Idiopathic Arteritis With Multiple Intracranial Aneurysms

Abstract

37-year-old white male engineer, presented to his physician in 1963 with the sudden onset of severe abdominal pain, frequent diarrhea, and generalized malaise. These subacute symptoms persisted for two weeks when abdominal pain and malaise decreased in severity and diarrhea became less frequent. The patient, however, continued to experience for the remaining six years of his life two to four loose stools per day and fleeting abdominal pains. Upper gastrointestinal and colonic radiocontrast studies were negative as were other evaluatory studies.In 1963, he also developed bilateral chronic uveitis, more severe in his right eye, for which he received two short courses of topical corticosteroids. The eyes developed local steroid-induced glaucoma which responded to topical epinephrine therapy, but by September, 1964, complete posterior synechiae on the right had produced iris bombe and intermittent angle closure glaucoma. Therefore, a peripheral iridectomy was performed. In December, 1964, vision worsened in the right eye to 20/200 with an increase in the activity of the inflammation. Vision and glaucoma continued to fluctuate with local corticosteroids inducing elevated intraocular pressure. By September, 1966, cataract had progressed so that the vision in the right eye had dropped to counting fingers at 1 to 2 feet. The patient underwent an intracapsular lens extraction of the right eye under local anesthesia. By September, 1966, contact lenses corrected the right eye to 20/30. The left eye cataract progressed and in January, 1967, it was removed.In 1967, the patient was admitted to The Johns Hopkins Hospital to evaluate the cause of his chronic diarrhea. Radiographic studies of his bowels were repeatedly negative. A rectal biopsy as well as an appendix with attached lymph nodes revealed no abnormalities. At this time, the erythrocyte sedimentation rate was 26 mm/hr and all other serological tests were normal.

Endovascular Methods for the Treatment of Intracranial Cerebral Aneurysms

“…[26][27][28] Several medical conditions are associated with the development of intracranial aneurysms, including systemic lupus erythematosus, Takayasu disease, giant cell arteritis, autosomal polycystic kidney disease, type IV Ehlers-Danlos syndrome, Marfan syndrome, fibromuscular dysplasia, type 1 neurofibromatosis, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, coarctation of the aorta, and a1-antitrypsin. [29][30][31][32][33][34][35][36][37] Genetics also plays a role in the formation of intracranial aneurysms. If 2 first-degree relatives in the same family have an intracranial aneurysm with no heritable connective tissue disorder, immediate family members have up to a 17% incidence of harboring an unruptured aneurysm.…”