关键词： Bilateral facial paralysis   Sjogren syndrome   Diagnosis   Treatment  

摘要： Primary Sjogren's syndrome (pSS) is a chronic autoimmune disorder associated with a variety of systemic presentations. Varied neurological dysfunctions of newly diagnosed adult patients with pSS have been observed in recent years. We aimed to describe a rare case of acute bilateral facial paralysis diagnosed with pSS for the first time and review the previous studies including similar cases. A 69-year-old female, who had experienced ocular and oral dryness for more than 10 years, presented with bilateral facial palsy. Her laboratory test results showed positive anti-Ro (SSA) and anti-Ro-52 antibodies. Ophthalmic examination and test of saliva secretion verified xerophthalmia and xerostomia, respectively. Other possibilities of Lyme disease, Mobius syndrome, tumor, bilateral temporal bone fracture, Guillain-Barre syndrome, central nervous system lymphoma and HIV infection were ruled out. A diagnosis of pSS associated with bilateral facial paralysis was made. The literature review revealed one article describing a similar patient. Our case was the only one suffering from acute bilateral facial palsy without other nerve involvement. The presence of such patients reveals that pSS is an underlying cause of acute bilateral facial paralysis.

关键词： Paraneoplastic neurological syndrome   breast cancer   HER2 blockade   opsoclonus-myoclonus syndrome  

摘要： Paraneoplastic neurological syndrome is associated with anti-Ri antibodies, which are typically present with opsoclonus-myoclonus-ataxia. Human epidermal growth factor receptor 2 (HER2) overexpression is present in 15%-25% of breast cancer and is associated with poor prognosis. There are a few reports of paraneoplastic neurological syndrome associated with HER2-positive breast cancer in the literature, of which most are anti-Yo-associated paraneoplastic neurological syndrome. We present herein the case of a female patient with HER2-positive breast cancer who had atypical anti-Ri antibody associated with opsoclonus-myoclonus paraneoplastic neurological syndrome. Following the diagnosis of paraneoplastic syndrome, chemotherapy with dual HER2 blockade and immunomodulating treatment including intravenous immunoglobulin and oral prednisolone were administered. Although the patient was negative for serum anti-Ri antibodies, there was partial clinical improvement and her neurological deficit persisted. To our knowledge, this is the first case report of female patient with HER2-positive breast cancer who had atypical anti-Ri antibody associated with opsoclonus-myoclonus paraneoplastic neurological syndrome and treated with dual HER2 blockade.

关键词： Synthetic images   Quantitative MRI   T1   PD   Epilepsy   Focal cortical dysplasia   Multiple sclerosis (MS)  

摘要： Structural cerebral MRI analysis in patients with neurological diseases usually requires T1-weighted datasets for tissue segmentation. For this purpose, synthetic T1-weighted images which are constructed from quantitative maps of the underlying tissue parameters such as the T1 relaxation time and the proton density (PD) may provide advantages over conventional datasets. However, in some cases synthetic images may suffer from specific artifacts, hampering accurate tissue segmentation. The goal was to improve a previously described method for the calculation of synthetic magnetization-prepared rapid gradient-echo (MP-RAGE) datasets from quantitative T1 and PD maps. Improvements comprise a B0-correction for the water-selective excitation pulses employed in T1-mapping and the use of T1-based pseudo-PD maps. Synthetic T1-weighted MP-RAGE datasets were calculated, using the standard and the improved algorithm, for 10 patients with focal epilepsy (caused by focal cortical dysplasia in 9), 10 patients with multiple sclerosis and 10 healthy control subjects and segmented with the Freesurfer toolbox. Visual inspection disclosed that segmentation of the standard synthetic datasets was inaccurate in 6 out of 10 patients with epilepsy, 7 out of 10 patients with multiple sclerosis and 7 out of 10 healthy control subjects, while the improved synthetic datasets resulted in adequate segmentation outcomes in the majority of cases. Only for one patient with multiple sclerosis and one with epilepsy, segmentation in basal temporal regions was not sufficient. Furthermore, data based on the standard algorithm showed strong signal non-uniformities in basal regions. This effect was not present in the improved synthetic datasets.

关键词： Granular cell tumor   Pituicytoma   Pituitary   Sellar tumors   Spindle cell oncocytoma   TTF-1  

摘要： OBJECTIVE: Granular cell tumors (GCTs), pituicytomas, and spindle cell oncocytomas are rare, nonfunctioning pituitary tumors sharing positive staining of thyroid transcription factor 1. We present our series, the first single-institutional report with long-term surgical follow-up of all 3 tumor types. METHODS: Our institutional pathology database was queried for these 3 pathologic diagnoses. Clinical records were assessed for clinical presentation, preoperative and postoperative endocrine status, tumor location on imaging, surgical characteristics, pathology results, and tumor recurrence. RESULTS: Data were analyzed for 4 patients with GCTs, 4 with pituicytomas, and 3 with spindle cell oncocytomas. The most common symptoms at presentation were vision changes (64%), headache (55%), endocrine abnormalities (55%), and fatigue (46%). GCTs were the only subtype to present exclusively in the infundibulum and the only subtype in our series to be treated with a transcranial trans-sylvian approach to resection (n = 2). In our study, in contrast to other reports, estimated blood loss was less than 300 mL in all patients. Imaging confirmed gross total resection in all 11 cases with no known recurrences at a mean (standard deviation) follow-up of 4.7 (3.7) years. CONCLUSIONS: We present a single-institution series of rare thyroid transcription factor 1-staining posterior pituitary tumors of the sellar region. Key novel findings include gross total resection with no tumor recurrence at nearly 5 years of mean follow-up and no cases of excess or uncontrolled blood loss. Our findings reinforce the observation that GCTs present in the suprasellar space.

关键词： Cardiac arrest   Cerebral ischemia   Cardiopulmonary resuscitation   Clinical trials as topic   Critical care outcomes  

摘要： Background: Neurological status at hospital discharge is routinely used to assess patient outcome after cardiac arrest. However, attribution of impairment to the arrest is valid only if baseline neurological status is known. This study evaluated whether incorporating baseline neurological status improves performance of a widely employed neurological outcome scale for quantifying arrest-attributable morbidity. Methods: Retrospective cohort study of two U.S. hospitals. Neurological function was assessed via Cerebral performance category (CPC), an ordinal five-point scale with 1 indicating sufficient cognition to lead an independent life and 5 representing brain death. Hospitalized adult patients who suffered in-hospital cardiac arrest for which cardiopulmonary resuscitation was attempted between 2011-2015 were included. Patients were identified through a quality improvement registry that captures all inpatient arrests in the two hospitals. Results: Of 486 patients who suffered in-hospital cardiac arrest, 124 (25.5%) had baseline abnormal neurological function (pre-hospitalization CPC>1). Although 54 patients had a normal discharge CPC of 1, 80 patients had no change in CPC from their prior baseline (11.1% vs. 16.5% met criterion for "normal" outcome defined as CPC of 1 vs. change-in-CPC of 0;McNemar p < 01;kappa for agreement: .78, 95%. CI .69-.86). Across several formulations of criteria for "good" neurological outcome, similar discordance existed between conventional definitions considering only discharge CPC and modified definitions that included change-in-CPC from baseline. Conclusions: Incorporating change-in-CPC into criteria for "good" neurological outcome post-arrest yields discordant results from traditional approaches that consider discharge CPC only and increases face validity of reporting arrest-related morbidity.

关键词： Outcome assessment (health care)   walking   rehabilitation   reproducibility of results   gait speed  

摘要： Background: The 10-meter Walking Test (10MWT) is often used to assess people with, e.g., stroke, but often using different procedures. The aims of this study were to translate the 10MWT into Danish, to determine the number of trials needed to achieve performance stability, and to examine the interrater reliability and agreement of the 10MWT in people with neurological disorders. Methods: Translation followed international recommendations, and evaluated in a consecutive sample of 50 people with a neurological disorder. All participants performed 5 timed 10MWT trials (usual speed) with 20-seconds rest intervals between trials, supervised by a physical therapist. A second session was conducted with another physical therapist, separated with a mean (SD) of 2.7 (0.9) hours. The order of raters was randomized and they were blinded to each other's ratings. Repeated measures ANOVA determined performance stability, while ICC1.1, standard error of measurement (SEM), and minimal detectable change (MDC95) determined reproducibility. Results: Participant's improved their 10MWT scores significantly between the first and second trial only. The faster of the 2 trials took a mean of 11.95 (5.40) seconds, and significantly (P < 0.001) faster than the slowest;mean of 12.80 (6.13) seconds. The intraclass correlation coefficient (ICC;95% confidence interval), SEM, and MDC, based on the fastest of 2 trials, were 0.97 (0.95-0.98), 0.06 m/s, and 0.17 m/s, respectively, and with no systematic between rater's bias. Conclusions: We suggest that the faster of 2 timed trials be recorded for the 10MWT in people with neurological disorders, as we found excellent interrater reliability and low measurement error using this score.

关键词： Brain evolution   neocortex   paraflocculi   Reithroparamyinae   Paramyinae   Paleogene  

摘要： Little is known about the early evolution of the brain in rodents. We report on nine new virtual endocasts for one of the most primitive family of rodents, Ischyromyidae, based on five specimens of Pseudotomus and Notoparamys (Paramyinae) and four specimens of Reithroparamys and Rapamys (Reithroparamyinae), dating from the early Eocene to the late middle Eocene of North America (Colorado, Wyoming, Utah and Montana). The virtual endocasts were obtained from high-resolution X-ray micro-computed tomography data. Comparisons with previously described ischyromyid virtual endocasts allow us to make inferences about the ancestral condition of the brain in rodents. Since Reithroparamyinae are suggested to be more closely related to the squirrel-related clade than other Ischyromyidae, comparisons were also made with the oldest virtual endocast for a squirrel, which gave us the opportunity to look at finer neurological changes occurring in the early evolution of squirrels. These new data permit a preliminary assessment of the endocranial diversity in Ischyromyidae. The results do not show evidence for a clear increase in Encephalization Quotient through time for early rodents. Instead, variation among species could be due to ecological factors (e.g., locomotion). Significant expansion in the neocortex and increase in paraflocculi ratios may have occurred in the transition from Ischyromyidae to Sciuridae, as previously hypothesized. Large olfactory bulbs and exposed midbrain are inferred to have been features present in the common ancestor of rodents, while neocortical expansion is reconstructed as having occurred twice independently within Ischyromyidae.

关键词： Endoscopic endonasal surgery   juvenile angiofibroma   neurological morbidity   quality of life   dry eye syndrome  

摘要： Objectives/Hypothesis Although transnasal endoscopic resection (TER) of juvenile angiofibroma (JA) is unquestionably less invasive than traditional external approaches, several endonasal and neurovascular structures are sacrificed during the procedure. The aim of this study was to evaluate long-term neurological morbidity and related quality of life following TER of JA. Study Design Retrospective cohort study. Methods All patients who underwent TER for JA at the Unit of Otorhinolaryngology-Head and Neck Surgery of the University of Brescia from 1994 to 2016 were contacted to know their availability to undergo a battery of tests aimed to assess lacrimal secretion (Schirmer test), impairment of sensitive nerves (electrophysiological threshold test), and impact on quality of life of tearing reduction and sensitivity impairment with the Ocular Surface Disease Index (OSDI) and visual analogue scale (VAS) (0-10), respectively. Results Thirteen patients were included. Mean follow-up was 77 months (range, 19-156 months). The median Schirmer test value was 5.5 mm and 28.5 mm for the treated and untreated sides, respectively (P = .003). Analysis of sensitivity revealed significant impairment only in the hard palate. The mean OSDI score was 6.8 (normal). The mean values of the VAS scores for hard palate, buccal mucosa, gum, and premaxillary skin were 1.7, 1.7, 1.2, and 2.3, respectively. Conclusions TER for JA can result in objective reduction of lacrimal secretion and sensitivity impairment;nevertheless, their impact on quality of life is negligible. The predictable neurological morbidity of TER should be discussed during preoperative counseling. Level of Evidence 4 Laryngoscope, 129:2184-2188, 2019

关键词： Perioperative stroke   Morbidity   Mortality   NIS  

摘要： Perioperative stroke in non-vascular, non-neurological surgery is a potential cause of high levels of in hospital morbidity and mortality. Although, perioperative stroke following non-vascular and non neurological surgery is a relatively infrequent event;high surgical volume results in thousands of patients experiencing neurological deficits. We aim to determine if perioperative stroke is an independent risk factor for 30-day in-hospital morbidity and mortality following common non-vascular non-neurological surgery. This is a retrospective analysis of 4,264,963 surgical procedures identified in the Nationwide Inpatient Sample (NIS) from the years 2000 through 2011. The exposure of interest was stroke within 30 days of total knee arthroscopy, total hip arthroscopy, lung segmentation and resection, appendectomy, hemicolectomy, cholecystectomy, and lysis of peritoneal adhesions. Study outcomes were in-hospital mortality and in-hospital morbidity. Our study found an in-hospital morbidity, in-hospital mortality, and perioperative stroke rate of 5.5%, 0.8%, and 0.2%, respectively. Multivariable analysis revealed perioperative stroke to be a significant independent predictor (p < 0.001) of length of stay exceeding 14 days (OR = 4.55, 95% CI: 4.21-4.91), cardiovascular complications (OR = 1.96, 95% CI: 1.75-2.19), pulmonary complications (OR = 2.07, 95% CI: 1.89-2.27). The impact of perioperative stroke on in-hospital mortality was (OR = 8.53, 95% CI: 7.87-9.25), whereas cardiovascular complications impact on in-hospital mortality was (OR = 8.36, 95% CI = 7.67-9.10, p < 0.001). This study identified perioperative stroke as an independent predictor of 30-day in-hospital morbidity and mortality following non-vascular, non-neurological surgery. (C) 2019 Elsevier Ltd. All rights reserved.