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Tuberous Sclerosis With Intracranial Aneurysm

Tuberous Sclerosis With Intracranial Aneurysm Abstract The classic renal lesion in tuberous sclerosis (TS) is angiomyolipoma with aneurysmal formation in the interlobar arteries. Aneurysms of cerebral vessels in TS have been found only twice.1,2 We report a third case that suggests that renal and intracranial aneurysms in TS may represent a common defect. REPORT OF A CASE A 17-year-old girl had major motor seizures for seven years. At age 12 years, she had an IQ of 80. A maternal aunt also had seizures. New symptoms of déjà vu followed by right focal motor seizures prompted hospitalization. An adenoma sebaceum, pitting of her teeth, and one shagreen patch were noted. Neurologic examination showed a visual acuity of 20/20 OD, 20/30 OS, a Marcus Gunn's pupillary sign OS with optic pallor, and a left inferior altitudinal defect. A computed tomographic scan revealed periventricular calcifications and an enhancing lesion in the left parasellar region (Fig 1). Cerebral angiograms References 1. Davidson S: Tuberous sclerosis with fusiform aneurysms of both internal carotid arteries manifested by unilateral visual loss and papilledema . Bull Los Angeles Neurol Soc 1974;39:128-132. 2. Snowdon JA: Cerebral aneurysm, renal cysts and hamartomas in a case of tuberous sclerosis . Br J Urol 1974;46:583.Crossref 3. Bigelow NH: The association of polycystic kidneys with intracranial aneurysm and other related disorders . Am J Med Sci 1953;255:485-494.Crossref 4. Anderson D, Tannen RL: Tuberous sclerosis and chronic renal failure: Potential confusion with PKD . Am J Med 1969;47:163-168.Crossref 5. Chonko AM, Weiss SM, Stein JH, et al: Renal involvement in TS . Am J Med 1974;56:124-132.Crossref 6. Wenzl JE, Lagos JC, Albers DD: TS presenting as polycystic kidneys and seizures in an infant . J Pediatr 1970;77:673-675.Crossref 7. Perez-Atayde AR, Iwaya S, Lack EE: Angiomyolipomas and polycystic renal disease in tuberous sclerosis . Urology 1981;17:607-610.Crossref 8. Inglis K: The relation of the renal lesions to the cerebral lesions in the tuberous sclerosis complex . Am J Pathol 1954;30:739-755. 9. Mettinger KL, Ericson K: Fibromuscular dysplasia and the brain: Observations on angiographic, clinical and genetic characteristics . Stroke 1982;13:46-52.Crossref 10. Mettinger KL: Fibromuscular dysplasia and the brain: II. Current concept of the disease . Stroke 1982;13:53-58.Crossref 11. Sood S, Mancini AA, Kropp K: Tuberous sclerosis: Emphasis on the angiographic findings . J Urol 1975;114:185-189. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Tuberous Sclerosis With Intracranial Aneurysm

Beall, Steven; Delaney, Peyton
Archives of Neurology , Volume 40 (13) – Dec 1, 1983
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References (14)

Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1983.04050120076015
Publisher site
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Abstract

Abstract The classic renal lesion in tuberous sclerosis (TS) is angiomyolipoma with aneurysmal formation in the interlobar arteries. Aneurysms of cerebral vessels in TS have been found only twice.1,2 We report a third case that suggests that renal and intracranial aneurysms in TS may represent a common defect. REPORT OF A CASE A 17-year-old girl had major motor seizures for seven years. At age 12 years, she had an IQ of 80. A maternal aunt also had seizures. New symptoms of déjà vu followed by right focal motor seizures prompted hospitalization. An adenoma sebaceum, pitting of her teeth, and one shagreen patch were noted. Neurologic examination showed a visual acuity of 20/20 OD, 20/30 OS, a Marcus Gunn's pupillary sign OS with optic pallor, and a left inferior altitudinal defect. A computed tomographic scan revealed periventricular calcifications and an enhancing lesion in the left parasellar region (Fig 1). Cerebral angiograms References 1. Davidson S: Tuberous sclerosis with fusiform aneurysms of both internal carotid arteries manifested by unilateral visual loss and papilledema . Bull Los Angeles Neurol Soc 1974;39:128-132. 2. Snowdon JA: Cerebral aneurysm, renal cysts and hamartomas in a case of tuberous sclerosis . Br J Urol 1974;46:583.Crossref 3. Bigelow NH: The association of polycystic kidneys with intracranial aneurysm and other related disorders . Am J Med Sci 1953;255:485-494.Crossref 4. Anderson D, Tannen RL: Tuberous sclerosis and chronic renal failure: Potential confusion with PKD . Am J Med 1969;47:163-168.Crossref 5. Chonko AM, Weiss SM, Stein JH, et al: Renal involvement in TS . Am J Med 1974;56:124-132.Crossref 6. Wenzl JE, Lagos JC, Albers DD: TS presenting as polycystic kidneys and seizures in an infant . J Pediatr 1970;77:673-675.Crossref 7. Perez-Atayde AR, Iwaya S, Lack EE: Angiomyolipomas and polycystic renal disease in tuberous sclerosis . Urology 1981;17:607-610.Crossref 8. Inglis K: The relation of the renal lesions to the cerebral lesions in the tuberous sclerosis complex . Am J Pathol 1954;30:739-755. 9. Mettinger KL, Ericson K: Fibromuscular dysplasia and the brain: Observations on angiographic, clinical and genetic characteristics . Stroke 1982;13:46-52.Crossref 10. Mettinger KL: Fibromuscular dysplasia and the brain: II. Current concept of the disease . Stroke 1982;13:53-58.Crossref 11. Sood S, Mancini AA, Kropp K: Tuberous sclerosis: Emphasis on the angiographic findings . J Urol 1975;114:185-189.

Journal

Archives of Neurology – American Medical Association

Published: Dec 1, 1983

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