 18 interval events in 427 patient-years of follow-up review, for an overall annual event rate of 4.2%. Location was the most important factor for predicting interval event occurrence, with significantly higher rates for deeply located (10.6%/year) compared with superficially located lesions (0%/year) (p = 0.0001). Of patients suffering a neurological event, only 37% had complete resolution of their deficits. This largely prospective study indicates that deep cavernous malformations carry a worse prognosis than superficial lesions with respect to annual rates of neurological deterioration. The alarming rate of adverse clinical events occurring in patients with deep lesions is punctuated by the fact that less than one-half of them recover fully during long-term follow-up review.

Cavernous malformations of the brainstem: experience with 100 patients

1999 ◽  
Vol 90 (1) ◽  
pp. 50-58 ◽  
Author(s):  
Randall W. Porter ◽  
Paul W. Detwiler ◽  
Robert F. Spetzler ◽  
Michael T. Lawton ◽  
Jonathan J. Baskin ◽  
...  
Keyword(s):  
Natural History ◽  
Skull Base ◽  
Surgical Patients ◽  
Cavernous Malformations ◽  
Content Type ◽  
History Of ◽  
Lost To Follow Up ◽  
Pontomedullary Junction ◽  
Fine Print

Object. In this study the authors review surgical experience with cavernous malformations of the brainstem (CMBs) in an attempt to define more clearly the natural history, indications, and risks of surgical management of these lesions.Methods. The authors retrospectively reviewed the cases of 100 patients (38 males and 62 females; mean age 37 years) harboring 103 lesions at treated a single institution between 1984 and 1997. Clinical histories, radiographs, pathology records, and operative reports were evaluated. The brainstem lesions were distributed as follows: pons in 39 patients, medulla in 16, midbrain in 16, pontomesencephalic junction in 15, pontomedullary junction in 10, midbrain—hypothalamus/thalamus region in two patients, and more than two brainstem levels in five. The retrospective annual hemorrhage rate was most conservatively estimated at 5% per lesion per year. Standard skull base approaches were used to resect lesions in 86 of the 100 patients. Intraoperatively, all 86 patients were found to have a venous anomaly in association with the CMB. Follow up was available in 98% (84 of 86) of the surgical patients. Of these, 73 (87%) were the same or better after surgical intervention, eight (10%) were worse, and three (4%) died. Two surgical patients were lost to follow-up review. Incidences of permanent or severe morbidity occurred in 10 (12%) of the surgically treated patients. The average postoperative Glasgow Outcome Scale score for surgically treated patients was 4.5, with a mean follow-up period of 35 months.Conclusions. The natural history of CMBs is worse than that of cavernous malformations in other locations. These CMBs can be resected using skull base approaches, which should be considered in patients with symptomatic hemorrhage who harbor lesions that approach the pial surface. Venous anomalies are always associated with CMBs and must be preserved.

Natural history of the cavernous angioma

1991 ◽  
Vol 75 (5) ◽  
pp. 709-714 ◽  
Author(s):  
John R. Robinson ◽  
Issam A. Awad ◽  
John R. Little
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Cleveland Clinic ◽  
Surgical Excision ◽  
Cavernous Angioma ◽  
Mr Images ◽  
Focal Neurological Deficit ◽  
Content Type ◽  
History Of

✓ The incidence and natural history of the cavernous angioma have remained unclear in part because of the difficulty of diagnosing and following this lesion prior to surgical excision. The introduction of magnetic resonance (MR) imaging has improved the sensitivity and specificity of diagnosing and following this vascular malformation. Seventy-six lesions with an MR appearance typical of a presumed cavernous angioma were discovered in 66 patients among 14,035 consecutive MR images performed at the Cleveland Clinic between 1984 and 1989. Follow-up studies in 86% of the cases over a mean period of 26 months provided 143 lesion-years of clinical survey of this condition. The most frequent presenting features were seizure, focal neurological deficit, and headache. While most lesions exhibited evidence of occult bleeding on MR imaging, there was overt hemorrhage in seven of the 57 symptomatic patients and only one overt hemorrhage occurred during the follow-up interval. The annualized bleeding rate was 0.7%. Analysis of the hemorrhage group revealed a significantly greater risk of overt hemorrhage in females. Pathological confirmation of cavernous angioma was obtained in all 14 surgical cases. This information assists in rational therapeutic planning and prognosis in patients with MR images showing lesions suggestive of cavernous angioma.

The natural history of cerebral cavernous malformations

1995 ◽  
Vol 83 (5) ◽  
pp. 820-824 ◽  
Author(s):  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
John R. W. Kestle
Keyword(s):  
Natural History ◽  
Management Strategies ◽  
Operative Management ◽  
Neurological Deficits ◽  
Cavernous Malformations ◽  
Content Type ◽  
Increased Risk ◽  
History Of ◽  
Fine Print

✓ To determine the natural history of brain cavernous malformations, the authors entered patients referred to their center into a prospective registry between 1987 and 1993. All patients underwent magnetic resonance imaging, which showed the typical appearance of this lesion, and conservative management was recommended in all. Patients or their referring physicians were contacted for follow-up data. The purpose of the study was to define the rate of symptomatic hemorrhage and to determine the outcome in those patients who had suffered seizures. Follow-up data were available for 122 patients with a mean age at entry of 37 years (range 4–82 years). The malformation was located in the brainstem in 43 cases (35%), the basal ganglia/thalamus in 20 (17%), and a hemispheric area in 59 (48%). Fifty percent of patients had never had a symptomatic hemorrhage, 41% had one bleed, 7% had two, and 2% had three. Seizures were reported in 23% of patients and headaches in 15%. Lesions were solitary in 80% of patients and multiple in 20%. The retrospective annual hemorrhage rate (61 bleeds/4550.6 patient-years of life) was 1.3%. The mean prospective follow-up period was 34 months. There were nine bleeds during this time, six with new neurological deficits. In patients without a prior bleed, the prospective annual rate of hemorrhage was 0.6%. In contrast, patients with prior hemorrhage had an annual bleed rate of 4.5% (p = 0.028). Patient sex (p = 0.97) or the presence of seizures (p = 0.11), headaches (p = 0.06), or solitary versus multiple lesions (p = 0.15) were not significant predictors of later hemorrhage. There was no difference in the rate of bleeds between brain locations. Four patients with seizures became seizurefree and four patients without seizures later developed seizures; only one patient developed intractable seizures. Fourteen patients (11%) underwent surgery (two after hemorrhage, five with seizures, and seven with progressive deficits), and five had radiosurgery. No patient died in the follow-up period. This study indicates that conservative versus operative management strategies may need to be redefined, especially in patients who present with hemorrhage and who appear to have a significantly increased risk of subsequent rehemorrhage.

Evaluation of gamma thalamotomy for parkinsonian and other tremors: survival of neurons adjacent to the thalamic lesion after gamma thalamotomy

2000 ◽  
Vol 93 (supplement_3) ◽  
pp. 120-127 ◽  
Author(s):  
Chihiro Ohye ◽  
Tohru Shibazaki ◽  
Junji Ishihara ◽  
Jie Zhang
Keyword(s):  
Tissue Reaction ◽  
Vascular Lesions ◽  
Thalamic Lesion ◽  
Mr Images ◽  
Unsatisfactory Result ◽  
Content Type ◽  
Rhythmic Discharge ◽  
Latent Interval ◽  
Fine Print

Object. The effects of gamma thalamotomy for parkinsonian and other kinds of tremor were evaluated. Methods. Thirty-six thalamotomies were performed in 31 patients by using a 4-mm collimator. The maximum dose was 150 Gy in the initial six cases, which was reduced to 130 Gy thereafter. The longest follow-up period was 6 years. The target was determined on T2-weighted and proton magnetic resonance (MR) images. The point chosen was in the lateral-most part of the thalamic ventralis intermedius nucleus. This is in keeping with open thalamotomy as practiced at the authors' institution. In 15 cases, gamma thalamotomy was the first surgical procedure. In other cases, previous therapeutic or vascular lesions were visible to facilitate targeting. Two types of tissue reaction were onserved on MR imaging: a simple oval shape and a complex irregular shape. Neither of these changes affected the clinical course. In the majority of cases, the tremor subsided after a latent interval of approximately 1 year after irradiation. The earliest response was demonstrated at 3 months. In five cases the tremor remained. In four of these cases, a second radiation session was administered. One of these four patients as well as another patient with an unsatisfactory result underwent open thalamotomy with microrecording. In both cases, depth recording adjacent to the necrotic area revealed normal neuronal activity, including the rhythmic discharge of tremor. Minor coagulation was performed and resulted in immediate and complete arrest of the remaining tremor. Conclusions. Gamma thalamotomy for Parkinson's disease seems to be an alternative useful method in selected cases.

Gamma knife surgery for vestibular schwannoma: 10-year experience of 195 cases

2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 87-97 ◽  
Author(s):  
Wen-Yuh Chung ◽  
Kang-Du Liu ◽  
Cheng-Ying Shiau ◽  
Hsiu-Mei Wu ◽  
Ling-Wei Wang ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Gamma Knife ◽  
Vestibular Schwannoma ◽  
Tumor Volume ◽  
Radiation Effect ◽  
Gamma Knife Surgery ◽  
Homogeneous Distribution ◽  
Content Type ◽  
Fine Print

Object. The authors conducted a study to determine the optimal radiation dose for vestibular schwannoma (VS) and to examine the histopathology in cases of treatment failure for better understanding of the effects of irradiation. Methods. A retrospective study was performed of 195 patients with VS; there were 113 female and 82 male patients whose mean age was 51 years (range 11–82 years). Seventy-two patients (37%) had undergone partial or total excision of their tumor prior to gamma knife surgery (GKS). The mean tumor volume was 4.1 cm3 (range 0.04–23.1 cm3). Multiisocenter dose planning placed a prescription dose of 11 to 18.2 Gy on the 50 to 94% isodose located at the tumor margin. Clinical and magnetic resonance (MR) imaging follow-up evaluations were performed every 6 months. A loss of central enhancement was demonstrated on MR imaging in 69.5% of the patients. At the latest MR imaging assessment decreased or stable tumor volume was demonstrated in 93.6% of the patients. During a median follow-up period of 31 months resection was avoided in 96.8% of cases. Uncontrolled tumor swelling was noted in five patients at 3.5, 17, 24, 33, and 62 months after GKS, respectively. Twelve of 20 patients retained serviceable hearing. Two patients experienced a temporary facial palsy. Two patients developed a new trigeminal neuralgia. There was no treatment-related death. Histopathological examination of specimens in three cases (one at 62 months after GKS) revealed a long-lasting radiation effect on vessels inside the tumor. Conclusions. Radiosurgery had a long-term radiation effect on VSs for up to 5 years. A margin 12-Gy dose with homogeneous distribution is effective in preventing tumor progression, while posing no serious threat to normal cranial nerve function.

A fatal, chronically growing basilar artery: a new type of dissecting aneurysm

1996 ◽  
Vol 84 (6) ◽  
pp. 962-971 ◽  
Author(s):  
Tohru Mizutani
Keyword(s):  
Mr Imaging ◽  
Basilar Artery ◽  
Dissecting Aneurysm ◽  
Neurological Deficits ◽  
Vertebrobasilar Insufficiency ◽  
Content Type ◽  
Asymptomatic Patients ◽  
Basilar Arteries ◽  
Fine Print

✓ A long-term follow-up study (minimum duration 2 years) was made of 13 patients with tortuous dilated basilar arteries. Of these, five patients had symptoms related to the presence of such arteries. Symptoms present at a very early stage included vertebrobasilar insufficiency in two patients, brainstem infarction in two patients, and left hemifacial spasm in one patient. Initial magnetic resonance (MR) imaging in serial slices of basilar arteries obtained from the five symptomatic patients showed an intimal flap or a subadventitial hematoma, both of which are characteristic of a dissecting aneurysm. In contrast, the basilar arteries in the eight asymptomatic patients did not show particular findings and they remained clinically and radiologically silent during the follow-up period. All of the lesions in the five symptomatic patients gradually grew to fantastic sizes, with progressive deterioration of the related clinical symptoms. Dilation of the basilar artery was consistent with hemorrhage into the “pseudolumen” within the laminated thrombus, which was confirmed by MR imaging studies. Of the five symptomatic patients studied, two died of fatal subarachnoid hemorrhage (SAH) and two of brainstem compression; the fifth patient remains alive without neurological deficits. In the three patients who underwent autopsy, a definite macroscopic double lumen was observed in both the proximal and distal ends of the aneurysms within the layer of the thickening intima. Microscopically, multiple mural dissections, fragmentation of internal elastic lamina (IEL), and degeneration of media were diffusely observed in the remarkably extended wall of the aneurysms. The substantial mechanism of pathogenesis and enlargement in the symptomatic, highly tortuous dilated artery might initially be macroscopic dissection within a thickening intima and subsequent repetitive hemorrhaging within a laminated thrombus in the pseudolumen combined with microscopic multiple mural dissections on the basis of a weakened IEL. The authors note and caution that symptomatic, tortuous dilated basilar arteries cannot be overlooked because they include a group of malignant arteries that may grow rapidly, resulting in a fatal course.

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