Rupture of a previously documented small asymptomatic intracranial aneurysm in a patient with autosomal dominant polycystic kidney disease. Case report

Intracranial aneurysms are common extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD). Although their natural history is not completely understood, small asymptomatic intracranial aneurysms in patients with ADPKD often are not treated but are followed with serial magnetic resonance (MR) angiography. The authors report the unique case of a patient with ADPKD who bled from a previously documented asymptomatic 3-mm intracranial aneurysm. This 42-year-old man with ADPKD suffered a subarachnoid hemorrhage (SAH) from a 7-mm left pericallosal artery aneurysm. This aneurysm was clipped and the patient made an excellent recovery. An irregular asymptomatic 3-mm right middle cerebral artery (MCA) aneurysm had also been demonstrated on angiography. While the patient was considering elective surgery for the MCA aneurysm, he suffered a hemorrhage from this lesion 10 weeks after the initial SAH. The aneurysm was clipped and the patient made a satisfactory recovery (he was moderately disabled). In this report the authors indicate that small asymptomatic intracranial aneurysms are not always innocuous in patients with ADPKD, and they suggest that treatment should be strongly considered for these lesions in this group of patients when there is a history of SAH or the aneurysm is irregular in appearance. Because MR angiography studies may not adequately define the configuration of small aneurysms and irregularity may easily be missed, conventional angiography is recommended for patients with ADPKD who are found to have an intracranial aneurysm on screening with MR angiography.     

Causes of death in autosomal dominant polycystic kidney disease

To determine the causes of death in autosomal dominant polycystic kidney disease (ADPKD) patients and to examine whether the extrarenal manifestations of ADPKD influence the causes of death, the medical records of 129 patients who died between 1956 and 1993 were reviewed; 58% of the 129 patients had an autopsy performed. Seventy-seven percent died after reaching ESRD. The mean age at death increased from 51 yr for those who died before 1975 to 59 yr for those who died after 1975, reflecting the introduction of renal replacement therapies. The most common cause of death before 1975 was infection (30%), followed by uremia (28%) and cardiac disease (21%); after 1975, these were cardiac disease (36%) and infection (24%). Infection was equally prevalent before and after 1975, presenting as sepsis in 94% and directly relating to ADPKD in 47% of these patients. Underlying factors for cardiac death were cardiac hypertrophy, seen in 89% of all autopsied patients, and coronary artery disease, seen in 81%. A neurologic event was the cause of death in 12% of patients; these were ruptured intracranial aneurysm in 6%, hypertensive intracranial hemorrhage in 5%, and ischemic stroke in 1%. The mean age of those who died of ruptured intracranial aneurysm was 37 yr. No patient died of renal cancer. Liver cysts were the most common extrarenal manifestation, seen in 70% of the autopsied cases; cysts in other organs were very rare. Colonic diverticula were found in 21%. Thus, the renal and extrarenal manifestations of ADPKD are important contributors to morbidity and mortality.     

Effects of difructose anhydride III on calcium absorption in small and large intestines of rats

Patients with autosomal dominant polycystic kidney disease (ADPKD) have an increased risk of intracranial aneurysms. Reports on arterial aneurysms in other locations have not been conclusive. The present study was initiated to investigate the prevalence of coronary aneurysms. Thirty ADPKD patients who had undergone coronary angiography on clinical indication were identified, 15 after renal transplantation. For each ADPKD patient, a control patient was identified with end-stage renal disease, investigated by coronary angiography, and matched for age, sex, and time relation to transplantation. All angiograms were retrieved and reevaluated with respect to aneurysms, defined as an increase in artery diameter by 50% or more, as well as to pathologic ectasias not fulfilling this criterion. Aneurysms were detected in four ADPKD patients and two control subjects. Five more ADPKD patients, but none of the control subjects, had minor ectasias. One ADPKD patient had a dissecting aortic aneurysm, and another died of aortic dissection during bypass surgery. This study adds to the evidence of an increased risk of extracranial aneurysms in ADPKD patients.     

Use of polymerase chain reaction in clinical diagnostic laboratories in viral hepatitis B and C]

alpha 1-Antitrypsin deficiency has been associated with a variety of vascular disorders including arterial aneurysms, spontaneous extracranial arterial dissections, and arterial fibromuscular dysplasia. We determined the distribution of alpha 1-antitrypsin phenotypes in patients with intracranial arterial dissections, a rare cause of subarachnoid hemorrhage. The study population consisted of 4 consecutive patients with subarachnoid hemorrhage due to spontaneous intracranial arterial dissections. The vertebral artery was involved in 3 patients and the posterior inferior cerebellar artery in 1 patient. Three of these 4 patients were found to have a heterozygous alpha 1-antitrypsin deficiency (PiMZ or PiMS phenotypes). These data support previous studies suggesting that patients with alpha 1-antitrypsin deficiency may be at an increased risk of developing spontaneous arterial dissections.     

Dolichoectasia of the intracranial arteries in patients with first ischemic stroke: a population-based study

OBJECTIVE: The objective of this study was to estimate the frequency of intracranial arterial dolichoectasia among patients with first ischemic stroke and to compare clinical characteristics, survival, and recurrence in those with and without the abnormality. BACKGROUND: Dolichoectasia may cause cerebral infarction by thrombosis, embolism, stenosis, or occlusion of deep penetrating arteries. METHODS: The chi-square, Fisher's exact, and logrank tests were used to compare clinical characteristics, survival, and recurrence for patients with and without dolichoectasia among the 387 residents of Rochester, MN, who had brain CT or MRI for first cerebral infarction from 1985 through 1989. RESULTS: Twelve patients (3.1%) had dolichoectasia. Patients with dolichoectasia were more likely to have had stroke fitting a clinical and radiographic pattern of lacunar infarction than those without (42% and 17% respectively; p=0.04). Dolichoectasia was detected in the vertebrobasilar system in eight patients (66.7%), in the carotid system in two patients (16.7%), and in both circulatory systems in two patients (16.7%). There were no significant differences in the following characteristics among those with and without dolichoectasia: age, sex, hypertension, diabetes, smoking, and preceding transient ischemic attack. Patients with dolichoectasia had better survival (relative risk [RR] for death, 0.26; p=0.04) after first cerebral infarction but higher rates of stroke recurrence (RR, 2.4; p=0.02). CONCLUSIONS: Dolichoectasia is detected in 38 of patients with first cerebral infarction and is associated with better survival but higher rates of stroke recurrence.     

MRI and MR angiography of vertebral artery dissection

A review of 4,500 angiograms yielded 11 patients with dissection of the vertebral arteries who had MRI and (in 4 patients) MR angiography (MRA) in the acute phase of stroke. One patient with incidental discovery at arteriography of asymptomatic vertebral artery dissection and two patients with acute strokes with MRI and MRA findings consistent with vertebral artery dissection were included. Dissection occurred after neck trauma or chiropractic manipulation in 4 patients and was spontaneous in 10. Dissection involved the extracranial vertebral artery in 9 patients, the extra-intracranial junction in 1, and the intracranial artery in 4. MRI demonstrated infarcts in the brain stem, cerebellum, thalamus or temporo-occipital regions in 7 patients with extra- or extra-intracranial dissections and a solitary lateral medullary infarct in 4 patients (3 with intracranial and 1 with extra-intracranial dissection). In 2 patients no brain abnormality related to vertebral artery dissection was found and in one MRI did not show subarachnoid haemorrhage revealed by CT. Intramural dissecting haematoma appeared as crescentic or rounded high signal on T1-weighted images in 10 patients examined 3-20 days after the onset of symptoms. The abnormal vessel stood out in the low signal cerebrospinal fluid in intracranial dissections, whereas it was more difficult to detect in extracranial dissections because of the intermediate-to-high signal of the normal perivascular structures and slow flow proximal and distal to the dissection. In two patients examined within 36 h of the onset, mural thickening was of intermediate signal intensity on T1-weighted images and high signal on spin-density and T2-weighted images. MRA showed abrupt stenosis in 2 patients and disappearance of flow signal at and distal to the dissection in 5. Follow-up arteriography, MRI or MRA showed findings consistent with occlusion of the dissected vessel in 6 of 8 patients.     

Asymptomatic occlusive lesions of carotid and intracranial arteries in Japanese patients with ischemic heart disease: evaluation by brain magnetic resonance angiography

BACKGROUND AND PURPOSE: The purpose of this study was to elucidate the prevalence and degree of asymptomatic occlusive lesions in the carotid and intracranial arteries in Japanese patients with ischemic heart disease (IHD). METHODS: We performed carotid and intracranial MR angiography (MRA) on 67 patients (49 men, 18 women; age range, 40 to 78 years; mean age, 60.1 years) who had received selective coronary angiography for the clinical diagnosis of IHD. On the basis of these images, degree of stenosis in the regions of the bilateral carotid artery bifurcation and five regions of the intracranial arteries, ie, bilateral intracranial portions of the internal carotid arteries and the middle cerebral arteries and the basilar artery were estimated. RESULTS: Stenosis of more than 25% narrowing of the diameter of the target arteries was found in 15 patients (22.4%) in the extracranial carotid arteries and in 11 patients (16.4%) in the intracranial arteries. Most of the stenotic lesions were mild. The incidence of extracranial carotid stenosis and the severity of coronary atherosclerosis showed a significant correlation. The mean age of the patients with intracranial arterial lesions was statistically higher than those without intracranial lesions. CONCLUSIONS: Our data suggest that asymptomatic occlusive lesions in the carotid and intracranial arteries are fairly common in Japanese patients with IHD, although the degree of stenosis is relatively mild. Coexistence of carotid atherosclerosis should be suspected in IHD patients with severe coronary atherosclerosis, and the possibility of atherosclerosis in the intracranial arteries should be considered in aged IHD patients.     

Digital subtraction rotational angiography for aneurysms of the intracranial anterior circulation: injection method and optimization

PURPOSE: To optimize parameters of rotational angiography for examination of the internal carotid circulation; to compare rotational angiography with standard digital subtraction angiography (DSA) in the evaluation of aneurysms of the intracranial internal carotid circulation; and to determine tolerance and safety limits of prolonged internal carotid injection angiography. METHODS: Rotational angiograms were obtained during injection of the internal carotid circulation as part of the clinical angiographic evaluation of aneurysms in 41 patients. Injection rates, X-ray delays, and fields of view were studied retrospectively. Findings at rotational angiography and standard DSA were compared. Nonionic contrast material was injected over 6 seconds, and patients were studied before and after prolonged injection angiography by physical and laboratory examination, including measurement of blood pressure, pulse, and intracranial pressure. RESULTS: Vascular conspicuity was equivalent at carotid injection rates of 4 and 5 mL/s delivered over 6 seconds. At 3 mL/s, more image manipulation was required to see small vascular structures. One-second X-ray delay combined with 6-second injection duration provided the best arterial depiction of intracranial vessels from start to end of rotational angiography. Maximal rotational resolution was with a 17-cm field of view. Identification of aneurysms and small vessels was equivalent at all injection rates. Aneurysm detection was equivalent with rotational angiography and DSA. In 9 of 31 aneurysms, the neck was defined more clearly with rotational angiography than with DSA, compared with 2 of 31 that were seen better with DSA. Aneurysms of the intracranial internal carotid circulation were seen with rotational angiography and not DSA in 12 of 41 cases. No change was noted in clinical or laboratory findings. CONCLUSION: Rotational angiography provided better definition of the aneurysmal neck and greater clarity of aneurysms than did DSA; it also improved the level of confidence in predicting the presence or absence of aneurysms, especially in the anterior communicating artery; however, in our small series it did not significantly increase the detection of aneurysms. Prolonged injection angiography was well tolerated in all patients.     

Identification of patients with autosomal dominant polycystic kidney disease at highest risk for end-stage renal disease

To identify those potential factors that, early in the course of disease, mark a population of patients with autosomal dominant polycystic kidney disease (ADPKD) who have worse renal survival, survival analysis and risk ratio calculation for 1215 ADPKD patients were performed. Survival times were calculated as time to dialysis, transplantation, or death. Risk ratios were calculated using the Cox proportional hazards model. Three hundred eighty-eight patients entered end-stage renal disease and 205 patients died. ADPKD2 subjects had longer renal survival than ADPKD1 subjects (median survival, 68 versus 53 yr; P < 0.0005; risk ratio, 2.5). Women had significantly better renal survival than men (56 versus 52 yr; P < 0.0001; risk ratio, 1.6). Subjects who were diagnosed before age 30 and those who developed hypertension before age 35 had worse renal survival than those subjects who were diagnosed after age 30 or those who remained normotensive after age 35, respectively (age of diagnosis: 49 versus 59 yr; P < 0.0001; risk ratio, 3.2; hypertension: 51 versus 65 yr; P < 0.0001; risk ratio, 4.4). Similarly, those who had an episode of gross hematuria before age 30 had a worse renal outcome than those who did not (49 versus 59 yr; P < 0.0001; risk ratio, 2.6). We have also calculated risk ratios for a combined model. When therapeutic interventions become available for this disease, these populations with high risk ratios should be considered for such interventions.     

Cerebrovascular complications of HIV in children

Two uncommon but important cerebrovascular manifestations of human immunodeficiency virus (HIV) infection in children are arteritis with formation of fusiform aneurysms and arterial sclerosis with vascular occlusion. We studied the CT and MR imaging features of HIV in two girls and one boy (9 to 18 years old) and compared them with autopsy findings in two patients. One of the children had findings consistent with small areas of subacute infarction and the other two had fusiform dilatation of the major vessels of the circle of Willis. The ischemic lesions and arteriopathy were confirmed at autopsy. In one patient, an incidental B-cell lymphoma (not visible on the imaging studies) was diagnosed.     

Brain scanning in patients with recurrent breast cancer

Brain scans were performed on 116 patients with recurrent breast cancer. Seventy-eight of 79 (98%) asymptomatic patients with recurrent disease had negative brain scans. Eleven of 37 (30%) patients with central nervous system (CNS) symptoms had scans indicative of intracranial metastases. There was no difference in the distribution of extracranial metastaic disease in patients who had positive brain scans when compared with those who did not. Brain scaning is of value in detecting intracranial metastases in patients with CNS symptoms. Brain scanning seems unnecessary, however, in the staging of patients with recurrent breast cancer who have no CNS symptoms.     

Familial aorto-cervicocephalic arterial dissections and congenitally bicuspid aortic valve

BACKGROUND: A primary arteriopathy is often implicated in the etiology of spontaneous cervicocephalic arterial dissections, but its exact nature usually remains unknown. We describe the familial occurrence of spontaneous arterial dissections and congenitally bicuspid aortic valve (BAV) and propose a common developmental defect in these families. SUMMARY OF REPORT: In the first family, a 63-year-old man suffered an extracranial internal carotid artery (ICA) dissection, and his 43-year-old cousin with BAV suffered an intracranial vertebral artery (VA) dissection. Two other family members had pathologically proven BAV. In the second family, a 31-year-old woman suffered bilateral extracranial ICA and VA dissections. Her father, at age 46, suffered an aortic dissection associated with cystic medial necrosis and BAV. Her paternal uncle died from an aortic dissection at age 59. In the third family, a 39-year-old woman suffered extracranial ICA and VA dissections, and her brother died at age 48 from an aortic dissection associated with a BAV. CONCLUSIONS: The familial occurrence of spontaneous arterial dissections and BAV suggests a common developmental defect. The aortic valvular cusps and the arterial media of the aortic arch and its branches are derived from neural crest cells, suggesting that a neural crest defect may be the underlying abnormality in these families.     

Congenital atrophic plaque

OBJECTIVE: To determine the distribution and severity of arterial lesions of intracranial and extracranial arteries in stroke patients in Chinese. METHODS: We studied 120 consecutive autopsy cases including 100 cases of stroke (50-80 years) and 20 cases of cancer as controls (50-80 years). Medium intracranial arteries and their primary branches, small intracerebral arteries, arterioles and extracranial carotid arteries (from 16 cases only) were examined with light microscopy. RESULTS: The stroke patients had significantly lower mean LA-VA ratios of the medium sized intracranial arteries and their primary branches, small intracerebral arteries and arterioles than the controls (P < 0.001). The stroke patients had higher mean VW-VR ratios of these arteries than the controls (P < 0.001). Atherosclerotic narrowing of the medium sized intracranial arteries and their primary branch were more severe than those of the extracranial caotid arteries. Moreover, the proximal portion of the primary branch arteries was much more severe than that of extracranial caotid arteries (P < 0.01, P < 0.05) in the stroke patients. CONCLUSION: Stroke patients had atherosclerotic lesions of the intracranial medium sized arteries and their primary branches and sclerotic lesions of the small intracerebral arteries and arterioles. These pathological changes resulted in thickening arterial walls and narrowing arterial lumens.     

Progressive intracranial occlusive disease associated with deficiency of protein S. Report of two cases

BACKGROUND AND PURPOSE: Deficiency of the free fraction of protein S has been associated with arterial or venous stroke. The pathogenesis of vascular occlusion in patients with protein S deficiency is not known. We present two cases of cerebral infarction and deficiency of protein S in which the subjects had progressive intracranial occlusions. CASE DESCRIPTION: A 16-year-old girl was admitted because of left brain stem infarction and protein S deficiency. Cerebral angiography disclosed stenosis of the right intracranial vertebral artery and occlusion of the left posterior cerebral artery. A second angiogram performed 18 months later disclosed occlusion of the right vertebral intracranial artery. In the second case, a 17-year-old girl was admitted because of left hemispheric cerebral infarction and protein S deficiency. Cerebral angiography showed stenosis of the left anterior cerebral artery, left supraclinoid internal artery, and left middle cerebral artery. A second cerebral angiogram performed 5 months later disclosed occlusion of the left anterior cerebral artery and poor hemispheric perfusion through the left middle cerebral artery. CONCLUSIONS: Based on our cases, we postulate that some patients with prothrombotic states may develop progressive intracranial arterial occlusions, possibly secondary to a permanent thrombogenic stimulus. We suggest routinely searching for prothrombotic states in young patients with intracranial occlusion, especially if the occlusion is progressive and other causes are not obvious.     

Apoptosis as a proinflammatory event: what can we learn from bacteria-induced cell death?

At least 2 genes, detectable by DNA methods, encode autosomal dominant polycystic kidney disease (ADPKD), which remains the most frequent and serious hereditary renal disease. PKD1 gene, localized on chromosome 16, responds for the clinical course in the majority of ADPKD patients, whereas PKD2 gene, localized on chromosome 4, is responsible for less than 10-15% of cases, with presumed milder phenotypic manifestations. To start the clinical and genetic correlation in patients with different genotypes (PKD1 vs. PKD2) in the Czech population, a pilot group of 88 patients with ADPKD was analysed. Families with PKD1 (n = 44) represented 95.6% and families with PKD2 (n = 2) 4.4% of all families investigated (n = 46). Our clinical analysis, yet based only on a limited number of PKD2 subjects, does not definitely support the concept of a milder phenotype and prognosis in PKD2 versus PKD1 patients, in terms of mean age of diagnosis (29 vs. 29 years), mean age at onset of arterial hypertension (33 vs. 33 years), more favourable renal function or ultrasound findings.     

Brain damage after aortic arch repair using selective cerebral perfusion

BACKGROUND: Selective cerebral perfusion is one of the most popular methods for cerebral protection during aortic arch repair. However, causes of postoperative brain damage are not fully understood. We analyzed brain damage after aortic arch repair using selective cerebral perfusion for true aortic arch aneurysm in regard to preoperative cerebral infarction and intracranial and extracranial occlusive arterial disease. METHODS: Over a 9-year period, 60 patients with true aortic arch aneurysm underwent aortic arch repair using selective cerebral perfusion. Postoperative brain damage was evaluated in regard to preoperative cerebral infarction detected by computed tomography, magnetic resonance imaging, or both in 50 patients and intracranial and extracranial occlusive arterial disease detected by digital subtraction angiography, magnetic resonance angiography, or both in 35 patients. RESULTS: Seven (12%) of the 60 patients died within 30 days of operation. Postoperative brain damage occurred in 6 (10.5%) (3, coma, and 3, hemiplegia) of 57 patients; 3 patients who died without awakening were excluded. Preoperatively, old cerebral infarction was detected in 9 patients (18%), and silent cerebral infarction (lacunar infarction and leukoaraiosis) was diagnosed in 26 patients (52%). Postoperative brain damage occurred in 3 (33%) of the 9 patients with preoperative cerebral infarction and in 3 (23%) of 13 patients with negative preoperative brain findings; this excludes 2 patients who died without awakening. No patient with silent cerebral infarction had postoperative brain damage. Occlusive arterial disease was detected in 7 patients (20%). The incidence of brain damage in these patients was 71% (5/7), which was significantly greater than that of 4% (1/28) in patients without occlusive arterial disease (p < 0.001). CONCLUSIONS: Silent cerebral infarction may not be a risk factor for postoperative brain damage. Preoperative evaluation of intracranial and extracranial occlusive arterial disease provides important information as to whether a patient might sustain brain damage after aortic arch repair using selective cerebral perfusion.     

Fibromuscular dysplasia of the internal carotid artery associated with alpha1-antitrypsin deficiency

OBJECTIVE: A deficiency of alpha1-antitrypsin has been implicated in the development of various disorders affecting medium-sized arteries, including intracranial aneurysms, cervicocephalic arterial dissections, and fibromuscular dysplasia (FMD). We performed alpha1-antitrypsin phenotyping in three consecutive patients who underwent bypass surgery for FMD of the extracranial internal carotid artery to test the hypothesis that alpha1-antitrypsin deficiency is a genetic risk factor for the development of FMD. METHODS: The study population consisted of three women (aged 37, 49, and 53 years, respectively) who had bilateral internal carotid artery stenosis caused by FMD. The indications for surgery included ocular or cerebral ischemic symptoms in two patients and progressive stenosis in one patient. The diagnosis of FMD was confirmed by histological examination of the resected segment of artery. The alpha1-antitrypsin phenotype was determined by isoelectric focusing in polyacrylamide gels. RESULTS: Two of the three patients had a heterozygous alpha1-antitrypsin deficiency (PiMZ phenotype). Pathological examination of the resected arterial segment showed typical medial FMD with focal intimal fibroplasia in both patients with the PiMZ phenotype. CONCLUSION: These findings suggest that a heterozygous alpha1-antitrypsin deficiency may be a genetic risk factor for the development of FMD of the internal carotid artery.     

Insulin-like growth factor binding proteins from adult-hamster pancreatic islets: influence of glucose concentration

The recognized incidence of extra- and intracranial carotid artery dissection has increased concomitantly with the progress and development of diagnostic methods. However, management for this condition is still controversial. We report in the present study the management and long-term follow-up results of 15 patients with carotid artery dissection. Mean age of the patients at onset was 47.8 years old, and male/female ratio was 12:3. Two patients were considered to be traumatic dissections and the other 13 patients were spontaneous dissections. Dissection occurred in 10 patients at the extracranial carotid artery, in 4 at the intracranial carotid artery and in 1 at the middle cerebral artery. Nine of 15 patients demonstrated hemiparesis and 5 complained of headache or facial pain. However, it was not possible to identify a characteristic symptom of dissection. Final diagnosis of dissection was made by cerebral angiography in all patients. Serial angiography was carried out in 10 of those, and 5 of the 10 patients showed some improvements of dissection in the cerebral angiogram. Treatment for those patients was selected according to the neurological and angiographical changes. Five patients were managed conservatively and 10 patients underwent surgical revascularization. During the follow-up period (mean 77.6 months), none of them showed any symptoms of reattack, and all but one, who died of heart failure 193 months after revascularization surgery, have lived independently. Although diagnosis of dissection was difficult because of the lack of characteristic symptom, serial angiography was a useful method for diagnosis and adequate management has led to a good clinical outcome.     

Cardiovascular abnormalities in children with autosomal dominant polycystic kidney disease

It is known that adults with autosomal dominant polycystic kidney disease (ADPKD) have an increased incidence of cardiovascular abnormalities, including mitral valve prolapse. The cardiac manifestations of ADPKD in the pediatric population have not been well established. To determine the cardiac manifestations of children with ADPKD, echocardiography was performed in 154 children of 66 families in which one parent has ADPKD. Eighty-six affected children and 68 unaffected children were evaluated in a prospective, single-blinded manner by echocardiography. Affected children were defined as those with any cysts on a concurrent renal ultrasound or those predicted to be gene carriers by gene linkage analysis. A 12% incidence of mitral valve prolapse was found in the affected children compared with only 3% of the unaffected children (P < 0.05). ADPKD children, but not their unaffected siblings, demonstrate a significant correlation between left ventricular mass index and systolic blood pressure. Moreover, hypertensive ADPKD children have significantly larger left ventricular mass index than do normotensive ADPKD children. A 3.5% incidence of congenital heart disease was found in the affected group, whereas 2.9% of the unaffected children had congenital heart disease. It was concluded that systemic manifestations of ADPKD, particularly cardiovascular abnormalities, are present even in childhood and these warrant the clinician's attention.     

Ophthalmic manifestations of vertebral artery dissection. Patients seen at the Mayo Clinic from 1976 to 1992

PURPOSE: To determine the ophthalmic manifestations of vertebral artery dissections. METHODS: Fifty-one separate episodes of vertebral artery dissections evaluated at the Mayo Clinic from 1976 through 1992 were studied. In all cases, the diagnosis had been documented with angiography. RESULTS: There were 28 men and 19 women (mean and median age, 39 and 40 years, respectively; range, 8-61 years). There were ophthalmic findings in 86% of the episodes. Visual symptoms, in decreasing order of frequency, were diplopia (45% of the episodes), blurred vision (14%), transient visual dimming (8%), oscillopsia (4%), photophobia (4%), upside-down vision (2%), positional transient visual obscuration (2%), and unilateral dry eye (2%). Ophthalmic signs in decreasing order of frequency were nystagmus (37% of the episodes), ocular misalignment (cranial nerve palsy or skew) (33%), Horner syndrome (27%), decreased corneal sensation (22%), ptosis (16%), visual field defect (10%), abnormal pursuits and saccades (6%), ocular bobbing (4%), internuclear ophthalmoplegia (4%), anisocoria (4%), and pinpoint pupils (2%). CONCLUSION: Vertebral artery dissections occur in a relatively young population. Most of the patients in our study had ophthalmic manifestations at the time of diagnosis. Heightened awareness of this entity should enable the consulting ophthalmologist to have a role in the diagnosis and treatment of vertebral artery dissection.