PRESenting a Challenge: Posterior Reversible Encephalopathy Syndrome in Pediatric Patients With Guillain-Barré Syndrome: A Case Series and Review of Literature.

BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature. METHODS: Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed. RESULTS: All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care. CONCLUSIONS: GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.

Mimics of Guillain-Barré Syndrome in Pediatric Patients Admitted to the Neuro-Intensive Care Unit of a Tertiary Care Hospital-A Case Series.

Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, but several diseases mimic GBS. We aimed to identify and report the clinical pointers and battery of tests required to differentiate Guillain-Barré syndrome from its observed mimics in the pediatric population admitted to our neuro-critical care unit. We conducted a retrospective record analysis of all pediatric patients admitted over ten years from 2008-2018, whose initial presentation was compatible with a clinical diagnosis of GBS. Eighty-three patients were at first treated as GBS, of which seven (8.4%) were found to have an alternate diagnosis-three cases of paralytic rabies, one case each of acute disseminated encephalomyelitis, cervical myeloradiculopathy, neuromyelitis optica, and a case of community-acquired Staphylococcus aureus pneumonia associated sepsis. Neurophysiological and neuro-virological testing, central nervous system imaging, and sepsis screening helped to confirm the alternate diagnosis. Our case series provides knowledge of subtle clinical differences along with the mindful use of diagnostic testing to facilitate the accurate diagnosis of GBS mimics.

MRI Spectrum of Toxic Encephalopathy-An Institutional Experience.

Background: There are numerous toxins that affect our nervous system, both central and peripheral. Innumerable differentials exist in patients of acute encephalopathy and the list can be narrowed down with appropriate imaging. Specific neuroradiological features point to a particular diagnosis in a substantial number of cases. Objective: Through this study, we aimed to demonstrate the varied imaging findings of toxic encephalopathy on MRI encountered at our institute. Material and Methods: A retrospective analysis of the patients clinically diagnosed as toxic encephalopathy and referred for imaging between March 2015 and December 2019 was done. A total of 25 patients were included. Patient records were reviewed for clinical details, laboratory investigations, and treatment; the institute Picture Archiving and Communication System provided the imaging findings. Results: Patients presenting were aged between 22 and 55 years (mean-34.3 years). Four patients (16%) presented with imaging findings characteristic of Marchiafava-Bignami disease and six patients (24%) had MRI findings of Wernicke encephalopathy. Three patients (12%) had methanol poisoning sequelae while imaging findings of nitroimidazole drug toxicity were observed in another three patients (12%). Two patients (8%) each of carbon monoxide poisoning and lead toxicity were seen. We had one patient (4%) each of isoniazid, methyl iodide, dextropropoxyphene toxicity, chronic toluene abuse, and hyperglycemia-induced hemiballismus-hemichorea. Conclusion: Our study illustrates the amalgamated spectrum of MRI appearances in various subgroups of toxic encephalopathies. Imaging substantiated by relevant history and clinical manifestations can accurately diagnose the possible causative agent in the majority of the cases.

Pharmacokinetics and Pharmacodynamics of Avian Egg-Yolk Derived Pure Anti-Snake Venom in Healthy and Disease Animal-Model.

The absence of Fc receptor binding, cost-effective production potential in large quantities in pure form, and better storage stability of avian immunoglobulin (IgY) advocate its therapeutic use as anti-snake venom (ASV). This study develops pure anti-neurotoxin (ANT- IgY) by immunizing White Leghorn hens with Cobra and Krait venoms for demonstrating antigen-antibody binding in vitro/in vivo. The purified IgY from immunized egg-yolk showed immunoprecipitation in Ouchterlony's Double Diffusion (ODD) experiment. For characterizing ANT-IgY distribution and clearance pattern, the study utilized an enzyme-linked immunosorbent assay (ELISA) in serum at different intervals following intravenous (IV) administration. The Kinetica 5.1 software estimated pharmacokinetic parameters, including half-life. The IgY showed a time-dependent elimination through the intestinal route in fecal matter. After conjugating with a fluorochrome-Vivotag-750S, injected the purified ANT-IgY intravenously into the healthy mice. Subsequently, captured live-animal images to demonstrate the distribution and elimination profile of the molecule. Intramuscular injection of fluorochrome-tagged venom created the envenomed mice model. The live-animal images demonstrated the quick mobilization of venom into vital tissues. Intravenous administration of tagged ANT-IgY in the envenomed model showed the movement of ASV to the tissues venom traffics. The observed pharmacological benefit promise scope of ASV-IgY for therapeutic use.

"Trapped labelled spins"-related signal on arterial spin labelling in the assessment of flow-diverted aneurysms: preliminary experience.

PURPOSE: To investigate ASL-MRI features of flow-diverted aneurysms, review their haemodynamic surrogates, and discuss their pertinent clinical implications. METHODS: Retrospective single institutional analysis was performed on the clinical and imaging data of patients who underwent digital subtraction angiography (DSA) and ASL-MRI after endovascular flow diversion for cerebral aneurysms. Pseudo-continuous ASL-MRI was performed with post-label delays of 1525-1800 ms. Intra-aneurysmal "trapped labelled spins" (TLS)-related hypersignal, as seen on cerebral blood flow (CBF)-weighted maps of ASL-MRI, was investigated. Intermodality equivalence with DSA [O'Kelly-Marotta (OKM) grading for occlusion], 3D-TOF-MRA, and 3D spin-echo T1-weighted ("black-blood") images was assessed. RESULTS: Ten cases were included. "TLS" signal was demonstrable in 7/8 (87.5%) of the DSA-visible flow-diverted aneurysms (OKM grade B3, n = 6; OKM grade A3, n = 2). No TLS was seen in both OKM-D (excluded) aneurysms. TLS was not visualised in an OKM-B3 aneurysm with < 3 mm opacifying remnant. 3D-TOF-MRA and ASL-MRI were discordant at 5 instances (45.4%; TOF-MRA false negative, n = 4; false positive, n = 1). Loss of flow void on black-blood images corresponded to the absence of TLS and vice versa in all cases but one. CONCLUSION: "Trapped labelled spins"-related signal on ASL-MRI occurs in patent large aneurysms that have undergone successful endovascular flow diversion. This phenomenon likely represents an interplay of a multitude of haemodynamic factors including decelerated intra-aneurysmal inflow and outflow restriction. Serial intra-saccular TLS signal changes may hold diagnostic value, including contexts where 3D-TOF-MRA interpretation becomes dubious. "Trapped labelled spins"-related signal as a non-invasive proxy marker of aneurysm patency can possibly obviate unnecessary DSA.

Novel daidzein molecules exhibited anti-prostate cancer activity through nuclear receptor ERß modulation, in vitro and in vivo studies.

Eight novel ERß selective daidzein analogues (NCE1-8) were synthesized and their anti-cancer activity was evaluated by in vitro and in vivo methods. Cytotoxicity study, Receptor binding studies, Luciferase assay, cMYC & Cyclin D1 expression and Caspase 3, 8 & 9 activities were measured to ascertain the anticancer activity and mechanism. Uterotropic, anti-androgenic and anti-tumour activities were performed in rodents. The results revealed that NCEs produced anti-prostate cancer activity in DU145, LNCaP and PC3 cell lines and 50% more active than genistein. NCEs was significantly down-regulated cMYC & Cyclin D1 genes and elevated caspase 3 & 9 levels and did not show any difference in uterotropic, anti-androgenic activities. The tumour weight was also reduced. The NCE 1 and 2 have shown ERß selectivity in receptor binding studies. Daidzein with methyl substitution at R or R1 position exhibited more ERß selectivity and could be considered as lead molecules for anti-prostate cancer activity.

HIV-related Neuropathy: Pathophysiology, Treatment and Challenges.

HIV-sensory neuropathy (HIV-SN) is a debilitating complication in HIV patients with or without anti-retroviral treatment (ART). Common symptoms of HIV-SN include pain, decreased sensation, paresthesias, and dysesthesias in a symmetric stocking-glove distribution. While HIV-1 protein such as gp120 is implicated in HIV-SN (e.g. impaired large-diameter fiber), ART itself was recently shown to contribute to HIV-SN in HIV patients and impair thin fiber. Multiple host mechanisms may play roles during the pathogenesis of HIV-SN, including neuron-glia interactions in the spinal dorsal horn (SDH), inflammation, mitochondrial dysfunction and endoplasmic reticulum stress. Concurrent infections, such as tuberculosis, also carry a higher likelihood of HIV-SN as well as environmental or genetic predisposition. Pro-inflammatory cytokines such as IL-1, IL2 receptor-alpha, and tumor necrosis factor (TNF) along with abnormal lactate levels have been identified as potential players within the complex pathophysiology of this condition. In this paper, we review the pathophysiology of HIV neuropathy, focusing on the various treatment options available or under investigation. Although several treatment options are available e.g., the capsaicin patch and spinal cord stimulation, symptomatic control of HIV-SN are often challenging. Alternative approaches such as self-hypnosis, resistance exercise, cannabinoids, and acupuncture have all shown promising results, but need further investigation.

Dissecting aneurysms of the posterior cerebral artery - A retrospective review of imaging, angiographic characteristics, endovascular management and outcome.

PURPOSE: To review management, clinical and imaging outcomes of dissecting posterior cerebral artery (PCA) aneurysms with emphasis on endovascular management in the form of parent vessel occlusion (PVO) at a tertiary care center. METHODS: Thirty-six dissecting PCA aneurysms (19 ruptured) encountered at our center between January 2013 and November 2019 were reviewed for aneurysm location/size/presence of fetal PCA/management. Postprocedural imaging of patients who underwent endovascular intervention was reviewed for PCA territory infarcts and clinical records assessed for presence of neuro-deficits and outcome. None of the patients underwent a balloon test occlusion (BTO) prior to PVO. RESULTS: The location of the aneurysms was as follows-P1 = 8/P1-P2 = 11/P2 = 5/P2-P3 = 10/P3 = 2. The mean size was 11.7x6.8 mm. Endovascular intervention was carried out in 20 patients in the form of PVO with coiling in 16 patients, coiling alone in 3 patients, and reconstruction of the left PCA with flow diverter deployment in one patient. Postprocedural PCA territory infarct was seen in 5 cases of PVO out of which 4 had a good functional recovery. Overall, 15/16 patients (93.7%) who underwent PVO had mRS ≤ 2 on follow-up. The single case with flow diverter also developed an infarct and had adverse outcome on follow up. CONCLUSION: Among the various management strategies for dissecting PCA aneurysms, PVO is feasible and relatively safe even in absence of BTO in scenarios like critically ill patients with ruptured aneurysms, difficult access and financial constraints. Thromboembolic complications and antiplatelet therapy is a concern in reconstructive strategies.

Comparative Evaluation of the Efficacy of Bioactive Ceramic Composite Granules Alone and in Combination with Platelet Rich Fibrin in the Treatment of Mandibular Class II Furcation Defects: A Clinical and Radiographic Study.

INTRODUCTION: Predictable closure of furcation defects with bone grafts, Guided Tissue Regeneration (GTR) and a combination of the two has remained an elusive goal so far. Hence, evaluation of biomimetic agents as candidate technologies for periodontal regeneration merit due consideration. In this study, Choukroun's Platelet Rich Fibrin (PRF), a second generation platelet concentrate, is combined with bone graft to examine if the addition enhances the therapeutic potential of bone graft in the management of Class II furcation defects. AIM: To evaluate and compare the clinical effectiveness of Bioactive Ceramic Composite Granules (BCCG) alone and in combination with PRF in the treatment of mandibular Class II furcation defects. MATERIALS AND METHODS: Twenty mandibular Class II furcation defects in 16 systemically healthy patients were randomly allocated to test and control groups. Test sites were treated with PRF and bone graft, while control sites were treated with BCCG alone. Soft tissue parameters (probing pocket depth and clinical attachment loss), hard tissue parameters (vertical and horizontal depth of furcation defects) and radiographic parameter (radiographic alveolar bone density) were measured at baseline and six months post surgery. Statistical analysis was performed using Wilcoxon signed rank test for intragroup comparison of parameters and Mann-Whitney U test for intergroup comparison. RESULTS: Statistically significant improvement was observed in the test group compared to the control group with respect to all the measured parameters. However, complete furcation closure was not observed at any of the treated sites. CONCLUSION: Adjunctive use of PRF with bone graft may be a more effective treatment modality in the management of mandibular Class II furcation defects when compared to bone graft alone.

[EATING DISORDERS IN PEDIATRIC AGE: A BOOM PATHOLOGY]. / TRASTORNOS DE LA CONDUCTA ALIMENTARIA EN LA EDAD PEDIÁTRICA: UNA PATOLOGÍA EN AUGE.

AIMS: to determine the characteristics of pediatric patients suffering from eating disorders that were hospitalized at Hospital Universitario de Canarias. MATERIALS AND METHODS: a retrospective study in a cohort of pediatric patients diagnosed with eating disorders and admitted in our area was developed during the last seven years. RESULTS: out of 35 patients in our study, 85.7 % were women, onset average age 13.5. 77.1% of the cases were diagnosed as anorexia nervosa- restrictive type. The most frequent analytical alterations, detected when patients were in hospital, consisted in a drop in plasma levels in retinol binding protein (RBP)- in 57.6% of the cases- and D hypovitaminosis- in 46.9 % of them: the use of high - calories supplements was required in 71.4% of patients during hospitalization. The average weight gain was higher when the body mass index (BMI) was smaller at patient's admission to hospital (p = 0,006). Conclussions: eating disorders are increasing in pediatric age: puberty is a special vulnerable period for its development, as well as medical complications secondary to malnutrition. Admission to hospital is an essential tool for handling many cases; taking the necessary monitoring leading to a weight increase, preventing complications in nutritional support and tackling the underlying psychopathology. Diagnosis and a precocious treatment are crucial to avoid an excessive weight loss and more complications.

Objetivos: determinar las características de los pacientes en edad pediátrica afectados de trastornos de la conducta alimentaria que requirieron ingreso en el Servicio de Pediatría del Complejo Hospitalario Universitario de Canarias (CHUC). Material y método: se realizó un estudio retrospectivo de una cohorte de pacientes pediátricos diagnosticados de trastornos de la conducta alimentaria e ingresados en planta de hospitalización pediátrica durante los últimos siete años. Resultados: de los 35 pacientes de la muestra, un 85,7% fueron mujeres, con una edad media al debut de 13,5 años. En el 77,1% de los casos el diagnóstico principal fue el de anorexia nerviosa de tipo restrictivo. Las alteraciones analíticas más frecuentes detectadas en el momento del ingreso consistieron en descenso de los niveles plasmáticos de proteína fijadora del retinol (RBP), presente en el 57,6% de los casos, e hipovitaminosis D, que en esta muestra estaba presente en el 46,9% de los casos. Se requirió el uso de suplementos hipercalóricos en el 71,4% de los pacientes durante la hospitalización. La ganancia ponderal media durante la estancia fue mayor cuanto menor era el índice de masa corporal (IMC) al ingreso (p = 0,006). Conclusiones: los TCA son enfermedades con incidencia creciente en la edad pediátrica. La pubertad constituye un momento de especial vulnerabilidad para el desarrollo de los TCA (así como de complicaciones médicas secundarias a la desnutrición). En muchos casos el ingreso hospitalario constituye una herramienta necesaria para un correcto manejo, instaurándose las medidas de control necesarias para la recuperación ponderal, la prevención de complicaciones del soporte nutricional y el abordaje de la psicopatología subyacente. El diagnóstico y tratamiento precoz resultan cruciales para evitar una excesiva pérdida ponderal y mayor incidencia de complicaciones.

Trastornos de la conducta alimentaria en la edad pediátrica: una patología en auge / Eating disorders in pediatric age: a boom pathology

Objetivos: determinar las características de los pacientes en edad pediátrica afectados de trastornos de la conducta alimentaria que requirieron ingreso en el Servicio de Pediatría del Complejo Hospitalario Universitario de Canarias (CHUC). Material y método: se realizó un estudio retrospectivo de una cohorte de pacientes pediátricos diagnosticados de trastornos de la conducta alimentaria e ingresados en planta de hospitalización pediátrica durante los últimos siete años. Resultados: de los 35 pacientes de la muestra, un 85,7% fueron mujeres, con una edad media al debut de 13,5 años. En el 77,1% de los casos el diagnóstico principal fue el de anorexia nerviosa de tipo restrictivo. Las alteraciones analíticas más frecuentes detectadas en el momento del ingreso consistieron en descenso de los niveles plasmáticos de proteína fijadora del retinol (RBP), presente en el 57,6% de los casos, e hipovitaminosis D, que en esta muestra estaba presente en el 46,9% de los casos. Se requirió el uso de suplementos hipercalóricos en el 71,4% de los pacientes durante la hospitalización. La ganancia ponderal media durante la estancia fue mayor cuanto menor era el índice de masa corporal (IMC) al ingreso (p = 0,006). Conclusiones: los TCA son enfermedades con incidencia creciente en la edad pediátrica. La pubertad constituye un momento de especial vulnerabilidad para el desarrollo de los TCA (así como de complicaciones médicas secundarias a la desnutrición). En muchos casos el ingreso hospitalario constituye una herramienta necesaria para un correcto manejo, instaurándose las medidas de control necesarias para la recuperación ponderal, la prevención de complicaciones del soporte nutricional y el abordaje de la psicopatología subyacente. El diagnóstico y tratamiento precoz resultan cruciales para evitar una excesiva pérdida ponderal y mayor incidencia de complicaciones (AU)

Aims: to determine the characteristics of pediatric patients suffering from eating disorders that were hospitalized at Hospital Universitario de Canarias. Materials and methods: a retrospective study in a cohort of pediatric patients diagnosed with eating disorders and admitted in our area was developed during the last seven years. Results: out of 35 patients in our study, 85.7 % were women, onset average age 13.5. 77.1% of the cases were diagnosed as anorexia nervosa- restrictive type. The most frequent analytical alterations, detected when patients were in hospital, consisted in a drop in plasma levels in retinol binding protein (RBP)- in 57.6% of the cases- and D hypovitaminosis- in 46.9 % of them: the use of high - calories supplements was required in 71.4% of patients during hospitalization. The average weight gain was higher when the body mass index (BMI) was smaller at patient's admission to hospital (p = 0,006). Conclussions: eating disorders are increasing in pediatric age: puberty is a special vulnerable period for its development, as well as medical complications secondary to malnutrition. Admission to hospital is an essential tool for handling many cases; taking the necessary monitoring leading to a weight increase, preventing complications in nutritional support and tackling the underlying psychopathology. Diagnosis and a precocious treatment are crucial to avoid an excessive weight loss and more complications (AU)

Periodontitis and Alzheimer's disease: oral systemic link still on the rise?

Over the past few years, there has been a rapid rise in the older segments of the world population, which has brought along with it a major health concern: dementia. Alzheimer's disease, considered to be the most common cause of dementia, has become a prospect feared by the elderly. Inflammation of the brain is strongly implicated in Alzheimer's disease which could be enhanced by systemic inflammation. Periodontitis being a chronic inflammatory condition, which can cause systemic inflammation, the question is whether chronic periodontitis can initiate or hasten the rate of progression of Alzheimer's disease in susceptible individuals. In this article, the authors outline the proposed oral systemic link between periodontitis and Alzheimer's disease.

Imaging findings in intracranial aspergillus infection in immunocompetent patients.

AIM: To study the neuroimaging features of craniocerebral aspergillosis infection in immunocompetent patients. MATERIALS AND METHODS: The clinical and imaging data of 12 patients of aspergillus fungal infection were retrospectively reviewed. Diagnosis of fungal infection was confirmed by histopathologic examination of surgically excised specimen, stereotactic biopsy material, or endoscopic sinus biopsy. The radiologic studies were evaluated for anatomic distribution of lesions, signal intensity, contrast enhancement, presence of hemorrhage, diffusion restriction, perfusion, and spectroscopy characteristics. Medical records, biopsy reports, and autopsy findings were also reviewed. RESULTS: Twelve cases of aspergillosis infections in immunocompetent patients were diagnosed at our hospital over a period of 10 years. Lesions could be classified based on imaging of lesions of sinonasal origin, intracranial mass lesion including both parenchymal or extraparenchymal meningeal based and stroke. Coexisting meningitis was also noted in one patient. Disease of sinonasal origin commonly showed invasion of the cavernous sinus and orbital apex resulting in visual symptoms and multiple cranial nerve palsies. Intracranial mass lesions without sinonasal involvement were seen in five cases that included isolated parenchymal lesion in two patients and dural-based mass lesions in three patients. Isolated intraparenchymal lesions included two cases of fungal cerebritis. Dural-based lesions were large granulomas with a significant mass effect. Infarcts were seen in three patients and angiography showed vessel narrowing or occlusion in all the three patients. CT demonstrated isodense to hyperdense attenuation of primary sinus disease with evidence of bone destruction in all the cases of sinonasal origin. Primary parenchymal lesions showed heterogenous attenuation with predominantly low-density areas. Dural-based lesions showed isodense to hyperdense attenuation. Magnetic resonance imaging revealed isointense to hypointense signal intensity on both T(1)-weighted (T1W) and T(2)-weighted (T2W) images in all lesions of sinonasal origin and isolated dural-based mass lesions. Primary parenchymal lesions showed heterogenous signal intensity pattern with predominantly hypointense signal on T1W and hyperintense signal on T2W images. Diffusion weighted imaging, magnetic resonance spectroscopy, and perfusion-weighted imaging gave valuable ancillary information in these cases. CONCLUSION: Sinonasal disease with intracranial extension is the commonest pattern of aspergillus infection followed by intracranial mass lesions. Hyperdense sinonasal disease with bone destruction and intracranial extension on computed tomography, hypointense signal intensity of the lesions on T2W magnetic resonance images, presence of areas of restricted diffusion, decreased perfusion on perfusion-weighted imaging, and presence of hemorrhages are key to the imaging diagnosis of fungal infection.

Septo-optic dysplasia with encephalocele.

Septo-optic dysplasia is a rare congenital anomaly presenting with visual deficits or pituitary dysfunction. We report the first case of septo-optic dysplasia associated with encephalocele, partial callosal agenesis, polymicrogyria and subcortical nodular heterotopia.