High accuracy of bilateral and simultaneous petrosal sinus sampling with desmopressin for the differential diagnosis of pediatric ACTH-dependent Cushing's syndrome.

PURPOSE: To analyze the bilateral and simultaneous petrosal sinus sampling (BIPSS) in a subgroup of children and adolescents with ACTH-dependent Cushing's syndrome (ADCS) METHODS: Retrospective study in a tertiary reference center. From 1993 and 2017, 19 children and adolescents (PED) were submitted to the BIPSS, median age of 14 years (range 9-19 years), 53% were males, 18 had Cushing's disease (CD) and one had ectopic ACTH syndrome (EAS). All procedures were performed with 10 µg of intravenous desmopressin. RESULTS: The catheter positioning was successful in all cases. The central ACTH gradient was met in 17/19 cases. At baseline, central gradient occurred in 16/19 (84%) with gradient values of 7.2 ± 6.0. After stimulation, there was an increase in the center-periphery gradient values (33.6 ± 44.3). In one case, central gradient was defined only after stimulation. Two cases presented without a central gradient; one case of CD with a false-negative and one EAS case. Lateralization occurred in all cases with a central gradient. Confirmation of the tumor location presumed by the procedure with the surgical description occurred in 60% of the cases. The BIPSS in this PED subgroup of ADCS presented a sensitivity of 94.4% and specificity of 100%. There were no complications of the procedure. CONCLUSION: In a series of children and adolescents with ADCS, BIPSS was safe and highly accurate in defining the central to peripheral ACTH gradient using desmopressin as secretagogue. Nevertheless, there was a limited value of the ACTH-gradient between the petrosal sinuses for the tumor location.

Basilar artery occlusive disease in stroke survivors in a multiethnic population.

OBJECTIVES: To describe clinical, radiological findings, and outcome in a multiethnic population of stroke survivors with basilar artery occlusive disease (BAOC). METHODS: Forty patients with infarcts in the basilar artery (BA) territory, alive 30 days after the ictus, participated in the study. BA stenosis (>50%) or occlusion was shown by magnetic resonance or digital subtraction angiography in all patients. Demographical, clinical and radiological characteristics were described. Modified Rankin Scale (MRS) scores at 30 days and 6 months after the ischemic event were evaluated. Association between demographical, clinical, radiological features and outcome were analyzed with Chi-square and Fisher's exact tests. MRS scores at 30 days and 6 months were compared with the Wilcoxon test. RESULTS: Sixty percent of the patients were men, and 33% were Afro-Brazilian. Mean age was 55.8+/-12.9 years. Most (90%) had multiple vascular risk factors. Stroke was preceded by TIA in 48% of the patients, and 80% had a history of arterial hypertension. The most common neurological symptom was vertigo/dizziness (60%) and the sign, hemiparesis (60%). Most of the infarcts were located in the pons (85%) and the BA middle third was the most frequently affected segment (33%). BA occlusion occurred in 58% of the patients. More severe vascular occlusive lesions were present in Whites (p=0.002) and in patients with involvement of the middle third of the BA (p=0.021). Large-artery atherosclerosis was the most common stroke etiology (88%) and was more frequent in older patients (p<0.001). Most patients were treated with anticoagulation. MRS scores improved significantly at 6 months (p<0.001); at this time, 78% of the patients had MRS scores between 0 and 2. CONCLUSIONS: We observed different results compared with other series: greater proportion of Afro-descendents, higher frequency of atherosclerosis and BA occlusion. Rates of preceding TIAs and good outcome at 6 months were similar to previously published data. These results represent a step forward towards understanding BAOC in a multiethnic context.

[Inferior petrosal sinus catheterization: technical aspects]. / Cateterização dos seios petrosos inferiores: aspectos técnicos.

PURPOSE: Inferior petrosal sinus catheterization and sampling for corticotropin dosage helps to differentiate hypophisary and ectopic forms of Cushing syndrome. The aim of this paper is to describe the technique used in inferior petrosal sinus catheterization in our service, emphasizing the solution found for frequent difficulties, and verify the success rate achieved. PATIENTS AND METHODS: Between September/2000 and September/2005, forty-two (eighty-four sinuses) patients were submitted to inferior petrosal sinus sampling. The difficulties for correct catheter positioning were identified and correlated with their solutions. RESULTS: Anatomical variations, similarity between IPS and emissary vein of the basilar plexus and unfavorable flow to the contrastation of the structures (retrograde catheterization) were the main problems. Using pre-shaped catheters, curved, steerable guide-wires, road-mapping and venography by contalateral injection, besides criteria to differentiate IPS from the emissary vein. Of the 84 sinuses approached, one was thrombosed, and 80 (96.4%) of 83 possible were selectively catheterized. No clinical complication occurred. CONCLUSION: IPSC can be safe and successfully performed in most cases. The identification of the emissary vein of the basilar plexus and use of venography by contralateral injection, improved the method performance.

Intracranial vascular lesions associated with small epidural hematomas.

OBJECTIVE: We studied the angiographic findings in patients with small epidural hematomas and cranial fractures crossing over the trajectory of the middle meningeal artery and its branches. Additionally, the occurrence of traumatic vascular lesions and their clinical relevance and treatment are discussed. METHODS: A consecutive analysis was performed for 24 patients who harbored small epidural hematomas in middle meningeal artery topography associated with cranial fractures. Computed tomographic scans and plain x-ray studies were used to diagnose linear cranial fractures. Patients with large epidural hematomas or associated traumatic lesions were excluded from the study. Selective ipsilateral external carotid angiograms were obtained, and an endovascular procedure was performed if any vascular injury was evidenced. RESULTS: In all patients with cranial fractures crossing over the middle meningeal artery and its branches, some kind of vascular lesion was seen. Two types of findings were noted: active extravasation of the contrast medium (71%) and pseudoaneurysms (29%). Early filling of diploic vessels was found in 8.3% of fractures concomitantly with active extravasation. Embolization was performed in all patients. No additional enlargement of the epidural hematoma was observed, and the postoperative period was uneventful. CONCLUSION: This study shows that pseudoaneurysms and active extravasation of contrast are common findings in this subset of patients. Although the natural history of these lesions is still poorly understood, additional investigation with ipsilateral external carotid angiography may be recommended, considering the potentially catastrophic consequences of late rupture.

Endovascular treatment of a basilar artery dissecting aneurysm.

Basilar artery (BA) dissecting aneurysms pose difficulties to treatment because both bleeding and thrombosis can happen in the same patient, clinical course is unpredictable and high morbidity is usual. We report the case of a 37-year-old woman with a BA aneurysm probably caused by arterial dissection, presenting embolic and hemorrhagic complications. The aneurysm was submitted to endovascular treatment with stenting and coil embolization. Clinical and radiological results were excellent and no complications were observed, suggesting that BA stenting and coil embolization may be a safe and effective treatment for this condition.

"Salt and pepper" in the eye and face: a prelude to brainstem ischemia.

PURPOSE: To report the "salt and pepper"(SP) eye pain as a herald symptom of pontine ischemia. DESIGN: Observational case series. METHODS: We reviewed clinical and neuroimaging findings of four patients who presented initially with the sensation of SP in the eyes and then developed paramedian pontine infarcts confirmed by neuroimaging. RESULTS: All of the patients developed other neurologic symptoms or signs, either in association with the sensation of SP in the eyes, from hours to days later. Magnetic resonance imaging (MRI) showed paramedian pontine infarcts in all of the patients and angiography showed basilar artery occlusive disease in three of them. CONCLUSION: Impending pontine ischemia is an important differential diagnosis in patients with acute ocular pain. Prompt neurovascular evaluation and treatment may avoid devastating brainstem infarcts that cause death or long-term disability, particularly in patients with basilar artery occlusive disease.

The role of desmopressin in bilateral and simultaneous inferior petrosal sinus sampling for differential diagnosis of ACTH-dependent Cushing's syndrome.

OBJECTIVE: Bilateral inferior petrosal sinus sampling (BIPSS) with corticotrophin-releasing hormone (CRH) stimulation is currently the gold standard test for the differential diagnosis of ACTH-dependent Cushing's syndrome. Reports on the use of desmopressin in this approach are limited. The aim of this study was to evaluate the use of desmopressin during BIPSS in a cohort of patients with ACTH-dependent Cushing's syndrome. DESIGN: A retrospective case-record study. PATIENTS: Fifty-six patients with confirmed ACTH-dependent Cushing's syndrome underwent BIPSS with desmopressin stimulation when presenting negative pituitary tumour imaging. MEASUREMENTS: Central to peripheral (CEN:PER) ACTH gradient, lateralization of the ACTH source and surgical tumour confirmation were evaluated. RESULTS: A CEN:PER ACTH gradient was found in 40 patients under basal conditions (CEN:PER >or= 2) and in 47 patients after desmopressin stimulation (CEN:PER >or= 3). Ectopic ACTH-producing tumours (three lung carcinoid tumour, one thymus carcinoid tumour and one thymus hyperplasia) were confirmed in five out of nine patients without the CEN:PER ACTH gradient, and four cases were false negative for Cushing's disease. Lateralization (IPS:IPS >or= 1.4) was observed in 80.8% of patients under basal conditions (38/47) and in 97.8% after desmopressin (46/47), and it was surgically confirmed in 78.7%. There were no false-positive cases. Sensitivity and specificity were 92.1% and 100%, respectively. CONCLUSIONS: Desmopressin improves the differential diagnosis of ACTH-dependent Cushing's syndrome by amplifying the CEN:PER and IPS:IPS ACTH gradients, and is therefore a useful ACTH secretagogue in BIPSS.

Bilateral occipital infarcts associated with carotid atherosclerosis and a persistent hypoglossal artery.

The persistent hypoglossal artery (PHA) is the second most common persistent embryological carotid-basilar connection and usually represents an incidental finding in cerebral arteriograms. The hypoglossal artery connects the primordial carotid artery with the longitudinal neural arteries, which later form the basilar artery. The PHA leaves the internal carotid artery as an extracranial branch, enters the skull through the anterior condyloid foramen, the hypoglossal canal and joins the caudal portion of the basilar artery. We report magnetic resonance and digital subtraction angiography findings in the first case of bilateral occipital infarctions associated with PHA and carotid atherosclerosis. The probable mechanism underlying bilateral occipital infarcts was embolism from the carotid territory to the posterior cerebral arteries. PHA may present a challenge in diagnosis and management of patients with carotid atherosclerosis and vertebrobasilar ischemia.

Comparison between digital subtraction angiography and magnetic resonance angiography in investigation of nonlacunar ischemic stroke in young patients: preliminary results.

PURPOSE: We preliminarily investigated the relevance of performing digital subtraction angiography (DSA) in addition to magnetic resonance angiography (MRA) in definition of ischemic stroke etiology in young patients. METHOD: DSAs and MRAs from 17 young patients with nonlacunar ischemic stroke were blindly analyzed and their impact on stroke management was evaluated. RESULTS: Etiologies were the same considering results of either DSA or MRA in 12/17 cases. In 15/17 patients no changes would have been made in treatment, regardless of the modality of angiography considered. CONCLUSION: These preliminary results suggest that DSA may be redundant in two thirds of ischemic strokes in young patients. Further larger prospective studies are necessary to determine indications of DSA in this age group.

[Clinical and interventional revascularization in the acute ischemic stroke: national opinion]. / Revascularização clínica e intervencionista no acidente vascular cerebral isquêmico agudo: opinião nacional.

The Brazilian Stroke Society constituted a committee composed by specialists from different areas of Brazil that emitted a viewpoint called "National Opinion," considering the interventional procedures and thrombolysis in the treatment of the acute ischemic stroke. This study presents the conclusions of this committee.

Revascularização clínica e intervencionista no acidente vascular cerebral isquêmico agudo: opinião nacional / Clinical and interventional revascularization in the acute ischemic stroke: national opinion

Este artigo apresenta as conclusões sobre revascularização clínica e intervencionista no acidente vascular cerebral isquêmico agudo, um dos temas discutidos na reunião "Opinião Nacional sobre o Tratamento do AVC". Tratou-se de reunião promovida e coordenada pela Sociedade Brasileira de Doenças Cerebrovasculares, com neurologistas especializados em doenças cerebrovasculares, que analisaram e discutiram as evidências e experiências atuais sobre o uso de trombólise e técnicas intervencionistas em pacientes com acidente vascular cerebral isquêmico agudo.

Progressive cervicocranial arteriopathy with dilatations and stenoses: case report.

We report the case of a 36 year-old woman who presented occlusion of a basilar artery fusiform aneurysm (FA) associated with pontine infarction, and two episodes of subarachnoid hemorrhage possibly due to arterial dissection. She also had asymptomatic FAs in the right middle cerebral and left internal carotid arteries. Over 5 years, lesions suggestive of fibromuscular dysplasia in the right vertebral artery and occlusion of the left vertebral artery were observed. This combination of lesions emphasizes the possibility of a common pathogenetic mechanism causing different degrees of media disruption in cervicocranial arteries.

[Spontaneous cervical carotid and vertebral arteries dissection: study of 48 patients]. / Dissecção espontânea cervical carotídea e vertebral: estudo de 48 pacientes.

OBJECTIVE: To report a Brazilian series of spontaneous cervical arterial dissections, risk factors, warning symptoms, clinical manifestations, diagnostic tests, treatment and prognosis. METHOD: We performed the retrospective analysis of clinical and neuroradiological records (MRI, A-MRI and Angiography) of patients with this diagnosis who were evaluated in a tertiary hospital for the period of 1997-2003. RESULTS: 48 patients (24 men) with median age 37.9 years: 26 patients with unilateral internal carotid dissection (ICAD), 15 with unilateral vertebral artery dissection (VAD) and 7 with multivessel dissections. All patients presented neurological deficits. Hypertension, smoking and dyslipidemia were the main risk factors. More than 80% of patients presented at least one initial symptom, most of them temporoparietal headache. 44% of patients with VAD and only 3.4% of patients with ICAD had neck pain. The median interval between the onset of symptom and the appearance of neurological deficit was 5.4 days for ICAD and 13.5 days for VAD. Five patients with ICAD presented preceding TIA. Angiography was performed in 93% of patients. In 42% of these patients, MRI and A-MRI were associated. In three patients the diagnosis was made just through cervical MRI. 75% of patients received anticoagulation. Two patients received intravenous thrombolytic therapy with no complications. Prognosis was good for all patients but two patients with bilateral ICAD died. CONCLUSION: Our results are similar to the literature, except for the low frequency of neck pain in ICAD patients and predominance of temporoparietal headache in cervical artery dissection patients. Vascular risk factors were commonly found.

Treatment of subclavian steal syndrome with percutaneous transluminal angioplasty and stenting: case report.

Subclavian steal syndrome refers to the association of neurological symptoms related to vertebrobasilar insufficiency and the phenomenon of subclavian steal. We report the case of a 63 year-old male patient that presented subclavian steal syndrome and severe proximal (80%) stenosis of the left subclavian artery. The patient was submitted to percutaneous transluminal angioplasty and stenting on the left SA. The procedure was well tolerated and immediately afterwards, there was complete remission of the symptoms and of the phenomenon of subclavian steal evaluated by angiography and transcranial doppler. We propose that percutaneous transluminal angioplasty with stenting placement is a good therapeutic option for subclavian steal syndrome.