-
Mashup Score: 18N-of-1 Trial in Epilepsy With Fixation-Off Sensitivity - 6 month(s) ago
Background and Objectives To evaluate the use of transparent goggles in the prevention of eye closure and subsequent seizures due to fixation-off sensitivity (FOS). Methods An N-of-1 trial was conducted during which 7 consecutive cycles of showering with and without goggles were evaluated. Results We found 100% provocation of eyelid myoclonia during each cycle without goggle usage on daily review of smartphone videos with seizure freedom during each cycle with goggles. The semiology on smartphone videos was identical to absences with eyelid myoclonia confirmed by inpatient video-EEG monitoring. At the 6-month follow-up, subjective quality of life improved with no shower-related seizures, greater independence while bathing, and reduction of anticipatory anxiety. Discussion Transparent swimming goggles were effective in this N-of-1 trial to render a patient with drug-resistant epilepsy seizure-free and avert ongoing injury due to FOS. Behavioral modification is a critical adjunct to medi
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 59Kelch-like Protein-11 Encephalitis Associated With Squamous Cell Carcinoma of Unknown Origin - 6 month(s) ago
Objectives We report a case of Kelch-like protein-11 (KLHL11) encephalitis associated with squamous cell carcinoma (SCC) of unknown origin in the neck. Methods A 70-year-old man, diagnosed with prurigo nodularis 4 months prior, presented with subacute-onset progressive dysarthria, imbalance, and diplopia. Neurologic examination revealed horizontal and downbeating nystagmus with lateral gaze, dysarthria, dysmetria, and hearing difficulties. Skin examination showed localized pruritus with excoriated ulcerations on the neck and proximal upper extremities. MRI of the brain revealed T2 hyperintensities in the pons and left cerebellum, and CT of the neck showed a right neck mass. Results Extensive workup for acquired brainstem syndromes revealed KLHL11 antibodies in both the serum and CSF, together with superimposed unclassified antibodies. The patient was diagnosed with KLHL11 encephalitis and was treated with intravenous immunoglobulin, intravenous methylprednisolone, and plasmapheresis. H
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 8Measurement Properties of 2 Novel PROs, the Pompe Disease Symptom Scale and Pompe Disease Impact Scale, in the COMET Study - 6 month(s) ago
Background and Objectives The Pompe Disease Symptom Scale (PDSS) and Impact Scale (PDIS) were created to measure the severity of symptoms and functional limitations experienced by patients with late-onset Pompe disease (LOPD). The objectives of this analysis were to establish a scoring algorithm and to examine the reliability, validity, and responsiveness of the measures using data from the COMET clinical trial. Methods The COMET trial was a randomized, double-blind study comparing the efficacy and safety of avalglucosidase alfa and alglucosidase alfa in patients with LOPD aged 16–78 years at baseline. Adult participants (18 years or older) completed the PDSS and PDIS daily for 14 days at baseline and for 2 weeks before quarterly clinic visits for 1 year after randomization using an electronic diary. Data were pooled across treatment groups for the current analyses. Factor analysis and inter-item correlations were used to derive a scoring algorithm. Test-retest and internal consistency
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 6Dispatcher Stroke/TIA Recognition in Emergency Medical Call Center and Out-of-Hours Service Calls in Copenhagen, Denmark - 6 month(s) ago
Background and Objectives Recognition of stroke/TIA symptoms by emergency medical services (EMS) is instrumental in providing timely recanalization treatments. We assessed the recognition of stroke/TIA by EMS via the emergency medical call center (EMCC) dispatchers and out-of-hours health service (OOHS) dispatchers. Methods In a registry study, based on 2015–2020 data from the Copenhagen EMS, we calculated sensitivity, positive predictive value (PPV), specificity, and negative predictive value (NPV) of dispatcher suspicion of stroke or transient ischemic attack (TIA) and compared against discharge diagnosis. Results We included 462,029 contacts to EMCC and 2,573,865 contacts to OOHS. In total, 19,798 contacts had a stroke or TIA diagnosis at hospital discharge. Sensitivity was 0.64 for EMCC dispatchers and 0.25 for OOHS. PPV was 0.28 for EMCC and 0.22 for OOHS; specificity was 0.96 for EMCC and >0.99 for OOHS, and NPV was 0.99 for EMCC and >0.99 for OOHS. Sensitivity improved over the
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 5Friedreich's Ataxia-Health Index - 6 month(s) ago
Background and Objectives To develop a valid, disease-specific, patient-reported outcome (PRO) measure for adolescents and adults with Friedreich ataxia (FA) for use in therapeutic trials. Methods We conducted semistructured qualitative interviews and a national cross-sectional study of individuals with FA to determine the most prevalent and burdensome symptoms and symptomatic themes to this population. These symptoms and symptomatic themes were included as questions in the first version of the Friedreich’s Ataxia-Health Index (FA-HI). We subsequently used factor analysis, beta interviews with 17 individuals with FA, and test-retest reliability assessments with 20 individuals with FA to evaluate, refine, and optimize the FA-HI. Finally, we determined the capability of the FA-HI to differentiate between subgroups of FA participants with varying levels of disease severity. Results Participants with FA identified 18 symptomatic themes of importance to be included as subscales in the FA-HI
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 10No Increase in Symptoms Toward the End of the Ocrelizumab Infusion Cycle in Patients With Multiple Sclerosis - 6 month(s) ago
Background and Objectives Some patients with multiple sclerosis (MS) receiving ocrelizumab (OCR) report worsening symptoms toward the end of the 6-month infusion cycle (‘wearing off’). The objective of our study was to comprehensively assess changes in symptom burden across 2 consecutive OCR infusion cycles. Methods SYMptom Burden on Ocrelizumab, a Longitudinal Study (SymBOLS; [NCT04855617][1]) was an investigator-initiated, 2-center study of patients with MS starting or receiving OCR. Patients’ symptoms were assessed with NeuroQoL short forms, SymptoMScreen, and Work Productivity and Activity Impairment Questionnaire at the start-cycle, mid-cycle, and end-cycle time points in each of the 2 infusion cycles. Symptom scores at the 3 time points within each cycle were compared with repeated-measures ANOVA or the Friedman rank-sum test for non-normal variables. The proportions of patients with a meaningful symptomatic change from the start to the end of each infusion cycle were calculated,
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 148Differential Diagnosis of Tumor-like Brain Lesions - 6 month(s) ago
Purpose of Review Tumor-like brain lesions are rare and commonly suggest a neoplastic etiology. Failure to rapidly identify non-neoplastic causes can lead to increased morbidity and mortality. In this review, we describe 10 patients who presented with atypical, non-neoplastic tumor-like brain lesions in which brain biopsy was essential for a correct diagnosis and treatment. Recent Findings There has been increasing recognition of autoimmune conditions affecting the nervous system, and many of those diseases can cause tumor-like brain lesions. Currently available reports of non-neoplastic tumor-like brain lesions are scarce. Most case series focus on tumefactive demyelinating lesions, and a comprehensive review including other neuroimmunological conditions such as CNS vasculitis, neurosarcoidosis, histiocytic and infectious etiologies is lacking. Summary We review the literature on tumor-like brain lesions intending to increase the awareness and differential diagnosis of non-neoplastic
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 7Value-Based Care and Neurology - 6 month(s) ago
There is a growing interest noted nationally in moving from fee for service to value-based care. Health care systems are increasingly involved in various payer contracts focused on reducing the total cost of care and are forming clinically integrated networks to be more efficient with care delivery. Although these trends affect primary care significantly, the specialty services are also learning to navigate and understand the roles they will need to play in these new care models. With this “value-based care” digital collection, we hope to provide insight into the current landscape while also sharing ideas to think innovatively as to what the next iteration should be for neurology. First, some background on several concepts is necessary for neurologists as they think about their role as a stakeholder in the care continuum for chronic neurologic diseases.
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 13Abnormal Neurologic Findings in Patients With Sickle Cell Disease Without a History of Major Neurologic Events - 6 month(s) ago
Background and Objectives Patients with sickle cell disease (SCD) are prone to symptomatic neurologic complications. Previous studies reported accrual of neural injury starting at early age, even without having symptomatic neurologic events. The aim of this study was to assess the prevalence and risk factors of abnormal neurologic findings in patients with SCD with no history of major symptomatic neurologic events. Methods Our study extracted patients diagnosed with SCD from the Cooperative Study of Sickle Cell Disease. Patients who underwent a neurologic evaluation were included in our analysis. Patients with previous documented major symptomatic neurologic events were excluded. We compared patients with SCD with abnormal neurologic findings with those without in terms of clinical and laboratory parameters using multivariate binary logistic regression. Results A total of 3,573 patients with SCD were included (median age = 11 [IQR = 19] years, male = 1719 [48.1%]). 519 (14.5%) patients
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
-
Mashup Score: 68Zonisamide - 6 month(s) ago
Purpose of Review Zonisamide (ZNS) was first approved in the United States in 2000 for the adjunctive treatment of patients aged 16 years or older with partial (focal) seizures. Although ZNS has been proven to treat multiple seizure types, it has been largely underutilized in US clinical practice. Recent Findings Published literature demonstrated that antiseizure medications (ASMs) acting on Na+ and Ca2+ channels may add beneficial effects in many seizure types by reducing seizure frequency and leading to overall improvements. In addition, effects of ZNS may lead to clinical improvements in Parkinson disease, alcohol and sleep disorders, pain, and migraine. ZNS is available in multiple formulations and is a safe and effective, broad spectrum ASM. Summary The purpose of this review was to provide an update to what is known about the efficacy of ZNS and where it shows benefits in the treatment of patients with epilepsy and other CNS disorders through its many unique mechanisms of action.
Source: cp.neurology.orgCategories: General Medicine News, NeurologyTweet
In this N-of-1 trial, preventing eye closure in a patient with fixation-off sensitivity with the use of transparent goggles during showering led to a reduction of breakthrough #seizures and improved quality of life. Learn more: https://t.co/ync5SFvfTc #NeuroTwitter https://t.co/rZE33eIghR