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Intracranial Aneurysms in Patients With Autosomal Dominant Polycystic Kidney Disease: How to Diagnose and Who to Screen

1993; Elsevier BV; Volume: 22; Issue: 4 Linguagem: Inglês

10.1016/s0272-6386(12)80924-3

1523-6838

Arlene B. Chapman, Margaret Johnson, Patricia A. Gabow,

Moyamoya disease diagnosis and treatment

HEN A PATIENT with autosomal dom­ inant polycystic kidney disease (ADPKD) presents with an acute neurologic event heralded by a sudden loss of consciousness or focal neu­ rological signs such as a dense hemiparesis, the red flag of ruptured intracranial aneurysm (ICA) is usually waved. However, in ADPKD other di­ agnostic possibilities, including infarction and embolic phenomena, must be considered. Hy­ pertension is common, occurring in 75% of ADPKD patients, I and, as in the general popu­ lation, one would expect a possible association between hypertension and cerebral vascular events. 2 In addition, mitral valve prolapse occurs in 26 % of ADPKD patients 3 and can be asso­ ciated with ventricular dysrhythmia leading to cerebral emboli. The importance of considering all possibilities in the differential diagnosis of acute neurologic events in ADPKD is under­ scored by a number of studies. Zeier et al 4 performed a retrospective au­ topsy study of 86 ADPKD patients. Twenty percent of patients died of cerebral complica­ tions, of which 70% were due to hypertensive intracerebral hemorrhage, 17.5 % were due to cerebral infarction, and 11.5 % were due to rup­ tured saccular aneurysm. Similarly, Ryu 5 pre­ sented an autopsy review of 900 consecutive cases of hemorrhagic stroke from Taiwan. Eleven of 900 patients were identified with ADPKD. Seventy-five percent of ADPKD paFrom the Departmelll of Medicine. Unirersit) o/Colorado Hl!alth Sciences Cemer. Dem-er. CO. R eceived February]]. 1993: accepted in revised/orm March 16. 1993.