Paraneoplastic Neuromyelitis Optica Spectrum Disorder Associated With Lung Adenocarcinoma: A Case Report

Neuromyelitis Optica spectrum disorder is an inflammatory demyelinating disease affecting the central nervous system (CNS), characterized by triad optic neuritis, transverse myelitis, and area postrema syndrome. Antibodies directed against aquaporin-4 (AQP-4), a water channel expressed on the astrocytic membrane, are supposed to play a pathogenic role and are detected in ~80% of cases. Clinical signs of Neuromyelitis Optica spectrum disorder (NMOSD) in elderly patients should arouse the suspicion of paraneoplastic etiology. In this article, we discussed a case of a 76-year-old woman with a 2-month history of confusion, dysarthria, and progressive bilateral leg weakness. A whole-body CT scan showed a neoformation of 5 cm in diameter in the median lobe infiltrating the mediastinal pleura. The tumor had already spread to both the upper and lower right lobes, parietal pleura, and multiple lymph nodes. Pleural cytology revealed adenocarcinoma cells. The brain MRI documented hyperintense alteration in fluid-attenuated inversion recovery (FLAIR) images, involving the anterior portion of the corpus callosum and the periependymal white matter surrounding the lateral ventricles, with mild contrast enhancement on the same areas and meningeal tissue. T2-weighted spinal cord MRI sequences showed extended signal hyperintensity from bulbo-cervical junction to D7 metamer, mainly interesting the central component and the gray matter. Cerebrospinal fluid analysis revealed no neoplastic cells. Serum AQP-4 immunoglobulin (IgG) antibodies were found. Meanwhile, the patient rapidly developed progressive paraparesis and decreased level of consciousness. High-dose intravenous methylprednisolone therapy was started but her conditions rapidly deteriorated. No other treatment was possible.

Comparing two artificial intelligence software packages for normative brain volumetry in memory clinic imaging

Purpose To compare two artificial intelligence software packages performing normative brain volumetry and explore whether they could differently impact dementia diagnostics in a clinical context. Methods Sixty patients (20 Alzheimer’s disease, 20 frontotemporal dementia, 20 mild cognitive impairment) and 20 controls were included retrospectively. One MRI per subject was processed by software packages from two proprietary manufacturers, producing two quantitative reports per subject. Two neuroradiologists assigned forced-choice diagnoses using only the normative volumetry data in these reports. They classified the volumetric profile as “normal,” or “abnormal”, and if “abnormal,” they specified the most likely dementia subtype. Differences between the packages’ clinical impact were assessed by comparing (1) agreement between diagnoses based on software output; (2) diagnostic accuracy, sensitivity, and specificity; and (3) diagnostic confidence. Quantitative outputs were also compared to provide context to any diagnostic differences. Results Diagnostic agreement between packages was moderate, for distinguishing normal and abnormal volumetry (K = .41–.43) and for specific diagnoses (K = .36–.38). However, each package yielded high inter-observer agreement when distinguishing normal and abnormal profiles (K = .73–.82). Accuracy, sensitivity, and specificity were not different between packages. Diagnostic confidence was different between packages for one rater. Whole brain intracranial volume output differed between software packages (10.73%, p < .001), and normative regional data interpreted for diagnosis correlated weakly to moderately (rs = .12–.80). Conclusion Different artificial intelligence software packages for quantitative normative assessment of brain MRI can produce distinct effects at the level of clinical interpretation. Clinics should not assume that different packages are interchangeable, thus recommending internal evaluation of packages before adoption.

Sparse Representation-Based Discriminative Metric Learning for Brain MRI Image Retrieval

Magnetic resonance imaging (MRI) can have a good diagnostic function for important organs and parts of the body. MRI technology has become a common and important disease detection technology. At the same time, medical imaging data is increasing at an explosive rate. Retrieving similar medical images from a huge database is of great significance to doctors’ auxiliary diagnosis and treatment. In this paper, combining the advantages of sparse representation and metric learning, a sparse representation-based discriminative metric learning (SRDML) approach is proposed for medical image retrieval of brain MRI. The SRDML approach uses a sparse representation framework to learn robust feature representation of brain MRI, and uses metric learning to project new features into the metric space with matching discrimination. In such a metric space, the optimal similarity measure is obtained by using the local constraints of atoms and the pairwise constraints of coding coefficients, so that the distance between similar images is less than the given threshold, and the distance between dissimilar images is greater than another given threshold. The experiments are designed and tested on the brain MRI dataset created by Chang. Experimental results show that the SRDML approach can obtain satisfactory retrieval performance and achieve accurate brain MRI image retrieval.

Recovery Nystagmus in Vestibular Neuritis with Minimal Canal Paresis. Clinical Observation and Interpretation

Recovery nystagmus in vestibular neuritis patients is a reversal of spontaneous nystagmus direction, beating towards the affected ear, observed along the time course of central compensation. It is rarely registered due either to its rarity as a phenomenon per se, or to the fact that it is missed between follow-up appointments. The aim of the manuscript is to describe in detail a case of recovery nystagmus found in an atypical case of vestibular neuritis and discuss pathophysiology and clinical considerations regarding this rare finding. A 26-year-old man was referred to our Otorhinolaryngology practice reporting “dizziness” sensation and nausea in the last 48 h. Clinical examination revealed left beating spontaneous nystagmus (average slow phase velocity aSPV 8.1°/s) with absence of fixation. The head impulse test (H.I.T.) was negative. Cervical vestibular evoked myogenic potentials (cVEMP) and Playtone audiometry (PTA) were normal. Romberg and Unterberger tests were not severely affected. A strong directional preponderance to the left was found in caloric vestibular test with minimal canal paresis (CP 13%) on the right. The first follow-up consultation took place on the 9th day after the onset of symptoms. Right beating weak (aSPV 2.4°/s) spontaneous nystagmus was observed with absence of fixation, whereas a strong right directional preponderance (DP) was found in caloric vestibular test. A brain MRI scan was ordered to exclude central causes of vertigo, which was normal. The patient was seen again completely free of symptoms 45 days later. He reported feeling dizzy during dynamic movements of the head and trunk for another 15 days after his second consultation. The unexpected observation of nystagmus direction reversal seven days after the first consultation is a typical sign of recovery nystagmus. Recovery nystagmus (RN) is centrally mediated and when found, it should always be carefully assessed in combination with the particularities of vestibular neuritis.

Whole-Body MRI Surveillance—Baseline Findings in the Swedish Multicentre Hereditary TP53-Related Cancer Syndrome Study (SWEP53)

A surveillance strategy of the heritable TP53-related cancer syndrome (hTP53rc), commonly referred to as the Li–Fraumeni syndrome (LFS), is studied in a prospective observational nationwide multi-centre study in Sweden (SWEP53). The aim of this sub-study is to evaluate whole-body MRI (WB-MRI) regarding the rate of malignant, indeterminate, and benign imaging findings and the associated further workup generated by the baseline examination. Individuals with hTP53rc were enrolled in a surveillance program including annual whole-body MRI (WB-MRI), brain-MRI, and in female carriers, dedicated breast MRI. A total of 68 adults ≥18 years old have been enrolled to date. Of these, 61 fulfilled the inclusion criteria for the baseline MRI scan. In total, 42 showed a normal scan, while 19 (31%) needed further workup, of whom three individuals (3/19 = 16%) were diagnosed with asymptomatic malignant tumours (thyroid cancer, disseminated upper GI cancer, and liver metastasis from a previous breast cancer). Forty-three participants were women, of whom 21 had performed risk-reducing mastectomy prior to inclusion. The remaining were monitored with breast MRI, and no breast tumours were detected on baseline MRI. WB-MRI has the potential to identify asymptomatic tumours in individuals with hTP53rc syndrome. The challenge is to adequately and efficiently investigate all indeterminate findings. Thus, a multidisciplinary team should be considered in surveillance programs for individuals with hTP53rc syndrome.

Torcular dural sinus malformation

The dural sinus malformation (DSM) are rare congenital vascular anomalies with variable anatomic features, clinical conditions, and outcomes. There are two forms of disease: a lateral subtype, affecting the jugular bulb with associated high flow arteriovenous fistula; and a midline subtype, called torcular DSM. The torcular subtype is more common and characterized by a giant dural sinus lake involving the confluens sinuum (torcular Herophili). We present a case of a 28-year-old woman with an ultrasound at 32 weeks’ gestation showing a fetal intracranial thrombosed lesion, measuring 6x4 cm. An elective c-section was performed at 38 weeks’ gestation, and a male neonate was born. He remained asymptomatic, and a brain MRI performed 9 days later showed a thrombosed midline mass due to a torcular DSM. The outcome with conservative treatment was favorable, and further development was unremarkable. The 2-year follow-up imaging demonstrated spontaneous involution of the clot. The torcular DSM is frequently diagnosed prenatally as an intraluminar lake thrombosis, and differential diagnosis include tumors, subdural collections, vein of Galen aneurysmal malformations, pial malformations, arachnoid and dermoid cysts. A literature review involving 126 patients with torcular DSM found an overall mortality of 22.1%. Torcular DSM seems to have better prognosis than those having dural arteriovenous shunts, and the worst outcomes were associated to brain damage and patent feeders. In addition, antenatal diagnosis and thrombosis of pouch have good prognosis, possibly because the clot may spontaneously obliterate the fistulas. Treatment options must be individualized, and include conservative, embolization, ventriculoperitoneal shunt and endoscopic third ventriculostomy. Although systemic anticoagulation is controverse in infants, it should be considered for patients with sinovenous thrombosis affecting outflow pathways, especially those aggravated by venous congestion. Intervention is recommended for grade III patients because they progress to grade IV, and endovascular treatment is the gold standard therapy due to the risk of fatal intraoperative exsanguination. Whenever possible, embolization should be prioritized over hydrocephalus treatment because it may avoid ventriculoperitoneal shunt. Close follow-up imaging is recommended to detect any changes, with lesion reduction indicating favorable outcome.

Cognitive Sequelae of Silent Ischemic Lesions Following Carotid Artery Stenting: Possible Role of Age-Related Moderation

Background: The occurrence of ischemic lesions is common in patients receiving carotid artery stenting (CAS), and most of them are clinically silent. However, few studies have directly addressed the cognitive sequelae of these procedure-related silent ischemic lesions (SILs).Objective: In this study, we attempted to investigate the effects of SILs on cognition using a comprehensive battery of neuropsychological tests.Method: Eighty-five patients with unilateral carotid stenosis and 25 age-matched healthy volunteers participated in this study. Brain MRI was performed within 1 week before and 1 week after CAS to monitor the occurrence of post-CAS SILs. A comprehensive battery tapping reading ability, verbal and non-verbal memory, visuospatial function, manual dexterity, executive function, and processing speed was administered 1 week before and 6 months after CAS. To control for practice effects on repeated cognitive testing, the reliable change index (RCI) derived from the healthy volunteers was used to determine the cognitive changes in patients with carotid stenosis.Results: Among the 85 patients with carotid stenosis, 21 patients received medical treatment (MED group), and procedure-related SILs were noted in 17 patients (SIL+ group) but not observed in 47 patients (SIL– group) after undergoing CAS. Two-way (group × phase) ANOVA revealed that the volunteer group showed improved scores in most cognitive tests while only limited improvement was noted in the SIL– group. The MED and control groups tended to show improvement in the follow-up cognitive testing than the SIL+ group. However, most of the cognitive changes for each patient group did not exceed the upper or lower limits (z = ±1.0) of the RCI.Conclusions: Although the occurrence of procedure-related SILs is common in patients undergoing CAS, their impacts on cognitive changes after CAS may be limited. The practice effect should be taken into consideration when interpreting cognitive changes following CAS.