Non-ADEM encephalitis in patients with myelin oligodendrocyte glycoprotein antibodies: a systematic review.

BACKGROUND AND PURPOSE: Non-(acute disseminated encephalomyelitis) (non-ADEM) encephalitis and/or fluid attenuated inversion recovery hyperintense lesions in anti-myelin-oligodendrocyte-glycoprotein-associated encephalitis with seizures (FLAMES) are rarely described in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies (Abs). The aim was (i) to describe the clinical features and disease course of children and adults with non-ADEM encephalitis and/or FLAMES associated with MOG Abs and (ii) to describe their association with other central nervous system autoantibodies. METHODS: This was a systematic review following the PRISMA guidelines. Patients fulfilled criteria for non-ADEM encephalitis and/or FLAMES, and all were MOG Ab positive. RESULTS: In total, 83 (79%) patients with non-ADEM encephalitis (48 also had FLAMES) and 22 (21%) with isolated FLAMES were included. At the first episode, children (n = 45) had more infections (11/45, 24.4%; p = 0.017) and more of the phenotype consisting of non-ADEM encephalitis (42/45, 93.3%; p = 0.014) than adults (n = 38). Children had more episodes consistent with working memory deficits (25/54, 46.3%; p = 0.014) but fewer psychiatric symptoms (16/54, 29.6%; p = 0.002). Twenty-eight (40.6%) of 69 patients had N-methyl-d-aspartate receptor (NMDAR) Abs in cerebrospinal fluid (CSF), being more frequent in adults (19/29, 65.5%; p < 0.001). Compared to negatives, positive CSF NMDAR Abs had more relapses (14/20, 70%; p = 0.050), required ventilatory support more frequently (8/34, 23.5%; p = 0.009) and had more psychiatric episodes (28/34, 82%; p < 0.001) or abnormal movements (14/34, 41.2%; p = 0.008). Apart from an older age in FLAMES, positive and negative CSF NMDAR Ab groups shared similar features. CONCLUSION: Non-ADEM encephalitis patients with MOG Abs show specific clinical and radiological features, depending on the age at first episode. The presence of MOG Abs in non-ADEM encephalitis patients should not rule out to test other autoantibodies, especially concomitant NMDAR Abs in patients with suggestive symptoms such as behavioural or movement alterations.

[Spontaneous visits to the emergency service and hepatology day hospital by patients with liver cirrhosis]. / Análisis de las consultas espontáneas de enfermos cirróticos a urgencias y hospital de día de hepatología.

INTRODUCTION: Because of the current overload of emergency services, new units, such as day units, have had to be created. Liver cirrhosis (LC) is a chronic disease with frequent decompensations requiring medical attention. The aim of this study was to compare differences between emergency consultations in a hepatology day hospital (HDH) and in an emergency service (ES) among patients with LC. METHODS AND MATERIAL: We performed an observational prospective study. All patients with LC attending the HDH or ES from September 2007 to August 2008 were asked to complete a questionnaire. Demographic, clinical, and radiological variables were collected. RESULTS: There were 743 consultations, of which 62% involved the HDH. The mean age was 65±12 years, and the male/female ratio was 2:3. The most frequent diagnosis in the ES was hepatic encephalopathy (26.2% ES versus 6% HDH, p<0.001) followed by upper gastrointestinal hemorrhage (17.7% ES versus 0.6% HDH, p<0.001), while the most frequent diagnosis in the HDH was ascites (66.2% HDH versus 22.7% ES, p<0.001). The tests performed were as follows: blood analysis: 95% ES versus 60% HDH (p<0.01); radiology: 71% ES versus 11% HDH (p<0.01) and paracentesis: 51% ES versus 74% HDH (p<0.01). The mean length of stay in the ES was 21.3±121.5 hours compared with 3.3±2.4 hours in the HDH (p<0.001). A total of 53% of patients attended in the ES were hospitalized compared with 12% of those attended in the HDH (p<0.05). CONCLUSION: Patients with LC preferentially attend the HDH, where fewer tests are performed and the length of stay is shorter. The care provided in the HDH is appropriate and efficient.

Image-guided percutaneous ablation for the treatment of lung malignancies: current state of the art.

Image-guided percutaneous lung ablation has proven to be a valid treatment alternative in patients with early-stage non-small cell lung carcinoma or oligometastatic lung disease. Available ablative modalities include radiofrequency ablation, microwave ablation, and cryoablation. Currently, there are no sufficiently representative studies to determine significant differences between the results of these techniques. However, a common feature among them is their excellent tolerance with very few complications. For optimal treatment, radiologists must carefully select the patients to be treated, perform a refined ablative technique, and have a detailed knowledge of the radiological features following lung ablation. Although no randomized studies comparing image-guided percutaneous lung ablation with surgery or stereotactic radiation therapy are available, the current literature demonstrates equivalent survival rates. This review will discuss image-guided percutaneous lung ablation features, including available modalities, approved indications, possible complications, published results, and future applications.

Usefulness of Cranial Ultrasound for Detecting Neonatal Middle Cerebral Artery Stroke.

Magnetic resonance imaging is the gold standard technique in establishing the diagnosis of neonatal arterial ischemic stroke (NAIS). The diagnostic value of cranial ultrasound scanning in this clinical context is controversial. We aimed to assess the current sensitivity of the cranial ultrasound scan (CUS) in detecting NAIS, as this issue has not been well described in the literature. Newborns with NAIS diagnosed by magnetic resonance imaging between 2010 and 2016 were included. All CUSs were blindly analyzed retrospectively by a neonatologist expert in neuroimaging and compared with the findings of non-expert evaluators recorded on medical charts immediately after performing the evaluation. The overall sensitivity of CUS in detecting an imaging finding suggestive of NAIS was 87% (95% confidence interval (CI): 79%-95%) for an expert evaluator, but declined to 72% (61%-83%) when performed by a non-expert evaluator (p 0.002). Sensitivity was 83% and 61% in the first 24 h and 86% and 66% at 24-48 h for expert and non-expert evaluators, respectively (p < 0.05). CUS has higher sensitivity than previously reported in the detection of a NAIS, for both expert and non-expert evaluators. These findings may be explained by the advanced technology of new ultrasound equipment. Expertise in performing CUS is useful, particularly in the first 48 h after clinical debut.

Acute flaccid weakness with myelopathy and peripheral nerve involvement in 2 children: Recent characterization of a previously observed phenomenon.

BACKGROUND: Acute flaccid weakness may be the first presentation of acute transverse myelitis (ATM), an immune-mediated central nervous system disorder or may be the first presentation of anterior horn cell syndrome or peripheral nervous system disease. CASE REPORTS: We describe two previously healthy female infants who presented with acute flaccid paralysis and encephalopathy. Neuroimaging revealed central cord signal changes in both cases and surprisingly electrophysiological studies performed revealed a generalized axonal motor neuropathy as well. CONCLUSION: Clinical, radiological and neurophysiological assessment are important to aid in the diagnosis and subsequent management of children with overlapping inflammatory peripheral and central nervous system syndromes.

Validity of the Spanish version of the Chronic Liver Disease Questionnaire (CLDQ) as a standard outcome for quality of life assessment.

The Chronic Liver Disease Questionnaire (CLDQ) measures the impact on quality of life of chronic liver diseases, regardless of underlying etiology. The aim of this study was to develop a Spanish version of the CLDQ, and to assess its acceptability, reliability, validity, and sensitivity to change. The forward and back-translation method by bilingual translators, with expert panel and pilot testing on patients, was used for the adaptation. The final version was self-administered, together with the Short Form-36 Health Survey (SF-36), on 149 consecutive patients with chronic liver disease. Child-Turcotte-Pugh scores were evaluated by a physician. To assess reproducibility and responsiveness the CLDQ was readministered to a subsample of stable patients and to those who had received a liver transplant. Validity was evaluated via exploratory factor analysis, the CLDQ pattern across severity groups, and correlation coefficients with "itching" and SF-36 scores. Cronbach's alpha and Intraclass Correlation Coefficient for CLDQ global score were 0.93 and 0.90, respectively, demonstrating good reliability. Validity was supported by correlations of the CLDQ with SF-36 and "itching," and CLDQ severity gradient (global score means were 5.5, 5.2, 5.0, and 4.5 in patients with no cirrhosis, cirrhosis Child-Turcotte-Pugh A, B, and C, respectively; P = 0.012). Responsiveness was shown by a high CLDQ improvement in patients who had received liver transplant (mean change = -1.4; P < 0.001). In conclusion, the Spanish CLDQ is reliable, valid, responsive, and equivalent to the original. These findings support its use as a standard outcome for patients with chronic liver diseases within the whole severity range, from "no cirrhosis" to transplant recipients, both in Spanish and international studies.