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  • By Author: Spetzler, Robert F. x
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Joseph M. Zabramski, Thomas M. Wascher, Robert F. Spetzler, Blake Johnson, John Golfinos, Burton P. Drayer, Ben Brown, Daniel Rigamonti and Geraldine Brown

✓ 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.

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Daniele Rigamonti, Robert F. Spetzler, Burton P. Drayer, W. Michel Bojanowski, John Hodak, K. Hoenig Rigamonti, K. Plenge, M. Powers and Harold Rekate

✓ The magnetic resonance (MR) imaging appearance of venous malformations, all angiographically verified, was evaluated in 11 patients. A venous malformation is characteristically depicted as a tubular area of decreased signal intensity in the white matter of the brain. In one patient, a histologically verified cavernous malformation was also present with a characteristic mixed signal-intensity core on the T2-weighted MR images. Care should be used when evaluating venous angiomas to exclude the presence of a lesion with associated prominent venous drainage, such as a glioma.

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Daniele Rigamonti, Burton P. Drayer, Peter C. Johnson, Mark N. Hadley, Joseph Zabramski and Robert F. Spetzler

✓ The angiographic, computerized tomography (CT), and magnetic resonance imaging (MRI) findings were compared in 10 patients with a total of 16 pathologically verified cavernous angiomas. Only three lesions had abnormal vasculature in the form of venous pooling or a capillary blush. The CT scans were positive in seven patients and detected 14 lesions, while high-field strength (1.5 Tesla) MRI was positive in each case and demonstrated 27 distinct lesions. On T2-weighted MRI, the combination of a reticulated core of mixed signal intensity (SI) with a surrounding rim of decreased SI strongly suggests the diagnosis of a cavernous malformation. Smaller lesions appear as areas of decreased SI (black dots). The sensitivity of MRI is based on magnetic susceptibility and possibly diffusion effects related to field heterogeneity that is more conspicuous on high-field imaging and caused by the presence of excessive iron (hemosiderin).