Magnetic Resonance Imaging Characteristics of LGI1-Antibody and CASPR2-Antibody Encephalitis.

Importance: Rapid and accurate diagnosis of autoimmune encephalitis encourages prompt initiation of immunotherapy toward improved patient outcomes. However, clinical features alone may not sufficiently narrow the differential diagnosis, and awaiting autoantibody results can delay immunotherapy. Objective: To identify simple magnetic resonance imaging (MRI) characteristics that accurately distinguish 2 common forms of autoimmune encephalitis, LGI1- and CASPR2-antibody encephalitis (LGI1/CASPR2-Ab-E), from 2 major differential diagnoses, viral encephalitis (VE) and Creutzfeldt-Jakob disease (CJD). Design, Setting, and Participants: This cross-sectional study involved a retrospective, blinded analysis of the first available brain MRIs (taken 2000-2022) from 192 patients at Oxford University Hospitals in the UK and Mayo Clinic in the US. These patients had LGI1/CASPR2-Ab-E, VE, or CJD as evaluated by 2 neuroradiologists (discovery cohort; n = 87); findings were validated in an independent cohort by 3 neurologists (n = 105). Groups were statistically compared with contingency tables. Data were analyzed in 2023. Main Outcomes and Measures: MRI findings including T2 or fluid-attenuated inversion recovery (FLAIR) hyperintensities, swelling or volume loss, presence of gadolinium contrast enhancement, and diffusion-weighted imaging changes. Correlations with clinical features. Results: Among 192 participants with MRIs reviewed, 71 were female (37%) and 121 were male (63%); the median age was 66 years (range, 19-92 years). By comparison with VE and CJD, in LGI1/CASPR2-Ab-E, T2 and/or FLAIR hyperintensities were less likely to extend outside the temporal lobe (3/42 patients [7%] vs 17/18 patients [94%] with VE; P < .001, and 3/4 patients [75%] with CJD; P = .005), less frequently exhibited swelling (12/55 [22%] with LGI1/CASPR2-Ab-E vs 13/22 [59%] with VE; P = .003), and showed no diffusion restriction (0 patients vs 16/22 [73%] with VE and 8/10 [80%] with CJD; both P < .001) and rare contrast enhancement (1/20 [5%] vs 7/17 [41%] with VE; P = .01). These findings were validated in an independent cohort and generated an area under the curve of 0.97, sensitivity of 90%, and specificity of 95% among cases with T2/FLAIR hyperintensity in the hippocampus and/or amygdala. Conclusions and Relevance: In this study, T2 and/or FLAIR hyperintensities confined to the temporal lobes, without diffusion restriction or contrast enhancement, robustly distinguished LGI1/CASPR2-Ab-E from key differential diagnoses. These observations should assist clinical decision-making toward expediting immunotherapy. Their generalizability to other forms of autoimmune encephalitis and VE should be examined in future studies.

Fine needle aspiration of human lymph nodes reveals cell populations and soluble interactors pivotal to immunological priming.

Lymph node (LN) fine needle aspiration (LN FNA) represents a powerful technique for minimally invasive sampling of human LNs in vivo and has been used effectively to directly study aspects of the human germinal center response. However, systematic deep phenotyping of the cellular populations and cell-free proteins recovered by LN FNA has not been performed. Thus, we studied human cervical LN FNAs as a proof-of-concept and used single-cell RNA-sequencing and proteomic analysis to benchmark this compartment, define the purity of LN FNA material, and facilitate future studies in this immunologically pivotal environment. Our data provide evidence that LN FNAs contain bone-fide LN-resident innate immune populations, with minimal contamination of blood material. Examination of these populations reveals unique biology not predictable from equivalent blood-derived populations. LN FNA supernatants represent a specific source of lymph- and lymph node-derived proteins, and can, aided by transcriptomics, identify likely receptor-ligand interactions. This represents the first description of the types and abundance of immune cell populations and cell-free proteins that can be efficiently studied by LN FNA. These findings are of broad utility for understanding LN physiology in health and disease, including infectious or autoimmune perturbations, and in the case of cervical nodes, neuroscience.

Automated detection of cerebral microbleeds on MR images using knowledge distillation framework.

Introduction: Cerebral microbleeds (CMBs) are associated with white matter damage, and various neurodegenerative and cerebrovascular diseases. CMBs occur as small, circular hypointense lesions on T2*-weighted gradient recalled echo (GRE) and susceptibility-weighted imaging (SWI) images, and hyperintense on quantitative susceptibility mapping (QSM) images due to their paramagnetic nature. Accurate automated detection of CMBs would help to determine quantitative imaging biomarkers (e.g., CMB count) on large datasets. In this work, we propose a fully automated, deep learning-based, 3-step algorithm, using structural and anatomical properties of CMBs from any single input image modality (e.g., GRE/SWI/QSM) for their accurate detections. Methods: In our method, the first step consists of an initial candidate detection step that detects CMBs with high sensitivity. In the second step, candidate discrimination step is performed using a knowledge distillation framework, with a multi-tasking teacher network that guides the student network to classify CMB and non-CMB instances in an offline manner. Finally, a morphological clean-up step further reduces false positives using anatomical constraints. We used four datasets consisting of different modalities specified above, acquired using various protocols and with a variety of pathological and demographic characteristics. Results: On cross-validation within datasets, our method achieved a cluster-wise true positive rate (TPR) of over 90% with an average of <2 false positives per subject. The knowledge distillation framework improves the cluster-wise TPR of the student model by 15%. Our method is flexible in terms of the input modality and provides comparable cluster-wise TPR and better cluster-wise precision compared to existing state-of-the-art methods. When evaluating across different datasets, our method showed good generalizability with a cluster-wise TPR >80 % with different modalities. The python implementation of the proposed method is openly available.

Oxygen-enhanced MRI and radiotherapy in patients with oropharyngeal squamous cell carcinoma.

Background and purpose: This study aimed to assess the role of T1 mapping and oxygen-enhanced MRI in patients undergoing radical dose radiotherapy for HPV positive oropharyngeal cancer, which has not yet been examined in an OE-MRI study. Materials and methods: Variable Flip Angle T1 maps were acquired on a 3T MRI scanner while patients (n = 12) breathed air and/or 100 % oxygen, before and after fraction 10 of the planned 30 fractions of chemoradiotherapy ('visit 1' and 'visit 2', respectively). The analysis aimed to assess to what extent (1) native R1 relates to patient outcome; (2) OE-MRI response relates to patient outcome; (3) changes in mean R1 before and after radiotherapy related to clinical outcome in patients with oropharyngeal squamous cell carcinoma. Results: Due to the radiotherapy being largely successful, the sample sizes of non-responder groups were small, and therefore it was not possible to properly assess the predictive nature of OE-MRI. The tumour R1 increased in some patients while decreasing in others, in a pattern that was overall consistent with the underlying OE-MRI theory and previously reported tumour OE-MRI responses. In addition, we discuss some practical challenges faced when integrating this technique into a clinical trial, with the aim that sharing this is helpful to researchers planning to use OE-MRI in future clinical studies. Conclusion: Altogether, these results suggest that further clinical OE-MRI studies to assess hypoxia and radiotherapy response are worth pursuing, and that there is important work to be done to improve the robustness of the OE-MRI technique in human applications in order for it to be useful as a widespread clinical technique.

Thrombus Distribution in Vaccine-induced Immune Thrombotic Thrombocytopenia after ChAdOx1 nCov-19 Vaccination.

Vaccination strategies have been at the forefront of controlling the COVID-19 pandemic. An association between vaccine-induced immune thrombotic thrombocytopenia (VITT) and one of these vaccines, the ChAdOx1 nCov-19 vaccine, is now recognized. The purpose of this study was to investigate the frequency and location of thrombosis in each vascular system using CT, MRI, and US to identify additional sites of thrombus in a United Kingdom-wide sample of patients with confirmed VITT. Thirty-two radiology centers identified through the national collaborative Radiology Academic Network for Trainees were invited from the United Kingdom; seven of these contributed to this study. All patients with confirmed VITT ¬between February 3 and May 12, 2021, who met the inclusion criteria were included. The location and extent of thrombi were evaluated using CT, MRI, and US. A total of 40 patients (median age, 41 years [IQR, 32-52]; 22 [55%] men) with confirmed vaccine-induced immune thrombotic thrombocytopenia after administration of their first ChAdOx1 nCov-19 vaccine were included. Thirty-two patients (80%) developed symptoms within the first 14 days, and eight (20%) developed symptoms within 14-28 days. Twenty-nine patients (72%) experienced neurologic symptoms and were confirmed to have cerebral venous sinus thrombosis, 12 (30%) had clinical deterioration and repeat imaging demonstrated extension of their primary thrombus, and eight (20%) died. Twenty-five of 30 patients (83%) who underwent additional imaging had occult thrombosis. In conclusion, patients with VITT are likely to have multiple sites of thrombosis, with the most frequent being cerebral venous sinus thrombosis in combination with pulmonary embolism and portomesenteric venous thrombosis. Whole-body imaging with contrast-enhanced CT can be used to identify occult thrombosis.

Using Variable Flip Angle (VFA) and Modified Look-Locker Inversion Recovery (MOLLI) T1 mapping in clinical OE-MRI.

BACKGROUND AND PURPOSE: The imaging technique known as Oxygen-Enhanced MRI is under development as a noninvasive technique for imaging hypoxia in tumours and pulmonary diseases. While promising results have been shown in preclinical experiments, clinical studies have mentioned experiencing difficulties with patient motion, image registration, and the limitations of single-slice images compared to 3D volumes. As clinical studies begin to assess feasibility of using OE-MRI in patients, it is important for researchers to communicate about the practical challenges experienced when using OE-MRI on patients to help the technique advance. MATERIALS AND METHODS: We report on our experience with using two types of T1 mapping (MOLLI and VFA) for a recently completed OE-MRI clinical study on oropharyngeal squamous cell carcinoma. RESULTS: We report: (1) the artefacts and practical difficulties encountered in this study; (2) the difference in estimated T1 from each method used - the VFA T1 estimation was higher than the MOLLI estimation by 27% on average; (3) the standard deviation within the tumour ROIs - there was no significant difference in the standard deviation seen within the tumour ROIs from the VFA versus MOLLI; and (4) the OE-MRI response collected from either method. Lastly, we collated the MRI acquisition details from over 45 relevant manuscripts as a convenient reference for researchers planning future studies. CONCLUSION: We have reported our practical experience from an OE-MRI clinical study, with the aim that sharing this is helpful to researchers planning future studies. In this study, VFA was a more useful technique for using OE-MRI in tumours than MOLLI T1 mapping.

Hypothesis: Modulation of microglial phenotype in Alzheimer's disease drives neurodegeneration.

The pathophysiology of Alzheimer's disease (AD) remains to be elucidated. The amyloid hypothesis holds explanatory power but has limitations. This article suggests that amyloid deposition and increased permeability of the blood-brain barrier are independent early events in the disease process, which together fashion a distinct microglial activation phenotype. Downstream events including, phagocytosis of synapses and persistent glutamate signaling through N-methyl-D-aspartate receptors drive neurodegeneration and tau pathology. This hypothesis draws on several strands of evidence and aims to illuminate several of the unexplained temporal and spatial features of AD.

Machine Learning for Health: Algorithm Auditing & Quality Control.

Developers proposing new machine learning for health (ML4H) tools often pledge to match or even surpass the performance of existing tools, yet the reality is usually more complicated. Reliable deployment of ML4H to the real world is challenging as examples from diabetic retinopathy or Covid-19 screening show. We envision an integrated framework of algorithm auditing and quality control that provides a path towards the effective and reliable application of ML systems in healthcare. In this editorial, we give a summary of ongoing work towards that vision and announce a call for participation to the special issue  Machine Learning for Health: Algorithm Auditing & Quality Control in this journal to advance the practice of ML4H auditing.

Incidental findings in a referral pathway for non-specific cancer symptoms.

RATIONALE AND OBJECTIVES: To determine the prevalence of incidental findings and define the rate and outcomes of further investigations in a referral pathway for patients with non-specific cancer symptoms. MATERIAL AND METHODS: Incidental findings in each category of a structured report were documented and details of subsequent investigations were obtained from the local PACS and Electronic Patient Record system. RESULTS: 1034 patients were included. Of these, 11% were diagnosed with cancer. The majority of patients (95%) had at least one additional finding reported. The majority of these were minor, but in 140 cases (14%), additional findings were considered to be of clinical significance. Further investigations were performed in 330 patients who did not receive a diagnosis of cancer (32%). In 301 patients (29%), further investigations were normal or non-significant. In 75 cases (7%), ongoing surveillance was required. Specialist referral was recommended for 102 patients (10%) who did not receive a diagnosis of cancer. CONCLUSIONS: In this cohort, there was a high rate of non-significant incidental findings and normal further investigations. However, these risks are likely to be outweighed by the high number of cancer diagnoses and significant non-cancer findings.

Diagnostic accuracy of initial chest radiograph compared to SARS-CoV-2 PCR in patients with suspected COVID-19.

OBJECTIVE: The chest radiograph (CXR) is the predominant imaging investigation being used to triage patients prior to either performing a SARS-CoV-2 polymerase chain reaction (PCR) test or a diagnostic CT scan, but there are limited studies that assess the diagnostic accuracy of CXRs in COVID-19.To determine the accuracy of CXR diagnosis of COVID-19 compared with PCR in patients presenting with a clinical suspicion of COVID-19. METHODS AND MATERIALS: The CXR reports of 569 consecutive patients with a clinical suspicion of COVID-19 were reviewed, blinded to the PCR result and classified into the following categories: normal, indeterminate for COVID-19, classic/probable COVID-19, non-COVID-19 pathology, and not specified. Severity reporting and reporter expertise were documented. The subset of this cohort that had CXR and PCR within 3 days of each other were included for further analysis for diagnostic accuracy. RESULTS: Classic/probable COVID-19 was reported in 29% (166/569) of the initial cohort. 67% (382/569) had PCR tests. 344 patients had CXR and PCR within 3 days of each other. Compared to PCR as the reference test, initial CXR had a 61% sensitivity and 76% specificity in the diagnosis of COVID-19. CONCLUSION: Initial CXR is useful as a triage tool with a sensitivity of 61% and specificity of 76% in the diagnosis of COVID-19 in a hospital setting. ADVANCES IN KNOWLEDGE: .Diagnostic accuracy does not differ significantly between specialist thoracic radiologists and general radiologists including trainees following training.There was a 40% prevalence of PCR positive disease in the cohort of patients (n = 344) having CXR and PCR within 3 days of each other.Classic/probable COVID-19 was reported in 29% of total cohort of patients presenting with clinical suspicion of COVID-19 (n = 569).Initial CXR is useful as a triage tool with a sensitivity of 61% and specificity of 76% in the diagnosis of COVID-19 in a hospital setting.

Dataset on a primary lithium battery cell with a ferroelectric Li-glass electrolyte and MnO2 cathode.

Here we show the electrochemical data for a Ferroelectric Electrolyte Battery (FEB) Li/ferroelectric Li-glass electrolyte (Li2·99Ba0·005ClO) in cellulose/γ-MnO2 pouch-cell with (2.5 × 2.5 cm2) discharged with a green LED load. The Li2·99Ba0·005ClO electrolyte was synthesized and ground in ethanol. A cellulose matrix was dipped into the Li-glass/ethanol slurry. The γ-MnO2 based cathode was doctor bladed onto a carbon-coated aluminum foil current collector. The cell was assembled in an Ar-filled glove-box and it was not sealed and, therefore, it remained inside the glove-box while discharging with a green LED at approximately 24 °C for 334 days (>11 months) corresponding to 764 mAhg-1 of the active cathode and to 224 mAhg-1 of the electrolyte. The maximum capacity of γ-MnO2 is 209 mAhg-1 and of the MnO2 in the commercial cell is 308 mAhg-1, corresponding to LiMnO2; therefore, the capacity of the FEB is 370% the capacity of the γ-MnO2 and 250% the capacity of the MnO2 in the commercial cell. Moreover, the experimental capacity of the electrolyte minus the maximum capacity of the γ-MnO2 is 163 mAhg-1 of the electrolyte. The potential difference between anode and cathode in a diode is non-linear and dependent on the input current and, therefore, the plateaus in the potential vs time curves do not correspond to thermodynamic equilibria of the electrochemical cell energy source. Nevertheless, the maximum output current as well as the FEB cell's discharge profile may be determined with an LED and compared with traditional battery cells' profiles. The present data might be used by the electrochemical (in particular, battery), electrostatic and ferroelectric materials researchers and industrials for comparative analysis. Furthermore, it can be reused to calculate the maximum energy stored electrostatically in these devices.

Dataset on a ferroelectric based electrostatic and electrochemical Li-cell with a traditional cathode.

Here we show the electrochemical raw data for a Li/ferroelectric Li-glass electrolyte/plasticizer/Li-rich, F doped LNMO coin cell where the plasticizer is succinonitrile-SN. The nominal composition of the active oxide-host cathode particles is Li1.36Ni0.49Mn1.15O3.28F0.36 (LNMO) that disproportionated into 78 wt% spinel phase LiNi1/2Mn3/2O3.8F0.2 and 22 wt% Li-rich, F-doped layered phase containing Li2MnO3 planes separated by Li+ and Ni2+ ions. The Li2.99Ba0.005OCl electrolyte was synthesized and ground in ethanol. A cellulose matrix was dipped into the glass/ethanol slurry. This cell has been cycling for two years and six months. The electrochemical performance was firstly published in graphs after cycling the cell for about one year and three months [1]. The Li//LNMO CR2032 coin cell was assembled in an argon-filled glove box and electrochemically tested in a battery testing analyzer (LAND) at room temperature and at constant specific current densities and potentials between 2.5 and 4.8 V. Moreover, the cell's cycling current is 23 mA g-1 (active cathode). The data might be used by the electrochemical (in particular, battery), electrostatic and ferroelectric researchers and industrials for comparative analysis. Furthermore, it can be reused by anyone interested in solid-state devices that wants to calculate the maximum energy stored electrostatically in these devices.

Extraordinary Dielectric Properties at Heterojunctions of Amorphous Ferroelectrics.

Materials having a high dielectric constant are needed for a variety of electrical applications from transistors to capacitors. Ferroelectric amorphous-oxide (glass) alkali-ion electrolytes of composition A2.99Ba0.005ClO (A = Li, Na) are shown by two different types of measurement and different consistent analyses to have extraordinarily high dielectric constants, varying from 109 at 25 °C to 1010 at 220 °C if the glass is properly conditioned. These anomalously high dielectric properties coexist with alkali-ion conductivities at 25 °C that are equivalent to those of the best organic-liquid electrolytes of a Li-ion cell, and cyclic voltammetry (CV) in a Au/glass electrolyte/Au cell is stable from -10 to +10 V. A model to interpret microscopically all the key features of the CV curves shows that the electric-double-layer capacitors that form at the gold/electrolyte interfaces in the Au/glass electrolyte/Au heterojunction reverse polarization at an applied voltage V = ±2.1 V, resulting in three almost equivalent discharging capacitances for a single physical capacitor from -10 to +10 V.

Correction to: Stent placement in patients with acute subarachnoid haemorrhage: when is it justified?

In the original version of this article one author name was published incorrectly: Tanja Ddjurdjevic has been corrected to Tanja Djurdjevic.

Nontraditional, Safe, High Voltage Rechargeable Cells of Long Cycle Life.

A room-temperature all-solid-state rechargeable battery cell containing a tandem electrolyte consisting of a Li+-glass electrolyte in contact with a lithium anode and a plasticizer in contact with a conventional, low cost oxide host cathode was charged to 5 V versus lithium with a charge/discharge cycle life of over 23,000 cycles at a rate of 153 mA·g-1 of active material. A larger positive electrode cell with 329 cycles had a capacity of 585 mAh·g-1 at a cutoff of 2.5 V and a current of 23 mA·g-1 of the active material; the capacity rose with cycle number over the 329 cycles tested during 13 consecutive months. Another cell had a discharge voltage from 4.5 to 3.7 V over 316 cycles at a rate of 46 mA·g-1 of active material. Both the Li+-glass electrolyte and the plasticizer contain electric dipoles that respond to the internal electric fields generated during charge by a redistribution of mobile cations in the glass and by extraction of Li+ from the active cathode host particles. The electric dipoles remain oriented during discharge to retain an internal electric field after a discharge. The plasticizer accommodates to the volume changes in the active cathode particles during charge/discharge cycling and retains during charge the Li+ extracted from the cathode particles at the plasticizer/cathode-particle interface; return of these Li+ to the active cathode particles during discharge only involves a displacement back across the plasticizer/cathode interface and transport within the cathode particle. A slow motion at room temperature of the electric dipoles in the Li+-glass electrolyte increases with time the electric field across the EDLC of the anode/Li+-glass interface to where Li+ from the glass electrolyte is plated on the anode without being replenished from the cathode, which charges the Li+-glass electrolyte negative and consequently the glass side of the Li+-glass/plasticizer EDLC. Stripping back the Li+ to the Li+-glass during discharge is enhanced by the negative charge in the Li+-glass. Since the Li+-glass is not reduced on contact with metallic lithium, no passivating interface layer contributes to a capacity fade; instead, the discharge capacity increases with cycle number as a result of dipole polarization in the Li+-glass electrolyte leading to a capacity increase of the Li+-glass/plasticizer EDLC. The storage of electric power by both faradaic electrochemical extraction/insertion of Li+ in the cathode and electrostatic stored energy in the EDLCs provides a safe and fast charge and discharge with a long cycle life and a greater capacity than can be provided by the cathode host extraction/insertion reaction. The cell can be charged to a high voltage versus a lithium anode because of the added charge of the EDLCs.

Stent placement in patients with acute subarachnoid haemorrhage: when is it justified?

PURPOSE: Endovascular stents are widely used for the elective treatment of cerebral aneurysms. Acute stenting is performed in the management of dissections, pseudo-aneurysms, broad-based aneurysms or as a 'bail out' measure after coil migration. The purpose of this study is to review the safety of using stents in acute subarachnoid haemorrhage. METHODS: The stent registry of our institution was reviewed for procedures in patients with acute subarachnoid haemorrhage. Imaging studies were reviewed on the hospital's PACS system and the patients' notes were retrieved to assess complications and clinical outcomes. Procedures were analysed according to the type of stent, treatment indication, antiplatelet regime, complications and outcomes. RESULTS: Between 2008 and 2016, 51 stents were placed during 50 stenting procedures in 49 patients with acute subarachnoid haemorrhage. This included 24 patients with saccular aneurysms, 10 with blister aneurysms, 10 dissections and five fusiform aneurysms. Stents were deployed in 'bail out' situations on eight occasions. In six cases, flow-diverting stents were used. Eighteen patients (37%) in the cohort suffered a stroke. Nine patients (18%) suffered persistent clinical deficits as a result of the stenting procedure, all but one of which occurred within 24 h. Two patients had a transient ischaemic episode, and there was evidence of asymptomatic ischaemia on imaging in four cases (8%). Five patients died, three (6%) as a result of procedural complications. Twelve patients (25%) required a further embolisation procedure. CONCLUSION: The use of stents in acute subarachnoid haemorrhage incurs a considerable complication risk and should be reserved for exceptional circumstances.

The importance of early immunotherapy in patients with faciobrachial dystonic seizures.

Faciobrachial dystonic seizures and limbic encephalitis closely associate with antibodies to leucine-rich glioma-inactivated 1 (LGI1). Here, we describe 103 consecutive patients with faciobrachial dystonic seizures and LGI1 antibodies to understand clinical, therapeutic and serological differences between those with and without cognitive impairment, and to determine whether cessation of faciobrachial dystonic seizures can prevent cognitive impairment. The 22/103 patients without cognitive impairment typically had normal brain MRI, EEGs and serum sodium levels (P < 0.0001). Overall, cessation of faciobrachial dystonic seizures with antiepileptic drugs alone occurred in only 9/89 (10%) patients. By contrast, 51% showed cessation of faciobrachial dystonic seizures 30 days after addition of immunotherapy (P < 0.0001), with earlier cessation in cognitively normal patients (P = 0.038). Indeed, expedited immunotherapy (P = 0.031) and normal cognition (P = 0.0014) also predicted reduced disability at 24 months. Furthermore, of 80 patients with faciobrachial dystonic seizures as their initial feature, 56% developed cognitive impairment after 90 days of active faciobrachial dystonic seizures. Whereas only one patient developed cognitive impairment after cessation of faciobrachial dystonic seizures (P < 0.0001). All patients had IgG4-LGI1 antibodies, but those with cognitive impairment had higher proportions of complement-fixing IgG1 antibodies (P = 0.03). Both subclasses caused LGI1-ADAM22 complex internalization, a potential non-inflammatory epileptogenic mechanism. In summary, faciobrachial dystonic seizures show striking time-sensitive responses to immunotherapy, and their cessation can prevent the development of cognitive impairment.awx323media15681705685001.

Predictive value of MRI parameters in severity and recovery of first-episode myelitis in aquaporin-4 antibody disease.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) associated with aquaporin-4 antibodies (AQP4-Ab) typically causes longitudinally-extensive transverse myelitis (LETM). Few data exist about the association of MRI features with LETM attack severity and recovery. METHODS: AQP4-Ab positive NMOSD patients with a first myelitis attack were retrospectively identified and spinal MRI scans reviewed. Association of MRI features with EDSS scores at attack nadir and recovery was evaluated. RESULTS: 22 patients were included. Median nadir EDSS score was 8 (range 1 to 8.5). Nadir EDSS scores correlated with total MRI lesion length (r=0.48, p=0.025), higher scores were seen in those with gadolinium enhancement (p=0.025) and there was a trend towards higher scores with central cord involvement. The median recovery EDSS was 6 (range 0 to 10). Total lesion length correlated with poor recovery (r=0.48, p=0.027) but this was confounded by correlation between nadir and recovery EDSS scores. CONCLUSION: We confirm that myelitis in AQP4-Ab disease is severe and show that the severity correlates with lesion length and residual disability. Spinal cord lesions in first myelitis attacks are similar to appearances reported later in the disease course, with propensity to involve the central grey matter and high frequency of cord oedema and T1 hypointensity.