Clinico-radiologic Spectrum and Outcome of Pediatric Acquired Demyelinating Disorders of Central Nervous System: A Retrospective Indian Tertiary Care Hospital Cohort.

BACKGROUND: Pediatric acquired demyelinating syndrome (ADS) constitutes a group of treatable disorders with acute neurologic dysfunction. Neuroimaging has played a significant role in diagnosis of ADS. We describe clinico-radiologic spectrum, outcomes, and comparison of the groups: acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorder (NMOSD), clinically isolated syndrome (CIS), multiple sclerosis (MS), and myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD). METHODS: Retrospective review of 70 children with ADS at a tertiary care hospital over 15 years (2008-2023) was performed. Diagnosis was assigned as per International Pediatric Multiple Sclerosis Study Group criteria 2016. Fisher's exact and chi-square tests were applied. RESULTS: Thirty-nine boys and 31 girls aged 8.2 ± 4.0 years with CIS (n = 27), ADEM (n = 16), NMOSD (n = 13), MS (n = 1), and MOGAD (n = 13) were included. Clinical syndromes with positive significant association included polyfocal symptoms, encephalopathy in ADEM, optic neuritis (ON) in MOGAD, brainstem, area postrema syndrome in NMOSD. MOGAD presented with atypical presentations like prolonged fever (PF; 76.9%) and aseptic meningitis (23%). Seropositivity for myelin oligodendrocyte glycoprotein immunoglobulin-G was 62% and for NMO-IgG 2.6%. Neuroimaging of MOGAD showed lesions predominantly in basal ganglia/thalami (69.2%), optic nerve (46.2%), and cerebellum (46.2%). Imaging patterns between ADEM and MOGAD were comparable except for more ON (p = 0.004), spinal cord (p = 0.01), and cerebellar lesions (p = 0.03) in MOGAD. Area postrema lesion was unique to NMOSD. All patients received immunotherapy, of whom 91.4% (n = 64) had good recovery, 8.6% (n = 6) had functional limitation on modified Rankin scale at discharge, and 12 (17.1%) relapsed. CONCLUSION: The largest group was CIS. Seropositivity of MOG was high with atypical presentations like PF and aseptic meningitis. Specific neuroimaging patterns correlated with ADS categories. Short-term outcome with immunotherapy was favorable in spite of relapses.

An Eye Opener for a Blinding Disease-Orbital Infarction Syndrome.

Orbital infarction syndrome (OIS) is a disease of rare occurrence owing to the rich orbital vascular anastomotic network. We describe a case of a middle-aged female who presented with an acute left middle cerebral artery (MCA) territory infarct with left terminal internal carotid artery and MCA occlusion, underwent emergency mechanical thrombectomy, and developed painful loss of vision shortly after diagnosed as OIS based on clinical and radiological findings. The rarity and severity of OIS, especially in the setting of mechanical thrombectomy, warrant radiologists to be aware of this entity to ensure preventive measures or aid in prompt diagnosis to institute timely treatment.

Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion following SARS-CoV-2 Infection-A Rare Case Report.

Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a clinicoradiological syndrome first recognized during the influenza pandemic in Japanese population in the late twentieth century. 1 In this article, we presented a rare case report of AESD in a young child due to severe acute respiratory syndrome coronavirus 2 infection (SARS-CoV-2) who presented with febrile status epilepticus, persistent encephalopathy, and had recurrence of seizures on day 4 of illness with characteristic magnetic resonance imaging findings and a relatively fair outcome.

Endoscopic Repair of Nasopharyngeal Glial Heterotopia in a Neonate with Persistent Craniopharyngeal Canal: A Case Report.

A case of nasopharyngeal glial heterotopia with persistent craniopharyngeal canal is being reported. These lesions though rare should be considered in the differential diagnosis of neonates who present with nasal obstruction. Careful radiological evaluation for a persistent craniopharyngeal canal and differentiating the nasopharyngeal mass from brain tissue is of paramount importance.

Post-traumatic Oculomotor Nerve Palsy due to Intraneural Hemorrhage: A Case Report.

Oculomotor nerve palsy following traumatic brain injury is a rare entity. A head injury can result in diffuse neuronal axonal injury with subsequent microbleed within the nerve tissue. We report an unusual case of a patient who presented with complete isolated right third nerve palsy following a road traffic accident. In this patient, magnetic resonance imaging (MRI) revealed swelling and edema of the right oculomotor nerve along its cisternal segment with contrast enhancement. The acquisition of susceptibility-weighted images on MRI helped to clinch the diagnosis of intraneural hemorrhage as a cause of post-traumatic oculomotor nerve palsy.

Functional network connectivity imprint in febrile seizures.

Complex febrile seizures (CFS), a subset of paediatric febrile seizures (FS), have been studied for their prognosis, epileptogenic potential and neurocognitive outcome. We evaluated their functional connectivity differences with simple febrile seizures (SFS) in children with recent-onset FS. Resting-state fMRI (rs-fMRI) datasets of 24 children with recently diagnosed FS (SFS-n = 11; CFS-n = 13) were analysed. Functional connectivity (FC) was estimated using time series correlation of seed region-to-whole-brain-voxels and network topology was assessed using graph theory measures. Regional connectivity differences were correlated with clinical characteristics (FDR corrected p < 0.05). CFS patients demonstrated increased FC of the bilateral middle temporal pole (MTP), and bilateral thalami when compared to SFS. Network topology study revealed increased clustering coefficient and decreased participation coefficient in basal ganglia and thalamus suggesting an inefficient-unbalanced network topology in patients with CFS. The number of seizure recurrences negatively correlated with the integration of Left Thalamus (r = - 0.58) and FC of Left MTP to 'Right Supplementary Motor and left Precentral' gyrus (r = - 0.53). The FC of Right MTP to Left Amygdala, Putamen, Parahippocampal, and Orbital Frontal Cortex (r = 0.61) and FC of Left Thalamus to left Putamen, Pallidum, Caudate, Thalamus Hippocampus and Insula (r 0.55) showed a positive correlation to the duration of the longest seizure. The findings of the current study report altered connectivity in children with CFS proportional to the seizure recurrence and duration. Regardless of the causal/consequential nature, such observations demonstrate the imprint of these disease-defining variables of febrile seizures on the developing brain.

Coronavirus Disease 2019 (COVID-19)-Associated Rhino-Orbito-Cerebral Mucormycosis: A Multi-Institutional Retrospective Study of Imaging Patterns.

BACKGROUND: Mucormycosis infection of the maxillofacial region and brain has been associated with coronavirus disease 2019 (COVID-19) infection. Mucormycosis was relatively a rare infection before COVID-19, and imaging findings are not very well described. MATERIALS AND METHODS: A retrospective imaging study of 101 patients diagnosed with COVID-19-associated mucormycosis by histopathology and/or culture was performed. All patients underwent computed tomography and/or magnetic resonance imaging based on the clinical condition of the patient and on consensus decision by the team of treating physicians. A simple 3-stage classification system based on imaging findings was adopted. RESULTS: One hundred one cases were included in the final analysis (mean age = 55.1 years; male/female ratio = 67:34). The affected patients had diabetes in 94% of the instances (n = 95), 80.1% (n = 81) received steroids), whereas 59.4% (n = 60) patients received supplemental oxygen. The majority underwent surgical intervention, whereas in 6 cases, patients were treated with antibiotic regimens. Sixty subjects improved following therapy, whereas 18 eventually succumbed to the illness. We noted a significant positive correlation between the imaging stage and outcomes. No association was seen between other clinical parameters and final clinical outcomes. Salient imaging findings include lack of normal sinonasal mucosal enhancement, perisinus inflammation, ischemic optic neuropathy, perineural spread, pachymeningeal enhancement, and presence of strokes. CONCLUSIONS: We describe the imaging findings in the largest cohort of patients with rhino-orbito-cerebral mucormycosis in the context of the current COVID-19 pandemic. A simplified staging system described here is helpful for standardized reporting and carries prognostic information.

High flow arteriovenous malformation with transverse-sigmoid sinus stenosis and congestive venopathy mimicking DAVF.

BACKGROUND: Arteriovenous malformations commonly present with hemorrhage, seizures, headache and other symptomatology. However, AVMs presenting as venous hypertension, owing to downstream stenosis/occlusion of venous sinuses, are very rare. This presentation is much more common in patients with dural AVFs. CASE DESCRIPTION: We present a young lady with left frontal arteriovenous malformation with bilateral transverse-sigmoid sinus stenosis, presenting with features of venous hypertension, which was treated with surgical excision of AVM. This case demonstrates a rare example of AVM with co-existing venous sinus stenosis distal to the nidus. CONCLUSIONS: High flow AVMs may co-exist with venous sinus stenosis or occlusion and lead to congestive venopathy. Treatment of AVM with surgical resection can be performed safely to relieve the hyper-dynamic venous congestion.

Spectrum of intracranial complications of rhino-orbito-cerebral mucormycosis - resurgence in the era of COVID-19 pandemic: a pictorial essay.

Rhino-orbito-cerebral mucormycosis (ROCM) has regained significance following its resurgence in the second wave of the COVID-19 pandemic in India. Rapid and progressive intracranial spread occurs either by direct extension across the neural foraminae, cribriform plate/ethmoid, walls of sinuses, or angioinvasion. Having known to have a high mortality rate, especially with intracranial extension of disease, it becomes imperative to familiarise oneself with its imaging features. MRI is the imaging modality of choice. This pictorial essay aims to depict and detail the various intracranial complications of mucormycosis and to serve as a broad checklist of structures and pathologies that must be looked for in a known or suspected case of ROCM.

Role of Quantitative Diffusion-Weighted Imaging in Differentiating Benign and Malignant Orbital Masses.

Aim To determine the role of diffusion-weighted imaging (DWI) with apparent diffusion coefficient (ADC) values in differentiating benign and malignant orbital masses. Materials and Methods After obtaining institutional ethical board approval and informed consent from all patients, an observational study was done for a period of 24 months in the radiology department of a tertiary care hospital in South India. Conventional magnetic resonance imaging and DWI using a 3T scanner was done for all patients with suspected orbital mass lesion. ADC value and clinicohistopathological correlation were studied for every patient. Chi-square test was used to compare the signal characteristics of DWI and ADC maps between benign and malignant lesions. A comparison of mean ADC values for benign and malignant masses was performed using Student's t -test for independent samples. The cut-off value for ADC was obtained using the receiver operating characteristic (ROC) curve. Results Of 44 patients with orbital lesions, 70% were benign and 30% were malignant. There was a significant difference in the mean ADC values of benign and malignant orbital masses. Using ROC curve analysis, an optimal ADC threshold of 1.26 × 10 -3 mm 2 /s was calculated for the prediction of malignancy with 100% sensitivity, 80.65% specificity, and 86.36% accuracy (95% confidence interval: 0.872, 1.00, p < 0.0001). Two ADC thresholds were used to characterize the orbital masses with more than 90% confidence. Conclusion Quantitative assessment of ADC is a useful noninvasive diagnostic tool for differentiating benign and malignant orbital masses. Malignant orbital lesions demonstrate significantly lower ADC values as compared with benign lesions.

Precipitating hydrophobic injectable liquid embolization of intracranial vascular shunts: initial experience and technical note.

Precipitating hydrophobic injectable liquid is a new liquid embolic agent used mainly for intracranial neurointervention. The agent is ready to use (no shaking is required), since iodine, the radiopaque material, is covalently bonded into the compound. Additionally, due to the absence of tantalum, minimal artifacts are seen on postprocedure follow-up CT scans, and the agent penetrates into vessels smaller than 10 µm. The authors report their initial experience with the use of this agent in neurovascular intervention.

Spinal dysraphism illustrated; Embroyology revisited.

Spinal cord development occurs through three consecutive periods of gastrulation, primary nerulation and secondary neurulation. Aberration in these stages causes abnormalities of the spine and spinal cord, collectively referred as spinal dysraphism. They can be broadly classified as anomalies of gastrulation (disorders of notochord formation and of integration); anomalies of primary neurulation (premature dysjunction and nondysjunction); combined anomalies of gastrulation and primary neurulation and anomalies of secondary neurulation. Correlation with clinical and embryological data and common imaging findings provides an organized approach in their diagnosis.