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.

The intracarotid etomidate Wada test: a 54-patient series.

Hemispheric language dominance and isolated hemispheric memory function evaluation can be undertaken with the intracarotid injection of a general anesthetic agent (Wada test). Amobarbital has been traditionally used as the anesthetic agent, but legal and commercial constraints limit its use. We evaluated the use of etomidate as an alternative agent for the Wada test in a series of 54 consecutive adult patients with mesial temporal sclerosis undergoing presurgical evaluation for epilepsy surgery. Language lateralization and hemispheric memory function evaluation were successfully achieved in all cases. Side effects (somnolence, tremor, and dystonia) were infrequent, minor, and transient and did not require interruption of the procedure. Etomidate appears to be a safe and effective alternative agent to amobarbital in the Wada test. Similarly to the amobarbital Wada test, the ability of the etomidate Wada test to predict postoperative memory decline remains unclear.

Endovascular management of acute epidural hematomas: clinical experience with 80 cases.

OBJECTIVE Small acute epidural hematomas (EDHs) treated conservatively carry a nonmeasurable risk of late enlargement due to middle meningeal artery (MMA) lesions. Patients with EDHs need to stay hospitalized for several days, with neurological supervision and repeated CT scans. In this study, the authors analyzed the safety and efficacy of the embolization of the involved MMA and associated lesions. METHODS The study group consisted of 80 consecutive patients harboring small- to medium-sized EDHs treated by MMA embolization between January 2010 and December 2014. A literature review cohort was used as a control group. RESULTS The causes of head injury were falls, traffic-related accidents (including car, motorcycle, and pedestrian vs vehicle accidents), and assaults. The EDH topography was mainly temporal (lateral or pole). Active contrast leaking from the MMA was seen in 57.5%; arteriovenous fistulas between the MMA and diploic veins were seen in 10%; and MMA pseudoaneurysms were found in 13.6% of the cases. Embolizations were performed under local anesthesia in 80% of the cases, with N-butyl-2-cyanoacrylate, polyvinyl alcohol particles, or gelatin sponge (or a combination of these), obtaining MMA occlusion and complete resolution of the vascular lesions. All patients underwent follow-up CT scans between 1 and 7 days after the embolization. In the 80 cases in this series, no increase in size of the EDH was observed and the clinical evolution was uneventful, without Glasgow Coma Scale score modification after embolization and with no need for surgical evacuation. In contrast, the control cohort from the literature consisted of 471 patients, 82 (17.4%) of whom shifted from conservative treatment to surgical evacuation. CONCLUSIONS This study suggests that MMA embolization is a highly effective and safe method to achieve size stabilization in nonsurgically treated acute EDHs.

"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.

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.

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.

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.

Surgical treatment of extracranial arteriovenous malformations after multiple embolizations: outcomes in a series of 31 patients.

BACKGROUND: Surgical resection after embolization is the most accepted approach to treating arteriovenous malformations. The authors analyzed the outcome of surgically treated patients and how surgical resection was influenced by multiple embolizations. METHODS: Thirty-one patients were included from January of 2000 to December of 2012. The mean patient age was 24.9 years. Anatomical involvement, definition of limits, functional impairment, number of embolizations, type of resection, reconstruction method, blood transfusion, and hospital stay were evaluated. Morbidity, mortality, and regrowth rates and need for additional procedures were evaluated. RESULTS: Lesions were preferentially located at the orbits, cheeks, and lips. The number of embolizations per patient increased with lesion complexity. In 22 cases, total excision was accomplished, and in nine, subtotal resections were performed to favor function. After multiple embolizations, better lesion identification was observed. Primary closure was performed in 20 cases, local flaps were performed in seven cases, axial flaps were performed in two patients, and free flaps were performed in two cases. There were no deaths. Regrowth rates were influenced by limits between arteriovenous malformations and surrounding tissues (15.8 percent of cases with precise limits versus 58.3 percent of lesions with imprecise limits; p = 0.021) and by type of resection (18.2 percent of cases after total resection versus 66.7 percent after subtotal resections; p = 0.015). CONCLUSIONS: Multiple therapeutic embolizations seem to increase safety in the treatment of arteriovenous vascular malformations and suggest an additional positive effect besides bleeding control. Preoperative definition of limits and establishment of conditions for total resection are critical to determine management and risk of regrowth. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Contralateral ictal electrographic involvement is associated with decreased memory performance in unilateral mesial temporal sclerosis.

PURPOSE: To evaluate the effect of contralateral electrographic involvement on memory performance (measured by neuropsychological and Wada memory testing) in patients with epilepsy associated with unilateral mesial temporal sclerosis (MTS). METHODS: We studied 51 patients with medically-refractory epilepsy associated with unilateral MTS (27 women, 30/51, left MTS) submitted to prolonged non-invasive video-EEG monitoring and bilateral Wada testing. According to ictal electrographic involvement, patients were classified as: Contralateral ictal involvement, when one or more seizures evolved with rhythmic activity in the temporal region contralateral to the MTS or exclusive ipsilateral ictal involvement if all seizures showed ictal EEG activity exclusively on the MTS side. Wada testing involved a twelve-item memory paradigm. Wada memory asymmetry score was calculated for each patient subtracting the number of recalled items after injection on the lesion side from the number of recalled items after contralateral injection. Expected asymmetry (EA) was considered if Wada memory asymmetry>0, and Symmetrical or Reversed memory asymmetry (S-RA) when ≤ 0. Neuropsychological testing was applied in the 51 patients and in 40 healthy controls. Verbal Memory was evaluated with the Rey Auditory Verbal Learning Test (RAVLT), considering the number of recalled items on immediate recall after the initial five consecutive encoding trials (RAVLT 6), a post-interference delayed (30 min) recall (RAVLT 7), and recall after 7 days. Nonverbal memory was tested with Wechsler Memory Scale-III (WMS-III) Faces subtests 1 e 2. RESULTS: Groups did not differ in demographic, clinical and video-EEG monitoring variables. S-RA was observed more frequently in the group with contralateral ictal involvement (57.2% vs. 27.0%; p: 0.03). Logistic regression analysis considering demographic, clinical, hippocampal volume and video-EEG monitoring variables showed contralateral ictal involvement as the only independent variable associated with S-RA (coefficient=1.32, p=0.029, odds ratio 3.77; 95% CI 1.1-12.47). Additionally, the patient group with contralateral ictal EEG involvement displayed worse verbal and nonverbal memory scores compared to healthy controls. CONCLUSION: In this cohort of unilateral MTS patients, contralateral ictal involvement was associated with decreased memory performance on Wada and on neuropsychological testing.

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.

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.