心血管领域-心律失常方向

METHODS::This article reports the case of a 40-year-old male patient with isolated metamorphopsia and visual pseudohallucinations lasting for 2 weeks. As the ophthalmological findings were not pathological, a further neurological examination was performed. After diagnosis of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, which can be manifested in various psychiatric, neurological and ophthalmological ways, the oligosymptomatic patient received high-dose corticosteroid treatment with intravenous immunoglobulins, which stopped the symptoms. ZUSAMMENFASSUNG:Wir berichten über einen 40-jährigen Patienten mit seit 2 Wochen anhaltenden isolierten Metamorphopsien und visuellen Pseudohalluzinationen. Bei ophthalmologisch nicht wegweisenden Befunden erfolgte eine weiterführende neurologische Diagnostik. Nach Diagnosestellung einer Anti-N-Methyl-D-Aspartat-(NMDA)Rezeptor-Enzephalitis, welche sich auf unterschiedlichste Weise psychiatrisch, neurologisch sowie ophthalmologisch äußern kann, erhielt der oligosymptomatische Patient eine Hochdosiskortikosteroidtherapie kombiniert mit intravenöser Immunglobulintherapie, worunter die Symptome sistierten.

METHODS::We introduce the first-ever statistical framework for estimating the age of Multiple Sclerosis (MS) lesions from magnetic resonance imaging (MRI). Estimating lesion age is an important step when studying the longitudinal behavior of MS lesions and can be used in applications such as studying the temporal dynamics of chronic active MS lesions. Our lesion age estimation models use first order radiomic features over a lesion derived from conventional T1 (T1w) and T2 weighted (T2w) and fluid attenuated inversion recovery (FLAIR), T1w with gadolinium contrast (T1w+c), and Quantitative Susceptibility Mapping (QSM) MRI sequences as well as demographic information. For this analysis, we have a total of 32 patients with 53 new lesions observed at 244 time points. A one or two step random forest model for lesion age is fit on a training set using a lesion volume cutoff of 15 mm3 or 50 mm3. We explore the performance of nine different modeling scenarios that included various combinations of the MRI sequences and demographic information and a one or two step random forest models, as well as simpler models that only uses the mean radiomic feature from each MRI sequence. The best performing model on a validation set is a model that uses a two-step random forest model on the radiomic features from all of the MRI sequences with demographic information using a lesion volume cutoff of 50 mm3. This model has a mean absolute error of 7.23 months (95% CI: [6.98, 13.43]) and a median absolute error of 5.98 months (95% CI: [5.26, 13.25]) in the validation set. For this model, the predicted age and actual age have a statistically significant association (p-value <0.001) in the validation set.

METHODS::COVID-19 is caused by the novel SARS-CoV-2 and is a potentially fatal disease that is of great global public health concern. In addition to respiratory symptoms, neurological manifestations have been associated with COVID-19. This is attributed to the neurotropic nature of coronaviruses. The authors present a case of Bell's palsy associated with COVID-19 in a term primigravida.

METHODS:ABSTRACT:Although the mutual relationship between ambulation and physical activity (PA) in people with multiple sclerosis (pwMS) has been described in several studies, there is still a lack of detailed information about the way in which specific aspects of the gait cycle are associated with amount and intensity of PA. This study aimed to verify the existence of possible relationships among PA parameters and the spatio-temporal parameters of gait when both are instrumentally assessed.Thirty-one pwMS (17F, 14 M, mean age 52.5, mean Expanded Disability Status Scale (EDSS) score 3.1) were requested to wear a tri-axial accelerometer 24 hours/day for 7 consecutive days and underwent an instrumental gait analysis, performed using an inertial sensor located on the low back, immediately before the PA assessment period. Main spatio-temporal parameters of gait (i.e., gait speed, stride length, cadence and duration of stance, swing, and double support phase) were extracted by processing trunk accelerations. PA was quantified using average number of daily steps and percentage of time spent at different PA intensity, the latter calculated using cut-point sets previously validated for MS. The existence of possible relationships between PA and gait parameters was assessed using Spearman rank correlation coefficient rho.Gait speed and stride length were the parameters with the highest number of significant correlations with PA features. In particular, they were found moderately to largely correlated with number of daily steps (rho 0.62, P< .001), percentage of sedentary activity (rho = -0.44, P < .001) and percentage of moderate-to-vigorous activity (rho = 0.48, P < .001). Small to moderate significant correlations were observed between PA intensity and duration of stance, swing and double support phases.The data obtained suggest that the most relevant determinants associated with higher and more intense levels of PA in free-living conditions are gait speed and stride length. The simultaneous quantitative assessment of gait parameters and PA levels might represent a useful support for physical therapists in tailoring optimized rehabilitative and training interventions.

METHODS::Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by progressive weakness and sensory loss, often affecting patients' ability to walk and perform activities of daily living independently. With the lack of a diagnostic biomarker, the diagnosis relies on clinical suspicion, clinical findings, and the demonstration of demyelinating changes on electrodiagnostic (EDx) testing and nerve pathology. As a result, patients can often be misdiagnosed with CIDP and unnecessarily treated with immunotherapy. Interpreting the EDx testing and cerebrospinal fluid findings in light of the clinical phenotype, recognizing atypical forms of CIDP, and screening for CIDP mimickers are the mainstays of the approach to patients suspected of having CIDP, and are detailed in this review. We also review the currently available treatment options, including intravenous immunoglobulin (IVIg), corticosteroids (CCS), and plasma exchange (PE), and discuss how to approach treatment-refractory cases. Finally, we emphasize the need to adopt objective outcome measures to monitor treatment response.

METHODS:BACKGROUND:Cognitive impairment is recognised as a significant clinical issue in Multiple Sclerosis (MS). It can occur at any stage of the disease, affecting quality of life, occupational activity, and adherence to therapy. This makes the availability of a validated assessment tool for detecting and monitoring cognitive dysfunction in multiple sclerosis essential. The Brief International Cognitive Assessment for Multiple Sclerosis is a practical and simple means of administering a battery of three neuropsychological tests, and does not require any formal neuropsychological training. OBJECTIVE:To establish the validity of BICAMS in the Polish MS population; to assess the correlations of cognitive status with demographic and clinical factors, including affective symptoms and fatigue. METHODS:BICAMS was administered to 61 MS patients and 61 HC subjects. Examination of 20 participants with MS was repeated after one to three weeks to assess test-retest reliability. The patients with MS and HC subjects also completed the Hospital Anxiety and Depression Scale (HADS) and Modified Fatigue Impact Scale (MFIS). RESULTS:The MS group performed worse than the HC group in all three BICAMS components, obtaining the following values respectively: 51.7 and 56.1 (p = 0.02) for CVLT, 25 and 28 (p = 0.03) for BVMT-R, and 48.8 and 57.2 (p < 0.001) for SDMT. All BICAMS tests had very significant correlations in test-retest reliability (r = 0.83, p < 0.001 for CVLT; r = 0.84, p < 0.001 for BVMTR; r = 0.9, p < 0.001 for SDMT). 34% of MS patients presented cognitive dysfunction based on the criterion of one or more test scores below the 5th percentile value of the HC group. Significant anxiety and depressive symptoms were reported by 31.1% and 18.0% of MS patients. 31.1% of PwMS reported significant fatigue. BICAMS test results were not associated with HADS or MFIS scores. CONCLUSIONS:The Polish version of BICAMS is a valid and reliable tool for the assessment of cognitive impairment in patients with MS.

METHODS:OBJECTIVES:To describe the immunotherapy and pharmacologic treatments administered to pediatric patients with N-methyl-D-aspartate receptor encephalitis (NMDARE) during inpatient rehabilitation as well as to examine clinical and demographic variables associated with early functional outcomes. DESIGN:Retrospective chart review and post hoc analysis. SETTING:Pediatric inpatient rehabilitation unit. PARTICIPANTS:Pediatric patients (N=26; mean age, 10.79±5.17y) admitted to an inpatient rehabilitation unit with a confirmed diagnosis of NMDARE. INTERVENTIONS:Inpatient rehabilitation; pharmacologic treatments. MAIN OUTCOME MEASURE:FIM for Children (WeeFIM) Developmental Functional Quotient (DFQ). RESULTS:All patients received first-line immunotherapies to treat NMDARE, and 69% also received second-line immunotherapies. Patients were prescribed an average of 8 medications for symptom management (range, 3-15 per patient), most often for the treatment of agitation (100%), psychiatric symptoms (92%), and seizures (65%). Sixty-five percent of patients demonstrated an improvement in Total WeeFIM DFQ over the course of inpatient rehabilitation, with 35% demonstrating limited to no change in Total WeeFIM DFQ ("unfavorable early outcome"). Those with unfavorable early outcome were significantly younger than those showing more favorable outcome. Pharmacologic treatment for seizures, movement disorders, and decreased arousal or level of consciousness were each associated with unfavorable early outcome independent of age differences. CONCLUSION:Findings highlight the symptomatic heterogeneity and polypharmacy involved in the care and treatment of patients with NMDARE, with patients receiving a variety of immunotherapies and medications for symptom management. The presence of (and treatment for) seizures, movement disorders, and deteriorated neurologic status may each be associated with poor early outcomes in this population. Further investigation is needed to better classify presentations and treatments for this disease and to determine how differences are associated with long-term outcomes.

METHODS:BACKGROUND:Little is known about how different outlier estimation methods affect cutoff limits for outliers in single fiber electromyography. METHODS:We compared in a prospective fashion the established 18th jitter value (18thjv) method to three, whole-distribution based, outlier detection methods: the interquartile range (IQR), the log-normal, and the Z-score method. The reference limits were probed in a normal cohort and in myasthenia gravis (MG) patients. RESULTS:Differences in outlier cutoff values between the different methods were in the range of 2 μs. The number of abnormal muscles according to the computed criteria was similar for all four methods in the control group. Classification metrics (sensitivity, specificity, Youden's statistic, and predictive values) were also similar among the different methods. In the MG group, however, the Z-score method failed to identify the abnormal jitter values. Accordingly, Kappa agreement was substantial to perfect (0.658 to 1) between the three methods (18thjv, IQR, and log-normal), but was equivalent to chance between the three methods and the Z-score in the MG group. CONCLUSIONS:The established 18thjv method proved largely robust when compared to whole-distribution based methods, and its use in clinical practice is justified. Simple estimation of outlier limits by adding two SDs to the mean of the data, leads to unacceptable deviations from the true cutoff values. Moreover, in a clinical scenario in which the final electrodiagnosis depends only on the number of outliers, it is meaningful to accept a tolerance zone of about 2 μs, which is the approximate variation range among the different methods.

METHODS:INTRODUCTION:The majority of patients with myasthenia gravis (MG) initially present with ocular symptoms, but it is difficult to predict which cases will remain as ocular MG (OMG) or will progress to generalized MG. Herein we evaluated the serologic profile of Japanese OMG and its relationship with clinical features. METHODS:Seventy-three patients with OMG from five Japanese myasthenia gravis (MG) centers were enrolled. Live cell-based assays (CBAs) were used to determine the presence of autoantibodies (Abs) to clustered adult (2α, β, δ, ε) and fetal (2α, β, δ, γ) acetylcholine receptor (AChR) isoforms, muscle-specific receptor tyrosine kinase (MuSK), and lipoprotein receptor-related protein-4 (LRP4). RESULTS:Thirty-four of 73 (46.5%) serum samples were positive for Abs against both the adult-type and fetal-type AChR, as expected, but 7 (9.6%) and 2 (2.7%) were positive only for fetal or adult AChR-Abs, respectively. Four (5.4%) samples were positive for MuSK-Abs, but two of these also contained antibodies to fetal AChR or LRP4. Twenty-six (35.6%) samples were seronegative. DISCUSSION:Abs against fetal-specific AChR, MuSK, and LRP4 are found in some patients with OMG. Future studies attempting to predict conversion from ocular symptoms to generalized MG may benefit from measurement of these antibodies.

METHODS::A newly introduced term, "axonal conduction block," brought a confusion in the electrodiagnostic diagnosis of Guillain-Barrè syndrome (GBS). I am proposing the term "nodal conduction block" for "axonal conduction block." This unifying concept of nodal conduction block will accommodate both the traditional concept of demyelination as well as the new concept of nodopathy in the "axonal form of GBS,", making the practice of electrodiagnosis much easier.