OBJECTIVES: To determine in-hospital mortality differences in individuals with dementia and acute myocardial infarction (AMI) when using invasive coronary procedures. DESIGN: Retrospective cohort study. SETTING: 2009 Nationwide Inpatient Sample. PARTICIPANTS: Individuals admitted with a primary diagnosis of AMI (N = 631,734) to 1,045 hospitals in 44 states during 2009. MEASUREMENTS: Dementia status and procedural use of diagnostic catheterization, percutaneous intervention (PCI), and coronary artery bypass grafts (CABG) as indicated by International Classification of Diseases, Ninth Revision, codes. The primary outcome was in-hospital mortality. Using multivariable analysis adjusted for covariates, associations were made between coronary procedural use in individuals with dementia and in-hospital mortality. Additional multivariable analysis identified the association between utilization of coronary procedures and in-hospital mortality in AMI patients with dementia. RESULTS: Dementia diagnosis (n = 15,335) was associated with greater likelihood of in-hospital mortality (odds ratio (OR) = 1.22, 95% confidence interval (CI) = 1.15–1.29, P < .001) and less use of diagnostic catheterization (OR = 0.37, 95% CI = 0.35–0.40, P < .001), PCI (OR = 0.37, 95% CI = 0.35–0.40, P < .001), and CABG (OR = 0.19, 95% CI = 0.16–0.22, P < .001). There was less likelihood of in-hospital mortality in participants with dementia who received diagnostic catheterization (OR = 0.36, 95% CI = 0.16–0.78, P < .001), PCI (OR = 0.57, 95% CI = 0.47–0.70, P < .001), or CABG (OR = 0.22, 95% CI = 0.08–0.56, P < .001) than in those not receiving respective interventions. CONCLUSION: Dementia is a significant predictor of in-hospital mortality for hospitalized individuals with AMI and is associated with less use of invasive coronary procedures. Beyond differing care patterns for individuals with AMI and dementia, these results indicate that individuals with dementia are at substantially greater risk for inhospital mortality when they do not receive procedural interventions.
In-office recurrent laryngeal nerve conduction studies (NCSs) are a technique that can potentially provide information about laryngeal innervation. NCS is essential in the management of other neuropathies including carpal tunnel syndrome and spinal cord injury. We hypothesize that laryngeal NCS may have similar utility in managing patients with vocal fold paralysis, atrophy, and neurodegenerative disease. NCSs are technically challenging because they require transcervical stimulation of the recurrent laryngeal nerve (RLN). This study combines radiographic data with cadaveric dissection to describe the anatomic parameters for optimal RLN stimulation.Study Design: Radiographic and Cadaveric Study. Methods: Fifty computed tomography scans were reviewed to determine the dimensions for ideal needle electrode placement. These values were compared to measurements from 12 fresh human cadaveric neck dissections. Ultrasound imaging was utilized in select cases. The neck was dissected to assess the accuracy of electrode placement.Results: Radiographically, the mean transcervical depth to the RLN was 33.2 mm AE 8.3 mm in males versus 29.4 mm AE 9.4 mm in females. The working space between the lateral trachea and carotid artery was 15.3 mm AE 3.6 mm on the right and 14.1 mm AE 2.9 mm on the left. After placement of stimulating electrodes into the cadaveric neck, the electrode tips were consistently within 8 mm of the RLN. Ultrasound guidance improved placement accuracy of the stimulating electrode.Conclusions: Laryngeal NCSs can provide detailed and objective information about laryngeal innervation that could dramatically improve the management of various neuropathies. In-office NCSs require technical precision, and this study describes anatomic factors that may affect the feasibility of performing this technique.
POEMS is a rare syndrome characterized by the unique constellation of polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes. Correct diagnosis is often delayed in early stages of the syndrome when patients exhibit only isolated polyneuropathy due to the clinical and electrodiagnostic similarities with chronic inflammatory demyelinating polyneuropathy. We describe a case in which early suspicion for POEMS uncovered underlying malignancy, and we review the clinical, electrophysiological, pathological, and laboratory findings characteristic of POEMS. The importance of high clinical suspicion is key in the proper diagnosis and management of this complex syndrome.
Platform trials allow efficient evaluation of multiple interventions for a specific disease. The HEALEY ALS Platform Trial is testing multiple investigational products in parallel and sequentially in persons with amyotrophic lateral sclerosis (ALS) with the goal of rapidly identifying novel treatments to slow disease progression. Platform trials have considerable operational and statistical efficiencies compared with typical randomized controlled trials due to their use of shared infrastructure and shared control data. We describe the statistical approaches required to achieve the objectives of a platform trial in the context of ALS. This includes following regulatory guidance for the disease area of interest and accounting for potential differences in outcomes of participants within the shared control (potentially due to differences in time of randomization, mode of administration, and eligibility criteria). Within the HEALEY ALS Platform Trial, the complex statistical objectives are met using a Bayesian shared parameter analysis of function and survival. This analysis serves to provide a common integrated estimate of treatment benefit, overall slowing in disease progression, as measured by function and survival while accounting for potential differences in the shared control group using Bayesian hierarchical modeling. Clinical trial simulation is used to provide a better understanding of this novel analysis method and complex design. ANN NEUROL 2023
I ntroduction: Multifocal motor neuropathy (MMN) is a rare, treatable, immune-mediated neuropathy often associated with multifocal conduction block (CB). The hallmark electrodiagnostic feature is the presence of CB occurring at non-entrapment sites. However, MMN without CB has also been described and can be diagnosed, even in the absence of CB. Therefore, it is crucial to diagnose and identify MMN cases without CB, as it is a treatable disorder. Case presentation: We present a case with progressive symptoms of asymmetric distal upper and lower extremity weakness with no sensory deficits. Intravenous immunoglobulin (IVIG) therapy was initiated, as the patient fulfilled the criteria for probable MMN, despite the absence of CB. The patient's symptoms demonstrated a relative plateau phase in response to IVIG. Although the patient lost follow-up visits, repeated electrodiagnostic study, conducted 11 years after initial presentation, revealed new CB in nerve segments that previously did not show any evidence of CB. Conclusion: This case emphasizes the importance of early diagnosis and respectively initiating early IVIG treatment in MMN, in order to maintain the clinical function. Underdiagnosis of clinically suspected MMN, based on absence of CB, will result in denial of treatment to potential IVIG responders. Keywords Multifocal motor neuropathy, conduction block, intravenous immunoglobulinDisclosure: Said R Beydoun has received grant research support from Alexion, Argenx, Daiichi Pharma, and Pfizer. He has served as an advisory board member for Grifols, MT Pharma and Sarepta, and is a speaker for Grifols. Leila Darki has nothing to disclose in relation to this article.Compliance with Ethics: All procedures were followed in accordance with the responsible committee on human experimentation and with the Helsinki Declaration of 1975 and subsequent revisions. Written informed consent was not obtained from the patient for publication of this case report due to loss of follow-up; no identifying information or images were used in the publication of this paper.Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any non-commercial use, distribution, adaptation and reproduction provided the original author(s) and source are given appropriate credit. 1,2 Presence of multifocal motor conduction block (CB) at locations other than common entrapment sites, with normal sensory nerve conduction in the corresponding segments, is the hallmark of MMN, but some patients with typical MMN have no detectable CB. 2-4Although, the pathophysiology underlying CB and other nerve dysfunction in MMN is not exactly clear, the node of Ranvier and its surrounding structures likely play an important role in MMN. [5][6][7] In addition, clinical response to immune modulation therapy and elevated titers of anti-ganglioside antibodies, such as immunoglobulin M (IgM) anti-GM1 antibodies in 30-88% of patients with MMN, support an immune-mediated etiology. [8][9][10] The absence of CB in some pa...
Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is a rare autosomal recessive disease that manifests with multiorgan presentation characterized by gastrointestinal, extraocular, and both peripheral and central nervous system involvement. MNGIE is caused by mutation in the TYMP (thymidine phosphorylase) gene, resulting in loss of thymidine phosphorylase enzyme activity. This causes its substrates, thymidine and deoxyuridine, to accumulate in tissues and plasma, while also causing secondary alterations in mitochondrial DNA. To date, more than 80 mutations have been reported in this gene. We present herein the clinical, neuroimaging, electrodiagnostic, and molecular findings of a patient with MNGIE caused by a novel homozygous missense mutation (C1175T > G) of the TYMP gene.
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