Search

The purpose of this article is to report brain MRI findings of merosin-negative congenital muscular dystrophy (CMD) in two cases and to discuss its differentiation from other CMD subtypes. The cases were investigated by 1.5 Tesla MRI equipment using T1-weighted, T2-weighted, fluid-attenuated inversion recovery (FLAIR), 3D fast spoiled gradient recalled (SPGR) and post-contrast gadolinium enhanced sequences and the diagnosis was confirmed by muscle biopsy with immunohistochemical staining for merosin. Magnetic resonance imaging showed bilateral subcortical U-fibre white matter changes with periventricular sparing in the first case. Though this imaging finding is non-specific and could be seen in other white matter diseases like Canavan disease, the absence of other distinguishing MRI and clinical features of those conditions allowed a proper diagnosis. The second case showed diffuse subcortical and deep white matter involvement and cerebellar cysts. There was no brainstem involvement, polymicrogyria or cobblestone lissencephaly in both of our cases which allowed differentiation of merosin-negative CMD from other CMDs. Muscle biopsy in both these patients showed dystrophic changes with absent staining for merosin confirming the diagnosis. Merosin-negative CMD should be considered in the imaging differential diagnosis of white matter diseases. In a patient presenting with features of CMD, MRI can be of help in further differentiation of the various CMD subtypes. [ABSTRACT FROM AUTHOR]

Purpose: To assess whether corneal nerve analysis can identify and differentiate patients with multiple sclerosis (MS) from those with epilepsy., Methods: Participants with MS (n = 83), participants with epilepsy (n = 50), and healthy controls (HCs) (n = 20) underwent corneal confocal microscopy (CCM) and quantification of automated corneal nerve fiber length (ACNFL), automated corneal nerve fractal dimension (ACNFrD), and ACNFrD/ACNFL ratio of the subbasal nerve plexus., Results: ACNFL (MS: P < 0.0001; epilepsy: P = 0.002) and ACNFrD (MS: P < 0.0001; epilepsy: P = 0.025) were significantly lower and the ACNFrD/ACNFL ratio (MS: P < 0.0001; epilepsy: P = 0.018) was significantly higher compared to HCs. ACNFL (P = 0.001), ACNFrD (P = 0.0003), and ACNFrD/ACNFL ratio (P = 0.006) were significantly lower in patients with MS compared to those with epilepsy. ACNFL had the highest diagnostic utility for identifying patients with MS (sensitivity/specificity 0.86/0.85, area under the curve [AUC] 0.90, P < 0.0001), and ACNFrD had the highest diagnostic utility for identifying patients with epilepsy (sensitivity/specificity 0.78/0.75, AUC 0.76, P = 0.0008). ACNFrD had the highest diagnostic utility for differentiating patients with MS from epilepsy (sensitivity/specificity 0.66/0.65, AUC 0.70, <0.0001)., Conclusions: Corneal neurodegeneration occurs in and is characterized by a distinct pattern that differentiates patients with MS and epilepsy., Translational Relevance: CCM identifies and differentiates patients with MS and epilepsy, albeit with moderate performance. Further validation, with a larger sample size, is needed.

Introduction: The thymus normally forms in the neck from the third pharyngeal pouch and descends to its normal position in the mediastinum. Arrest of descent or sequestration of thymic tissue can occur at any point along its path leading to an ectopic thymus which can present as a neck mass, usually in the paediatric age group., Purpose and Case Report: Ultrasound is generally performed in the presence of a neck mass in children. Although a characteristic 'starry sky' appearance of the thymus has been described on ultrasound, it is not considered sufficiently specific and cross-sectional imaging with magnetic resonance imaging is usually performed. On magnetic resonance imaging, the ectopic thymus appears as a homogeneous T1 isointense and T2 hyperintense mass and may actually appear ominous due to the tendency of ectopic thymus to sometimes show diffusion restriction unlike the normal thymus. Subsequent invasive biopsy or surgical removal is usually necessary to rule out a neoplastic lesion. In our observation, the ultrasound appearance of thymus is sufficiently distinctive to be confidently diagnosed as ectopic thymic tissue. This appearance is similar to the high-resolution appearance of an embroidery yarn. The reason most radiologists are not aware of the same is because the normal mediastinal thymus is not usually imaged by ultrasound., Conclusion: An accurate diagnosis on ultrasound would mean avoidance of expensive cross-sectional imaging and invasive biopsy or surgical excision in favour of regular non-invasive follow-up ultrasound scans until the lesion involutes in late childhood., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article., (© The Author(s) 2024.)

Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Malignant Triton Tumors (MTTs) are a rare and aggressive subtype of malignant peripheral nerve sheath tumors (MPNSTs), often associated with neurofibromatosis type 1. This case report describes a unique instance of recurrent sporadic MTT within the carotid sheath in a 33-year-old male without any personal or familial history of neurofibromatosis. The patient initially presented with a biopsy-confirmed MTT in the right neck, involving the carotid body and brachial plexus, and underwent partial resection, radiation therapy, and chemotherapy. Six months later, the patient presented with recurrent MTT, and subsequently underwent radical tumor resection, segmental right carotid artery resection, and deep femoral vein interposition. Recovery was complicated by hematoma formation, and the patient developed vocal fold paralysis and a left vocal fold cyst, necessitating further surgeries. Yearly follow-ups for 8 years revealed no recurrence. This case emphasizes the importance of comprehensive patient evaluation, including clinical history, imaging, and biopsy findings, for accurate diagnosis and prompt surgical intervention in managing such rare and aggressive tumors. Further research is needed to identify novel therapies and improve survival rates for patients with MTTs., Competing Interests: Conflict of interestThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Spondylolisthesis is characterized by the displacement of one vertebral body in relation to the adjacent vertebra. It is commonly observed in the lower lumbar region and can be caused by a variety of factors, including spondylolysis (a fracture in the pars interarticularis) or degenerative disease. Magnetic resonance imaging (MRI) is becoming increasingly popular as the primary modality for evaluation of low back pain and is often used in the absence of radiographs or Computed Tomography. However, it can be challenging for radiologists to differentiate between the two types of spondylolisthesis based on MRI alone. The goal of this article is to identify key imaging features on MRI that can aid radiologists in differentiating between spondylolysis and degenerative spondylolisthesis on MRI. Five key concepts are discussed: the "step-off" sign, the "wide canal" sign, T2 cortical bone signal on MRI, epidural fat interposition, and fluid in the facet joints. The utility, limitations and potential pitfalls of these concepts are also discussed to provide a comprehensive understanding of their use in differentiating between the two types of spondylolisthesis on MRI., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

The "puffed cheek" technique is routinely performed during CT neck studies in patients with suspected oral cavity cancers. The insufflation of air within the oral vestibule helps in the detection of small buccal mucosal lesions, with better delineation of lesion origin, depth, and extent of spread. The pitfalls associated with this technique are often underrecognized and poorly understood. They can mimic actual lesions, forfeiting the technique's primary purpose. This review provides an overview of the puffed cheek technique and its associated pitfalls. These pitfalls include pneumoparotid, soft palate elevation that resembles a nasopharyngeal mass, various tongue displacements or distortions that obscure tongue lesions or mimic them, sublingual gland herniation, an apparent exacerbation of the airway edema, vocal cord adduction that hinders glottic evaluation, and false indications of osteochondronecrosis in laryngeal cartilage. Most stem from a common underlying mechanism of unintentional Valsalva maneuver engaged in by the patient while trying to perform a puffed cheek, creating a closed air column under positive pressure with resultant surrounding soft-tissue displacement. These pitfalls can thus be avoided by instructing the patient to maintain continuous nasal breathing while puffing out their cheek during image acquisition, preventing the formation of the closed air column. Keywords: CT, Head/Neck © RSNA, 2024.

Vertebral osteomyelitis is a well-documented disease entity in literature with various known etiologies. However, vertebral diskitis-osteomyelitis secondary to an infected aortic aneurysm is an uncommon and life-threatening complication. We present the case of a 65-year-old male patient who presented with chronic low back pain that acutely worsened for 1 to 1.5 months and was diagnosed with vertebral diskitis-osteomyelitis secondary to a contiguous infection from an adjacent mycotic aortic aneurysm. To our knowledge, this is one of the few cases reported of vertebral diskitis-osteomyelitis secondary to mycotic aortic aneurysm. We discuss the findings on CT and MRI, as well as the value of imaging in guiding management., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

CT perfusion (CTP) imaging is increasingly used for routine evaluation of acute ischemic stroke. Knowledge about the different types of CTP software, imaging acquisition and post-processing, and interpretation is crucial for appropriate patient selection for reperfusion therapy. Conventional vendor-provided CTP software differentiates between ischemic penumbra and core infarct using the tiebreaker of critically reduced cerebral blood volume (CBV) values within brain regions showing abnormally elevated time parameters like mean transit time (MTT) or time to peak (TTP). On the other hand, RAPID automated software differentiates between ischemic penumbra and core infarct using the tiebreaker of critically reduced cerebral blood flow (CBF) values within brain regions showing abnormally elevated time to maximum (Tmax). Additionally, RAPID calculates certain indices that confer prognostic value, such as the hypoperfusion and CBV index. In this review, we aim to familiarize the reader with the technical principles of CTP imaging, compare CTP maps generated by conventional and RAPID software, and discuss important thresholds for reperfusion and prognostic indices. Lastly, we discuss common pitfalls to help with the accurate interpretation of CTP imaging., (Copyright © 2023. Published by Elsevier Inc.)

Background: Corneal immune cells (ICs) are antigen-presenting cells that are known to increase ocular and systemic inflammatory conditions., Objective: We aimed to assess longitudinal changes in corneal IC in patients with multiple sclerosis (MS) and relation to disability and ongoing treatment., Design: Prospective observational study conducted between September 2016 and February 2020., Methods: Patients with relapsing-remitting MS (RRMS) ( n  = 45) or secondary progressive MS (SPMS) ( n  = 15) underwent corneal confocal microscopy (CCM) at baseline and 2-year follow-up for estimation of corneal IC density [dendritic cells with (DCF) (cells/mm 2 ) or without nerve fiber contact (DCP); and non-dendritic cells with (NCF) or without nerve fiber contact (NCP)]. Optical coherence tomography, neuroimaging, and disability assessments were additionally performed. Healthy controls ( n  = 20) were assessed at baseline., Results: In both RRMS and SPMS compared to controls, DCP ( p  < 0.001 and p  < 0.001, respectively) and DCF ( p  < 0.001 and p  = 0.005) were higher and NCF ( p  = 0.007 and p  = 0.02) was lower at baseline. DCP showed excellent performance in identifying patients with MS (sensitivity/specificity = 0.88/0.90) followed by DCF (0.80/0.75) and NCF (0.80/0.85). At follow-up compared to baseline, DCP ( p  = 0.01) was significantly reduced, and NCP ( p  = 0.004) and NCF ( p  = 0.04) were increased. Subgroup analysis showed that baseline NCP and NCF were significantly higher ( p  = 0.04-0.05) in patients who switched disease-modifying treatment, and baseline NCP ( p  = 0.05) was higher in patients on interferon., Conclusion: Baseline and change in corneal IC were related to axonal degeneration and treatment status. Evaluation of corneal IC using CCM may allow an assessment of ongoing inflammation, disease progression, and the effect of treatment in MS., Competing Interests: The authors declare that there is no conflict of interest., (© The Author(s), 2023.)

Microscopic neuroanatomy of limbic system and basal forebrain on MRI is complex and is a terra incognita for many radiologists, clinicians, and neuroscientists. Interestingly, most of the important structures/at least anatomical regions containing these structures demonstrable on cadaveric and surgical dissections can be identified on clinical MRI, with 3T being much better than 1.5T. This article teaches the practical MRI identification of these structures which will greatly help in evaluating complex ailments like temporal lobe epilepsy, Alzheimer dementia, and other neuropsychiatric disorders. This knowledge will also aid in accurate reporting of tumor spread along the white matter fasciculi in the temporal stem/basal forebrain region. Limbic system includes the mesial temporal structures and their connections, piriform cortex including "area tempestas," and the septal area comprising of subcallosal area and paraterminal gyrus. Basal forebrain includes structures like substantia innominata with basal nucleus of Meynert, diagonal gyrus/diagonal band of Broca, and nucleus accumbens lying in between the anterior perforated substance inferiorly and the anterior commissure superiorly., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Middle meningeal artery (MMA) embolization is commonly performed as either a first-line or adjunct treatment for chronic subdural hematomas (cSDH). We present the case of a 59 year-old male patient who presented with right hemibody weakness and cognitive impairment and was diagnosed with left-sided cSDH. A left MMA embolization was performed due to the recurrent nature of the chronic subdural hemorrhage and the history of prior craniotomy. On postoperative day 1, the patient developed sudden onset left facial swelling and tenderness, and a contrast computed tomography (CT) of the neck revealed acute ischemia in the left parotid gland, adjacent superior aspect of the left masseter muscle, the left lateral pterygoid, and left temporalis muscles. The patient was treated conservatively with antibiotics, steroids, and analgesics and reported resolution of symptoms on the three-month follow-up. To our knowledge, this is the first reported case of the ipsilateral parotid gland, temporalis muscle, adjacent superior aspect of the masseter muscle, and pterygoid muscle ischemia secondary to non-target particle embolization following MMA embolization in cSDH. Knowledge of normal and variant origin of the MMA and various anastomoses of this vessel with branches of the internal carotid artery (ICA), external carotid artery (ECA), and vertebrobasilar system is crucial to avoid complications during embolization., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

99m Tc-sestamibi scintigraphy localizes parathyroid adenoma as a persistent focus of uptake on delayed images, whereas thyroid glands in normal or ectopic locations are seen on only early images and wash out on delayed images. We report a case of absence of eutopic neck thyroid activity and synchronous ectopic lingual thyroid and mediastinal parathyroid adenoma on scintigraphy confirmed with CT., (© 2023 by the Society of Nuclear Medicine and Molecular Imaging.)

Key Clinical Message: Papez' circuit is a unique neural pathway in the limbic system that is correlated with seizure activity. Injuries affecting Papez' circuit are often small and unusual in location but can be identifiable in MRI and functional imaging modalities, which can be helpful in the workup of refractory epilepsy., Abstract: The Papez circuit is a unique neural pathway in the limbic system of the brain. We review a patient presenting with recurrent seizures as the main manifestation of Papez' circuit pathology. The radiologic features of ischemia involving the mammillothalamic tract in Papez' circuit were correlated with the seizure activity., Competing Interests: The authors do not have any financial relationship with any commercial organization that may have a direct or indirect interest in the content. The authors do not have a conflict of interest to declare., (© 2023 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.)

Cerebellar, hippocampal, and basal nuclei transient edema with restricted diffusion (CHANTER) syndrome is a constellation of specific imaging findings characterized by cytotoxic edema in the bilateral hippocampi, cerebellar cortices, and basal ganglia in patients presenting with altered mental status in the setting of substance intoxication. Previous case reports have demonstrated a strong correlation between CHANTER syndrome and polysubstance abuse, particularly with opioid intoxication. The patient we present in this case was found unresponsive following opioid use and demonstrated a constellation of findings on initial and follow-up imaging, consistent with CHANTER syndrome. While cases of irreversible brain damage or death during hospitalization have been reported in the literature, our patient demonstrated near-full recovery a few days after admission to the hospital. We aim to highlight the presentation and progression of CHANTER syndrome and alert clinicians and radiologists to include this entity in their diagnostic checklist for patients with polysubstance abuse and altered mental status., (© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Background: Resourceful endpoints of axonal loss are needed to predict the course of multiple sclerosis (MS). Corneal confocal microscopy (CCM) can detect axonal loss in patients with clinically isolated syndrome and established MS, which relates to neurological disability., Objective: To assess corneal axonal loss over time in relation to retinal atrophy, and neurological and radiological abnormalities in MS., Methods: Patients with relapsing-remitting (RRMS) ( n  = 68) or secondary progressive MS (SPMS) ( n  = 15) underwent CCM and optical coherence tomography. Corneal nerve fibre density (CNFD-fibres/mm 2 ), corneal nerve branch density (CNBD-branches/mm 2 ), corneal nerve fibre length (CNFL-mm/mm 2 ) and retinal nerve fibre layer (RNFL-μm) thickness were quantified along with neurological and radiological assessments at baseline and after 2 years of follow-up. Age-matched, healthy controls ( n  = 20) were also assessed., Results: In patients with RRMS compared with controls at baseline, CNFD ( p  = 0.004) and RNFL thickness ( p  < 0.001) were lower, and CNBD ( p  = 0.003) was higher. In patients with SPMS compared with controls, CNFD ( p  < 0.001), CNFL ( p  = 0.04) and RNFL thickness ( p  < 0.001) were lower. For identifying RRMS, CNBD had the highest area under the receiver operating characteristic (AUROC) curve (0.99); and for SPMS, CNFD had the highest AUROC (0.95). At follow-up, there was a further significant decrease in CNFD ( p  = 0.04), CNBD ( p  = 0.001), CNFL ( p  = 0.008) and RNFL ( p  = 0.002) in RRMS; in CNFD ( p  = 0.04) and CNBD ( p  = 0.002) in SPMS; and in CNBD ( p  = 0.01) in SPMS compared with RRMS. Follow-up corneal nerve loss was greater in patients with new enhancing lesions and optic neuritis history., Conclusion: Progressive corneal and retinal axonal loss was identified in patients with MS, especially those with more active disease. CCM may serve as an imaging biomarker of axonal loss in MS., Competing Interests: The authors declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: Drs Ioannis N. Petropoulos, Fatima Al-Shibani, Gulfidan Bitirgen, Georgios Ponirakis, Adnan Khan, Hoda Gad, Ziyad R. Mahfoud, Heba Altarawneh, Muhammad Hassan Rehman, Karen John, Dhabia Al-Merekhi, Pooja George, Ali Ulvi Uca, Ahmet Ozkagnici, Faiza Ibrahim, Reny Francis, Beatriz Canibano, Dirk Deleu, Ahmed El-Sotouhy, Surjith Vattoth, Ahmed Own, Ashfaq Shuaib, Naveed Akhtar and Saadat Kamran declare no conflict of interest. Dr. Rayaz A. Malik is a principal investigator on grants from Proctor and Gamble and Pfizer and has received consulting honoraria for serving on advisory boards for Novo Nordisk, Aventis Pharma, and Proctor and Gamble., (© The Author(s), 2023.)

Susceptibility-weighted imaging (SWI) was one of the recent and helpful advancement in magnetic resonance imaging. Its utilization -provided valuable information for the radiologists in multiple fields, including neuroradiology. SWI was able to demonstrate cerebral paramagnetic and diamagnetic substances. Therefore, the applications of this imaging technique were diverse in research and clinical neuroradiology. This article reviewed the basic technical steps, various clinical applications of SWI, and potential limitations. The practicing radiologist needs to be oriented about using SWI and phase images in the right- and left-handed MRI systems to demonstrate different brain pathologies, including neurovascular diseases, traumatic brain injuries, brain tumors, infectious and inflammatory, and neurodegenerative diseases., (Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.)

History: Part one of this case appeared 4 months previously and may contain larger images. A 21-year-old immunocompetent man who was a long-term resident of Qatar presented to the emergency department with recurrent episodes of unprovoked generalized tonic-clonic seizures lasting 2-3 minutes that spontaneously resolved and were associated with postictal confusion. The patient also had progressive mild diplopia, intermittent dizziness, and numbness in the left arm over the course of 3 months. The patient did not have any other systemic symptoms or chronic medical diseases. He did not have any history of intake of illicit drugs, supplements, or regular medications; he had not received any recent vaccinations; and he had not undergone any surgical procedures. He had no history of travel. At presentation, vital signs were normal. Neurologic examination showed mild left homonymous hemianopia, normal gait with no cerebellar signs, and preserved sensations, power, tone, and reflexes in all four limbs. An electroencephalogram showed no epileptiform discharges. Chest CT and extensive laboratory work-up, including viral, fungal, bacterial, and parasite work-up, thyroid function tests, and immunologic blood tests yielded normal results. Those included normal complete and differential blood counts and normal serum chemistry. Serum analysis was negative for antinuclear antibody, Sjögren syndrome antigens A and B, cytoplasmic antineutrophil cvtoplasmic antibody, and paraneoplastic profile. Serum evaluation was also negative for human immunodeficiency virus type 1 and type 2 RNA, and Brucella , Schistosoma , and toxoplasma antibodies. Venereal Disease Research Laboratory (VDRL) and rapid plasma regain (RPR) test results were negative. Cerebrospinal fluid (CSF) analysis revealed clear fluid and normal pressure and biochemistry, except for elevated protein concentration (0.48 g/L) (normal range, 0.15-0.45 g/L). There were 43 leukocytes/µL (99% lymphocytes) (normal range, 0-5 leukocytes/µL; lymphocytes range, 40%-80%), with no atypical or malignant cells. CSF Gram staining, acid-fast staining, cryptococcal antigen, varicella-zoster virus polymerase chain reaction (PCR), herpes simplex virus PCR, VDRL, and RPR test results were negative. CSF cultures did not show any evidence of growth of bacteria, fungi, or acid-fast bacillus. CSF flow cytometry did not show a monoclonal lymphoid population. No CSF oligoclonal bands were detected. Conventional brain MRI with intravenous administration of contrast material and perfusion study were performed and included different sequences (Figs 1-3).

Introduction: This study compared the capability of corneal confocal microscopy (CCM) with magnetic resonance imaging (MRI) brain volumetry for the diagnosis of mild cognitive impairment (MCI) and dementia., Methods: In this cross-sectional study, participants with no cognitive impairment (NCI), MCI, and dementia underwent assessment of Montreal Cognitive Assessment (MoCA), MRI brain volumetry, and CCM., Results: Two hundred eight participants with NCI (n = 42), MCI (n = 98), and dementia (n = 68) of comparable age and gender were studied. For MCI, the area under the curve (AUC) of CCM (76% to 81%), was higher than brain volumetry (52% to 70%). For dementia, the AUC of CCM (77% to 85%), was comparable to brain volumetry (69% to 93%). Corneal nerve fiber density, length, branch density, whole brain, hippocampus, cortical gray matter, thalamus, amygdala, and ventricle volumes were associated with cognitive impairment after adjustment for confounders (All P 's < .01)., Discussion: The diagnostic capability of CCM compared to brain volumetry is higher for identifying MCI and comparable for dementia, and abnormalities in both modalities are associated with cognitive impairment., Competing Interests: The authors confirm that the manuscript has been read and approved by all named authors and that there are no other persons who satisfied the criteria for authorship and are not listed. They also confirm that the order of authors listed in the manuscript has been approved by all authors. Dr. Surjith Vattoth has Elsevier book author royalty, received consulting fee as an Elsevier master author consultant in head and neck imaging, and received payment for ESNR ‐ ECHNR course faculty. None of the other authors have received or anticipate receiving income, goods, or benefit from a company that will influence the design, conduct, or reporting of the study., (© 2022 The Authors. Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring published by Wiley Periodicals, LLC on behalf of Alzheimer's Association.)

Antibody-mediated encephalitides constitute a group of inflammatory brain diseases characterized by prominent neuropsychiatric symptoms and are associated with antibodies against neuronal cell-surface proteins, ion channels, or receptors. The diagnosis and management of autoimmune encephalitis include evaluation of the clinical presentation, brain imaging, cerebrospinal fluid (CSF) findings, antibody detection, and electroencephalography (EEG) findings. This is a retrospective study of adults 18 years or older with autoimmune encephalitis due to antibodies against membrane surface antigens as well as anti-glutamic acid decarboxylase (anti-GAD) antibodies. The electronic medical record was reviewed for demographic data, clinical data, laboratory results, EEG, and imaging findings. Antibody screening was requested for 341 patients between May 2014 and December 2019. Antibody screening was positive in 37 patients presenting with seizures and/or encephalopathy. Of these, 10 patients tested positive for antibodies against neuronal surface antigens or anti-GAD antibodies-2 patients had anti-GAD antibody encephalitis, 5 had anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, and 3 had anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis. Demographics, clinical presentation, EEG, imaging, and CSF findings are reported. Autoimmune encephalitides are a diverse group of disorders with a few common clinical features and MRI findings. MRI, EEG, and CSF findings can be normal or show nonspecific findings in autoimmune encephalitis. Therefore, early diagnosis of these disorders requires a high level of suspicion to avoid delaying the diagnosis. Carefully looking for diagnostic clinical features (e.g., faciobrachial dystonic seizures in anti-LGI1 encephalitis), significant findings in MRI (e.g., limbic encephalitis), and some EEG patterns (e.g., extreme delta brush and generalized rhythmic delta activity in anti-NMDAR encephalitis) may help in early diagnosis., (© 2021. Fondazione Società Italiana di Neurologia.)

Neurodevelopmental and neurodegenerative pathology occur in Schizophrenia. This study compared the utility of corneal confocal microscopy (CCM), an ophthalmic imaging technique with MRI brain volumetry in quantifying neuronal pathology and its relationship to cognitive dysfunction and symptom severity in schizophrenia. Thirty-six subjects with schizophrenia and 26 controls underwent assessment of cognitive function, symptom severity, CCM and MRI brain volumetry. Subjects with schizophrenia had lower cognitive function (P ≤ 0.01), corneal nerve fiber density (CNFD), length (CNFL), branch density (CNBD), CNBD:CNFD ratio (P < 0.0001) and cingulate gyrus volume (P < 0.05) but comparable volume of whole brain (P = 0.61), cortical gray matter (P = 0.99), ventricle (P = 0.47), hippocampus (P = 0.10) and amygdala (P = 0.68). Corneal nerve measures and cingulate gyrus volume showed no association with symptom severity (P = 0.35-0.86 and P = 0.50) or cognitive function (P = 0.35-0.86 and P = 0.49). Corneal nerve measures were not associated with metabolic syndrome (P = 0.61-0.64) or diabetes (P = 0.057-0.54). The area under the ROC curve distinguishing subjects with schizophrenia from controls was 88% for CNFL, 84% for CNBD and CNBD:CNFD ratio, 79% for CNFD and 73% for the cingulate gyrus volume. This study has identified a reduction in corneal nerve fibers and cingulate gyrus volume in schizophrenia, but no association with symptom severity or cognitive dysfunction. Corneal nerve loss identified using CCM may act as a rapid non-invasive surrogate marker of neurodegeneration in patients with schizophrenia., (© 2022. The Author(s).)

History A 21-year-old immunocompetent man who was a long-term resident of Qatar presented to the emergency department with recurrent episodes of unprovoked generalized tonic-clonic seizures lasting 2-3 minutes that spontaneously resolved and were associated with postictal confusion. The patient also had progressive mild diplopia, intermittent dizziness, and numbness in the left arm over the course of 3 months. The patient did not have any other systemic symptoms or chronic medical diseases. He did not have any history of intake of illicit drugs, supplements, or regular medications; he had not received any recent vaccinations; and he had not undergone any surgical procedures. He had no history of travel. At presentation, vital signs were normal. Neurologic examination showed mild left homonymous hemianopia, normal gait with no cerebellar signs, and preserved sensations, power, tone, and reflexes in all four limbs. An electroencephalogram showed no epileptiform discharges. Chest CT and extensive laboratory work-up, including viral, fungal, bacterial, and parasite work-up, thyroid function tests, and immunologic blood tests yielded normal results. Those included normal complete and differential blood counts and normal serum chemistry. Serum analysis was negative for antinuclear antibody, Sjögren syndrome antigens A and B, cytoplasmic antineutrophil cvtoplasmic antibody, and paraneoplastic profile. Serum evaluation was also negative for human immunodeficiency virus type 1 and type 2 RNA, and Brucella, Schistosoma , and toxoplasma antibodies. Venereal Disease Research Laboratory (VDRL) and rapid plasma regain (RPR) test results were negative. Cerebrospinal fluid (CSF) analysis revealed clear fluid and normal pressure and biochemistry, except for elevated protein concentration (0.48 g/L) (normal range, 0.15-0.45 g/L). There were 43 leukocytes/µL (99% lymphocytes) (normal range, 0-5 leukocytes/µL; lymphocytes range, 40%-80%), with no atypical or malignant cells. CSF Gram staining, acid-fast staining, cryptococcal antigen, varicella-zoster virus polymerase chain reaction (PCR), herpes simplex virus PCR, VDRL, and RPR test results were negative. CSF cultures did not show any evidence of growth of bacteria, fungi, or acid-fast bacillus. CSF flow cytometry did not show a monoclonal lymphoid population. No CSF oligoclonal bands were detected. Conventional brain MRI with intravenous administration of contrast material and a perfusion study were performed and included different sequences (Figs 1-3).

Introduction: This study assessed the association of cerebral ischemia with neurodegeneration in mild cognitive impairment (MCI) and dementia., Methods: Subjects with MCI, dementia and controls underwent assessment of cognitive function, severity of brain ischemia, MRI brain volumetry and corneal confocal microscopy., Results: Of 63 subjects with MCI ( n = 44) and dementia ( n = 19), 11 had no ischemia, 32 had subcortical ischemia and 20 had both subcortical and cortical ischemia. Brain volume and corneal nerve measures were comparable between subjects with subcortical ischemia and no ischemia. However, subjects with subcortical and cortical ischemia had a lower hippocampal volume ( P < 0.01), corneal nerve fiber length ( P < 0.05) and larger ventricular volume ( P < 0.05) compared to those with subcortical ischemia and lower corneal nerve fiber density ( P < 0.05) compared to those without ischemia., Discussion: Cerebral ischemia was associated with cognitive impairment, brain atrophy and corneal nerve loss in MCI and dementia., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Ponirakis, Elsotouhy, Al Hamad, Vattoth, Petropoulos, Khan, Gad, Al-Khayat, Chandran, Ramadan, Elorrabi, Gadelseed, Tosino, Gawhale, Alobaidi, Khan, Manikoth, Abdelrahim, Thodi, Almuhannadi, Al-Mohannadi, AlMarri, Qazi, Own, Mahfoud, Shuaib and Malik.)

We are presenting the imaging findings of COVID-19-related leukoencephalopathy associated with bilateral reticular formation diffusion restriction in brain magnetic resonance imaging. To the best of our knowledge, this is the first reported case of bilateral reticular formation affection in a COVID-19 patient., Competing Interests: Conflict of Interest: The authors do not have any financial relationship with any commercial organization that may have a direct or indirect interest in the content., (© 2021 The Authors. Published by the British Institute of Radiology.)

An outbreak of corona virus disease 2019 (COVID-19) began in China in December 2019, and rapidly spread to become a worldwide pandemic. Neurological complications encountered in hospitalized patients include acute arterial ischemic cerebrovascular stroke, cerebral venous thrombosis, critical illness-associated cerebral microbleeds, hypertensive hemorrhagic posterior reversible encephalopathy, meningoencephalitis/flare up of infections, flare up of multiple sclerosis, acute disseminated encephalomyelitis , cerebral hemodynamic/hypoxic changes such as watershed ischemic changes and hypoxic ischemic encephalopathy, and spine manifestations of Guillain Barre syndrome and viral myelitis. The purpose of our study is to illustrate the different neuroimaging features in critically ill hospitalized COVID-19 positive patients in the State of Qatar.

We are reporting the imaging findings of the rare entity of critical illness-associated cerebral microbleeds in a COVID-19-positive 66-year-old woman with hypoxic respiratory failure, who was eventually intubated and ventilated. Multiple scattered cerebral microhaemorrhages diffusely distributed in the juxtacortical white matter and internal capsule region, sparing the deep and periventricular white matter, basal ganglia, thalami and cortex were seen, which is a unique imaging finding in critically ill patients with respiratory failure and hypoxemia requiring mechanical ventilation. The mechanism underlying these microhaemorrhages relates to the endpoint of critical illness, rather than a specific underlying disease.

Background: Rosai-Dorfman disease (RDD) is an idiopathic nonneoplastic lymphadenopathy disorder which is characterized by lymph node enlargement, but it may also present s primarily involving a variety of extranodal sites, including central nerves system and craniospinal axis. This study reports five cases of craniospinal RDD, with review of epidemiology, clinical presentation, imaging, and histopathological features with current management strategies., Case Description: Five cases of RDD are diagnosed at Hamad General Hospital, Qatar, during 2013-2018. Two cases had dural-based cranial lesions with overlying cranial involvement while three cases were having extradural thoracic spine lesions. All cases underwent surgical intervention and confirmed by histopathology., Conclusion: Craniospinal RDD is a rare clinical presentation and poses significant diagnostic challenges preoperatively due to its similarity with other neoplastic or inflammatory diseases. Surgical option to remove compressive neural pathology provides a good clinical outcome with no recurrence in long-term follow-up., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Surgical Neurology International.)

Patients with chronic idiopathic hypoparathyroidism may develop neurological complications, including calcification of the basal ganglia and other areas of the brain. In Fahr's syndrome, intracranial calcification is associated with an underlying disorder such as hypo or hyperparathyroidism. We report the case of a 37-year-old gentleman, with a history of bilateral cataract surgery and seizures, who presented with a new episode of seizure and was found to have severe hypocalcemia and bilateral symmetric intracranial calcification due to previously diagnosed primary hypoparathyroidism. He had symptoms and signs mimicking ankylosing spondylitis (AS), but with negative radiological and serological findings, not fitting into the diagnosis of axial spondyloarthropathies (SpA), as per standard criteria. Patients with long-standing idiopathic hypoparathyroidism can have severe calcification of soft tissues and bones, including vertebrae and paravertebral soft tissues, causing inflammatory back pain and stiffness. It is vital to report such cases as their occurrence is rare, and physicians should be aware of the possibility while evaluating patients with inflammatory back pain. Treatment in these cases is directed towards hypocalcemia and underlying primary pathology rather than spondyloarthropathy., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Sasi et al.)

Coronavirus disease 2019 (COVID-19) is an infectious disease caused by severe acute respiratory syndrome coronavirus 2. COVID-19-associated thrombotic events are recognized. A wide variety of neurological presentations have been recently documented. We report the first case of COVID-19 presenting with generalized seizure secondary to cerebral venous sinus thrombosis., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2020 by S. Karger AG, Basel.)

Spinal cord imaging findings in COVID-19 are evolving with the increasing frequency of neurological symptoms among COVID-19 patients. Several mechanisms are postulated to be the cause of central nervous system affection including direct virus neuroinvasive potential, post infectious secondary immunogenic hyperreaction, hypercoagulability, sepsis and possible vasculitis as well as systemic and metabolic complications associated with critical illness. Only a few case reports of spinal cord imaging findings are described in COVID-19, which include transverse myelitis, acute disseminated encephalomyelitis and post-infectious Guillain Barre' syndrome. We are describing a case of myelitis which, to the best of our knowledge, is the first reported case of myelitis in COVID-19., (© 2020 The Authors. Published by the British Institute of Radiology.)

Background: There is ambiguity regarding the role of left ventricle wall motion abnormalities (LVWMAs) as a potential cardioembolic source in patients, who satisfy embolic stroke of undetermined source (ESUS) criteria., Methods and Results: We analyzed prospectively collected data in 345 acute stroke patients, 185 (53.6%) stroke with atrial fibrillation (SwAF), and 160 (46.4%) stroke with LVWMA. LVWMA were younger (P = .003), had significantly higher frequency of stroke risk factors and lower ejection fraction (P < .001). No significant difference was found between the stroke pattern in SwAF and LVWMA except focal cortical, cortical-subcortical lesions were more frequent in LVWMA (P = .002). Mean wall motion score index (WMSI) was 1.523 (range 1.05-2.71) without any correlation between the severity of WMSI and multiple strokes (P = .976). In subgroup analyses vertical basal WMSI (P = .030) and vertical mid cavity WMSI (P = .010) was significantly related to branch arterial stroke. LVWMA 94 (65%) patients were on antiplatelet/anticoagulation compared to 47 (52.4%) with atrial fibrillation (AF), with no significant difference in stroke recurrence during 4 years follow-up (P = .15)., Conclusions: Patients with LVWMA who satisfy ESUS criteria, have stroke pattern on diffusion-weighted magnetic resonance imaging and risk of stroke recurrence similar to AF-related stroke despite being on appropriate antiplatelet medications. Further studies with anticoagulation therapy may be required in this group of patients to improve the high risk of recurrent stroke., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Visual rating of medial temporal lobe atrophy (MTA) is an accepted structural neuroimaging marker of Alzheimer's disease. Corneal confocal microscopy (CCM) is a non-invasive ophthalmic technique that detects neuronal loss in peripheral and central neurodegenerative disorders., Objective: To determine the diagnostic accuracy of CCM for mild cognitive impairment (MCI) and dementia compared to medial temporal lobe atrophy (MTA) rating on MRI., Methods: Subjects aged 60-85 with no cognitive impairment (NCI), MCI, and dementia based on the ICD-10 criteria were recruited. Subjects underwent cognitive screening, CCM, and MTA rating on MRI., Results: 182 subjects with NCI (n = 36), MCI (n = 80), and dementia (n = 66), including AD (n = 19, 28.8%), VaD (n = 13, 19.7%), and mixed AD (n = 34, 51.5%) were studied. CCM showed a progressive reduction in corneal nerve fiber density (CNFD, fibers/mm2) (32.0±7.5 versus 24.5±9.6 and 20.8±9.3, p < 0.0001), branch density (CNBD, branches/mm2) (90.9±46.5 versus 59.3±35.7 and 53.9±38.7, p < 0.0001), and fiber length (CNFL, mm/mm2) (22.9±6.1 versus 17.2±6.5 and 15.8±7.4, p < 0.0001) in subjects with MCI and dementia compared to NCI. The area under the ROC curve (95% CI) for the diagnostic accuracy of CNFD, CNBD, CNFL compared to MTA-right and MTA-left for MCI was 78% (67-90%), 82% (72-92%), 86% (77-95%) versus 53% (36-69%) and 40% (25-55%), respectively, and for dementia it was 85% (76-94%), 84% (75-93%), 85% (76-94%) versus 86% (76-96%) and 82% (72-92%), respectively., Conclusion: The diagnostic accuracy of CCM, a non-invasive ophthalmic biomarker of neurodegeneration, was high and comparable with MTA rating for dementia but was superior to MTA rating for MCI.