Prosopamnesia: a case report of amnesia for faces.

Prosopamnesia is a face-selective memory disorder in which face learning is impaired, while face-perception disorder (prosopagnosia) and memory disorders for stimuli other than faces are not present. To date, only two cases of prosopamnesia have been reported in adults - one congenital and one secondary to brain damage. This article reports a case of a 68-year-old woman complaining difficulties recognizing persons she had got to know recently. Neuropsychological examination revealed face-specific anterograde amnesia in the absence of prosopagnosia and other memory impairments. Brain MRI did not present any focal abnormality; PET-scan revealed hypoactivation mostly in the frontotemporal area bilaterally. This patient represents the first case of late-onset primary prosopamnesia.

Neuropsychological assessment in acute stroke patients.

BACKGROUND AND PURPOSE: The number of people suffering from stroke is strongly increasing, giving rise to multiple cognitive deficits which frequently prevent a full recovery. The identification of both spared and impaired cognitive domains has a key role to plan adequate interventions. However, the existing standard tests are either too expensive in terms of time and efforts for patients in acute stage or they derived from instruments addressing different pathologies such as dementia. METHODS: We developed a brief neuropsychological battery (mental performance in acute stroke, MEPS) to assess different cognitive domains (language, memory, praxis, visual perception) in acute stroke patients. MEPS was validated by enrolling a sample of 204 patients suffering from stroke in acute stage, and 263 healthy controls participants. RESULTS: The results indicated an adequate construct validity and a high ability in discriminating patients from healthy controls. CONCLUSIONS: MEPS can be considered a simple and highly valuable bedside battery, easy to administer, with values of sensitivity and specificity suitable to be proposed as a screening tool for patients with acute stroke.

Degradable starch microspheres transarterial chemoembolization (DSMs-TACE) in patients with unresectable hepatocellular carcinoma (HCC): long-term results from a single-center 137-patient cohort prospective study.

PURPOSE: To evaluate safety and efficacy of degradable starch microspheres (DSMs) TACE in a large clinical cohort of patients with unresectable HCC. MATERIALS AND METHODS: This is a single-center consecutive patients cohort study. The study was approved by local institutional ethics committee. Written informed consent was obtained. From December 2013 to March 2018, 137 cirrhotic patients with unresectable HCC were enrolled. For DSMs-TACE, a mixture of 4 mL of DSMs, 6 mL of non-ionic contrast and doxorubicin at a dose of 50 mg/m2 were used. Primary end point was long-term outcome, in terms of time to progression (TTP) and overall survival (OS). Secondary endpoints were: safety, liver toxicity, 1-month percentage of tumor necrosis according to the modified RECIST criteria. RESULTS: Two hundred and sixty-seven DSMs-TACE were performed in 137 HCC patients (33 patients in BCLC stage A, 84 patients in BCLC stage B, and 20 in stage C). Patients had a mean nodule number of 3.5 ± 1.2 (SD). Major complications were observed in 6.8% of cases. Post-embolization syndrome was common (101 patients 73.7%). According to mRecist criteria, a high objective response rate was obtained even after just one treatment (84.3% of patients showed complete response or partial response). The median TTP and OS after DSMs-TACE were 12 months and 36 months, respectively. OS at 6 months, 1 year, 2 and 3 years was 98%, 81.3%, 57.9%, 34.9%, respectively. CONCLUSION: DSMs-TACE is a safe and effective therapy for patients with HCC, allowing to obtain a good rate of OS with excellent local tumor control.

Heparin during endovascular stroke treatment seems safe.

BACKGROUND AND PURPOSE: the effect of intravenous heparin during mechanical thrombectomy for acute ischemic stroke is not clear. We aimed to study efficacy and safety of heparin use during endovascular stroke treatment in a real-world setting. MATERIALS AND METHODS: patients with anterior circulation stroke were divided, based on the use of intraprocedural heparin, in those treated and those untreated. Main outcomes were successful reperfusion defined as a TICI Score ≥ 2b, 3-month functional independence defined as a modified Rankin Scale ≤ 2, symptomatic intracranial hemorrhage (sICH) and mortality. RESULTS: 361 patients were eligible for analysis; 200 were (H+) and 161 (H-). The (H-) group showed higher age and ASPECTS (74 ± 14 vs. 68.9 ± 12.2; P = 0.001; 8 ± 1.6 vs. 7.4 ± 2.1; P = 0.009) without differences in vascular risk factors. Heparin untreated patients showed a shorter onset-to-reperfusion time (271 ± 57.6 min vs. 309 ± 102.2 min; P < 0.001). No differences were found in 3-month functional independence, sICH and mortality whereas the rate of successful reperfusion was higher in the (H-) group. After logistic regression analysis successful reperfusion was independently associated with CT ASPECTS (OR: 1.16; 95%CI 1.01-1.35; P = 0.040) but inversely associated with the use of heparin (OR: 0.48; 95% CI 0.24-0.98; P = 0.045). CONCLUSIONS: Heparin use during mechanical thrombectomy for anterior circulation acute ischemic stroke in a real world setting is safe.

Endovascular Distal Plantar Vein Arterialization in Dialysis Patients With No-Option Critical Limb Ischemia and Posterior Tibial Artery Occlusion: A Technique for Limb Salvage in a Challenging Patient Subset.

PURPOSE: To detail a percutaneous technique for distal plantar venous arterialization in diabetic, end-stage renal disease (ESRD) patients with no-option critical limb ischemia (CLI). TECHNIQUE: After failure of standard intraluminal recanalization attempts, a subintimal approach through the posterior tibial artery (PTA) is begun using a 0.014-inch, 190- or 300-cm-long guidewire supported by a 2-×20-mm, low-profile balloon catheter positioned a short distance behind the narrow "U-shaped" loop in the guidewire. Typically, heavy calcification in the distal tortuous segment of the PTA prevents reentry to the arterial true lumen; however, an entry in the distal lateral or medial plantar vein from a subintimal channel in the plantar artery can be intentionally pursued as a bailout technique, pointing the tip of the guidewire opposite to the arterial wall calcifications. Venous access is confirmed by contrast injection through the balloon catheter. Once the guidewire is advanced in the distal lateral or medial plantar vein and a plantar arteriovenous fistula (AVF) has been created, the AV anastomosis and the occluded PTA segment are dilated with 0.014-inch balloon catheters. The technique has been attempted in 9 consecutive diabetic, ESRD patients (mean age 69 years; 5 men) with no-option CLI; an AVF was created between the PTA and plantar vein in 7 patients. The mean TcPO2 at 1 month was 30±17 mm Hg (vs 7.3±2.2 at baseline). Six ulcers healed over an average of 21±4 weeks. Three of the 9 patients had below-knee amputations. CONCLUSION: Although further investigations are required, distal plantar venous arterialization may represent a promising technique to improve recanalization rates and limb salvage in diabetic ESRD patients with extremely calcified PTA occlusions.

Role of MRI with hepatospecific contrast agent in the identification and characterization of focal liver lesions: pathological correlation in explanted livers.

AIM: To assess the diagnostic performance of magnetic resonance imaging (MRI) with gadoxetic acid in the identification of hepatocellular carcinoma (HCC) nodules by comparison with histological findings. METHODS: In a cohort of patients suffering from cirrhosis of various etiologies (chronic hepatitis C virus (HCV) or hepatitis B virus (HBV), alcohol abuse, cryptogenic forms), we selected 17 patients affected by HCC who were eligible for liver transplantation on the basis of a computed-tomography (CT) total-body examination. Such patients also underwent an MRI examination under basal conditions, and with four dynamic phases, as well as a hepatobiliary phase acquired after at least 20 min and recognized by the excretion of contrast agent into the bile duct, following intravenous administration of 0.05 mol/kg of gadoxetic acid (gadoxetate disodium, Primovist(®); Bayer, Osaka, Japan). The MRI images were then evaluated in a double-blinded experimental setup by two radiologists experienced in imaging of the liver. The diagnosis of HCC was made in the presence of nodular lesions that showed typical or atypical enhancement patterns. The liver was subsequently explanted (on average 47.4 days after MRI evaluation), dissected into 1-cm samples, and histologically evaluated according to the classification of Edmondson-Steiner. RESULTS: At the histopathological examination, 46 nodules were identified, on average 2.7 nodules for each patient. Of these, 37 were hepatocellular carcinomas, 3 were characterized by histologically unrecognizable complete necrosis, and 6 showed high-grade dysplasia. MRI with hepatospecific contrast medium showed inter-observer average values of sensitivity, specificity, and diagnostic accuracy of 94.6, 90, and 93.6 %, respectively. In one case, a nodule was not identified by MRI with gadoxetic acid, even in the hepatospecific phase (false negative (FN)). This result could be implicated to the long time interval between the analysis and the explant (88 days). In another case, there was an overdiagnosis of a HCC with a typical nodular pattern (false positive (FP)), but which most likely should have been attributed to a previous echinococcus cyst. MRI analysis, in combination with the study of the hepatobiliary phase, also showed a greater sensitivity, the same specificity, and a greater diagnostic accuracy compared to MRI evaluated only in the dynamic phases (with an average percentage between the two operators, respectively, of 75.7, 90, and 78 %). CONCLUSIONS: MRI with gadoxetic acid shows a diagnostic accuracy superior to contrast-enhanced MRI, allowing for the diagnosis of additional lesions, and it could be considered as an imaging method to carry out a more appropriate management of waiting lists for liver transplants.

Foot Embolization During Limb Salvage Procedures in Critical Limb Ischemia Patients Successfully Managed With Mechanical Thromboaspiration: A Technical Note.

PURPOSE: To illustrate the use of a mechanical thromboaspiration device originally designed for clot retrieval in acute stroke in the treatment of acute distal embolism occurring during percutaneous revascularization of the femoropopliteal and below-the-knee arterial segments. TECHNIQUE: The Penumbra system was adapted for aspiration of thrombus in the distal foot arteries as a standalone device. The 2 over-the-wire, tapered lumen catheters have long working lengths (139 cm for the 4MAX to 153 cm for the 3MAX) that allow advancement below the ankle even with a retrograde contralateral approach. Once the occluded arterial segment is reached, the catheters are connected to the dedicated pump for continuous vacuum aspiration. The use of the device is illustrated in 3 diabetic patients (1 woman and 2 men; ages 88, 70, and 73 years, respectively) undergoing limb salvage procedures who experienced distal embolization that would have seriously jeopardized the foot circulation. The lumens of the occluded arteries were restored without complication. CONCLUSION: While further evaluation in a larger cohort of patients is needed, this initial experience using the Penumbra system in the peripheral vasculature suggests that this is a rapid, effective approach to address intraprocedural foot embolization and avoid possible grave clinical sequelae.

Treatment of chronic SFA in-stent occlusion with combined laser atherectomy and drug-eluting balloon angioplasty in patients with critical limb ischemia: a single-center, prospective, randomized study.

PURPOSE: To compare the safety and efficacy of laser debulking (LD) and drug-eluting balloon (DEB) angioplasty to treatment with DEB angioplasty alone in patients affected by critical limb ischemia (CLI) and superficial femoral artery (SFA) chronic stent occlusion in a prospective, randomized study. METHODS: Among 448 CLI patients treated from December 2009 to March 2011, 48 patients (39 men; mean age 72.7±7.8 years) with chronic SFA in-stent occlusion were randomly assigned to treatment using LD+DEB (n=24) or DEB angioplasty alone (n=24). Patency at 12 months was the primary outcome measure; secondary outcomes were target lesion revascularization (TLR) and clinical success at 12 months. RESULTS: In the LD+DEB group, the patency rates at 6 and 12 months (91.7% and 66.7%, respectively) were significantly higher (p=0.01) than in the DEB only patients (58.3% and 37.5%, respectively). TLR at 12 months was 16.7% in the LD+DEB group and 50% in the DEB only group (p=0.01). Two (8%) patients needed major amputations in the LD+DEB group vs. 11 (46%) in the DEB only group at 12 months (p=0.003). CONCLUSION: In this small initial experience, combined treatment with LD and DEB angioplasty is correlated with better outcomes in CLI patients with occluded SFA stents.

Intracranial Stenosis Treated with Stenting in Patients with Suspected Cerebral Vasculitis: Two Case Reports.

Central nervous system vasculitis (CNSV) is an uncommon and poorly understood form of vasculitis. Early recognition is important because medical treatment might improve the outcome. However, randomized clinical trials on CNSV treatment do not exist. Endovascular treatment has been reported in few cases, but no data exist for intracranial stenting. We report 2 cases of patients with suspected CNSV and recurrent clinical episodes, treated with intracranial stenting. A 48-year-old man had relapsing episodes of right hemiparesis. Neuroradiological exams showed severe left carotid terminus stenosis. Despite immunosuppressive therapy, neuroradiological follow-up exams showed a worsening of the aforementioned stenosis with many transient episodes of weakness in the right limbs and aphasia. A 64-year-old woman had a sudden onset of dysarthria and transient aphasia. Neuroradiological exams showed a severe arterial stenosis involving the origin of left anterior cerebral artery and middle cerebral artery (MCA). Despite dual antiplatelet therapy, she presented an acute onset of severe aphasia, due to an occlusion of the left carotid terminus and proximal MCA. In both cases, endovascular procedure and intracranial stenting was performed, with marked improvement of cerebral blood flow. No more clinical episodes were reported. Intracranial stenting may be a valid therapeutic option in selected patients with CNSV and involvement of medium or large size vessels with clinical worsening despite best medical treatment.

Neuropsychological assessment of Boucher-Neuhäuser syndrome: A case report.

OBJECTIVE: Boucher-Neuhäuser Syndrome (BNS) is a rare autosomal recessive disorder characterized by hypogonadotropic hypogonadism, spinocerebellar ataxia, and chorioretinal syndrome, and associated with a variant in the PNPLA6 gene. Although many reports have mentioned the presence of cognitive impairment, a neuropsychological assessment of a BNS case has never been published. Here, we provide a detailed description of a young adult patient with BNS who has a homozygous pathogenic variant in the PNPLA6 gene. METHOD: A 21-year-old man with progressive ataxia and a history of hypogonadotropic hypogonadism and chorioretinal dystrophy was diagnosed with BNS. A comprehensive cognitive evaluation was performed, requiring the ad hoc selection and adaption of neuropsychological tests to overcome visual and motor impairments that characterize this syndrome. RESULTS: The patient presented an intact global cognitive profile with selective executive dysfunction and mild verbal reasoning dysfunction. In particular, attentional-inhibitory control, working memory, and set switching were impaired, and inadequate development of conceptual knowledge and abstract reasoning was observed. CONCLUSIONS: This is the first report of an explicitly documented comprehensive neuropsychological assessment in a patient with BNS. The battery we composed is an example of a methodology that can be used to conduct a detailed cognitive examination without being penalized for physical impairment.Further studies are needed to define the typical cognitive features that characterize BNS and possibly identify its cognitive phenotype(s).

A Single-Center Experience With Phoenix Atherectomy System in Patients With Moderate to Heavily Calcified Femoropopliteal Lesions.

PURPOSE: To evaluate efficacy and safety of a new rotational atherectomy (RA), the Phoenix Atherectomy™ System, for the treatment of de novo and re-stenotic or occlusions atherosclerotic moderate-heavily lesions of the femoro-popliteal axis. MATERIAL AND METHODS: From January 2015 to August 2017, 52 patients with heavily calcified femoro-popliteal lesions causing severe stenosis or occlusions were enrolled in our center to be treated using Phoenix catheters. Primary endpoints of this study were acute efficacy and safety at 30 days. Secondary endpoints were freedom from restenosis and target lesion revascularization (TLR)/target vessel revascularization (TVR) at 1-, 6- and 12- months. RESULTS: The mean lesion length was 9.2 cm (range 5-23 cm). The lesions were located in superficial femoral artery (SFA) in 61.5% (Fig. 1-A), in popliteal artery in 21.1% and involved femoral-popliteal axis in 15.4%. A primary technical success was achieved in 51/52 patients, with an optimal working channel after RA alone. Using Kaplan-Meyer analysis, primary vessel patency rates at 1, 6 and 12 -months was 96.1%, 86.5% and 76.9% respectively. Assisted primary patency at 1, 6- and 12 -months was 100%, 90.3% and 86.5% respectively. CONCLUSIONS: Recanalisation with the Phoenix Atherectomy System is simple and safe, with a high technical success rate.

Critical limb ischemia: current challenges and future prospects.

Critical limb ischemia (CLI) is considered the most severe pattern of peripheral artery disease. It is defined by the presence of chronic ischemic rest pain, ulceration or gangrene attributable to the occlusion of peripheral arterial vessels. It is associated with a high risk of major amputation, cardiovascular events and death. In this review, we presented a complete overview about physiopathology, diagnosis and holistic management of CLI. Revascularization is the first-line treatment, but several challenging cases are not treatable by conventional techniques. Unconventional techniques for the treatment of complex below-the-knee arterial disease are described. Furthermore, the state-of-the-art on gene and cell therapy for the treatment of no-option patients is reported.

Repeated Transarterial Chemoembolization with Degradable Starch Microspheres (DSMs-TACE) of Unresectable Hepatocellular Carcinoma: A Prospective Pilot Study.

OBJECTIVE: The aims of this study were to: a) evaluate tumor response rates using modified-Response-evaluation-criteria-in-solid-tumors (mRecist) criteria, b) evaluate safety of Degradable Starch Microspheres Trans-arterial-chemo-embolization (DSMs-TACE) for unresectable hepatocellular-carcinoma (HCC) treatment. MATERIALS AND METHODS: We prospectively enrolled 24 HCC cirrhotic patients (21/3 M/F, mean age 66.3 years) to be treated with repeated DSMs-TACE procedures, performed at 4-6 week intervals on the basis of tumor response and patients tolerance. Clinical and biochemical evaluations were performed before and after each procedure. Treatment response was also assessed by Computed-tomography (CT) or Magnetic-resonance-imaging (MRI)-scan 4-6 weeks following each procedure. RESULTS: In our experience, DSMs-TACE was both safe and effective. A total of 53 DSMs-TACE procedures were performed (2.2 per patient). No procedure-related death was observed. Complete Response (CR) was observed in 5/24 (20.8%), 4/17 (23.5%) and 5/12 (41.6%) patients after the first, second and third procedure, respectively. At the end of each treatment, all patients experienced at least a partial response. At the end of the repeated procedures, no differences between mono- or bi-lobar disease were observed in patients with CR (64.2% vs 50%; p=ns). In most cases, treatment discontinuation was due to worsening liver function. CONCLUSION: DSMs-TACE is a valid, well-tolerated alternative treatment to Lipiodol-TACE or DEB-TACE, as it has demonstrated to achieve a relatively high percentage of complete tumor necrosis. CR rates were similar between patients with mono- or bi-lobar disease indicating the possibility of carrying-out repeated procedure in a safe and effective way in both types of patients.

Mechanical thrombectomy of acute ischemic stroke with a new intermediate aspiration catheter: preliminary results.

BACKGROUND AND PURPOSE: To report clinical and procedural outcomes of acute ischemic stroke patients after endovascular treatment with the new thromboaspiration catheter AXS Catalyst 6. METHODS: Patients with anterior and posterior circulation stroke were selected. Successful reperfusion defined as a Thrombolysis in Cerebral Infarction (TICI) score ≥2 b and 3-month functional independence defined as a modified Rankin Scale (mRS) ≤2 were the main efficacy outcomes. Symptomatic intracranial hemorrhage and mortality were the main safety outcomes. RESULTS: 107 patients were suitable for analysis. Mean age was 73.18±12.62 year and median baseline NIHSS was 17 (range: 3-32). The most frequent site of occlusion was the middle cerebral artery (MCA) (60.7%). 76.6% of patients were treated with AXS Catalyst 6 alone without the need for rescue devices or thromboaspiration catheters. Successful reperfusion was achieved in 84.1%, functional independence in 47.6%, symptomatic intracranial hemorrhage occurred in 3.7%, and mortality in 21.4%. CONCLUSIONS: Endovascular treatment with AXS Catalyst 6 proved to be safe, technically feasible, and effective. Comparison analyses with other devices for mechanical thrombectomy are needed.

Endovascular Mechanical Thromboaspiration of Right Hepatic Arterial Thrombosis After Liver Transplantation.

A 56-year-old male Patient presented 27 days after a liver transplantation (LT) with fever and hyperbilirubinemia. He underwent CT examination resulting in a diagnosis of right hepatic artery (HA) occlusion with hepatic bilomas. Once placed a long right femoral 6F introducer at the origin of the HA, a 0.014" guidewire was advanced over the thrombus, in a segmental branch. A 4MAX (Penumbra, Alameda, USA) catheter was advanced and withdrawn under constant aspiration until complete clot removal was achieved. Follow-up CT and D-US assessments at 12 months demonstrated regular HA patency and bilomas reduction. Endovascular thromboaspiration is an effective strategy in cases of E-HAT after LT.

Endovascular treatment of below-the-elbow arteries in critical hand ischemia.

Although uncommon, critical hand ischemia (CHI) represents a cause of significant disability because of its effect on hand function. Percutaneous transluminal angioplasty (PTA) is now considered a first-line therapy for above-the-elbow artery disease while there are few data regarding the treatment of below-the-elbow (BTE) arteries. The aim of this study is to review the current literature on BTE vessel PTA and to describe the technical approach. In case of favorable anatomy (absence of inflow arteries disease, standard humeral artery bifurcation), a 4-F antegrade trans-brachial approach is usually the preferred option. Due to the small vessel size and the similarities to coronary and below-the-knee vessels, we consider the BTE vessel treatment as a domain of 0.014" compatible devices. The intraluminal approach is always the preferred technique of guidewire advancement, because the subintimal space in not favorable in BTE vessels. In case of failure of the antegrade approach, retrograde puncture and the radial-ulnar loop technique can be used. Long, low-profile balloons with a diameter between 1.5 and 3.5 mm represent the standard treatment of BTE vessel disease. The use of other devices such as Rotablator, stenting and drug coated balloons has been suggested in very selected cases. Endovascular treatment of BTE vessels is feasible and effective in patients with CHI, with an elevated immediate technical success and satisfactory clinical results.