Antidesmoglein 1 and 3 serum IgG and positivity by direct immunofluorescence microscopy is associated with relapse in pemphigus in a prospective bicontinental study.

Background: Prospective studies identifying immunological parameters that can predict clinical relapse in pemphigus are scarce.

Objective: To periodically assess immunological parameters in patients with pemphigus vulgaris and foliaceous in remission to understand immunological events preceding clinical relapse.

Methods: A total of 105 patients were included.

Primary intracerebral pleomorphic liposarcoma in a young male.

Liposarcoma is the commonest soft tissue neoplasm, usually located in the deep soft tissue of the limbs. The central nervous system (CNS) as the primary site is exceedingly rare. We report a 35-year-old male patient who presented with seizure and weakness of the left half of the body for three months.

Identification of novel pathogenic variants of Calpain-3 gene in limb girdle muscular dystrophy R1.

Background: Limb Girdle Muscular Dystrophy R1 (LGMDR1) is an autosomal recessive neuromuscular disease caused by mutations in the calpain-3 (CAPN3) gene. As clinical and pathological features may overlap with other types of LGMD, therefore definite molecular diagnosis is required to understand the progression of this debilitating disease. This study aims to identify novel variants of CAPN3 gene in LGMDR1 patients.

Differences among Indian and European pemphigus patients based on demographics, clinical parameters and propensity for relapse: results of a prospective bicontinental cohort study.

Limited studies have explored pemphigus variations among different ethnic groups residing in their respective geographical locations. This bicontinental study aimed to compare clinical and immunological parameters in Indian and European pemphigus patients in complete remission, off therapy, or on minimal therapy. 105 patients (India, n= 75; Bulgaria, n=15; Greece, n=15) with pemphigus vulgaris (PV) or pemphigus foliaceous (PF) in complete remission on minimal therapy (n=64) or complete remission off therapy (n=41) were recruited.

68 Ga-Pentixafor PET/CT for In Vivo Imaging of CXCR4 Receptors in Glioma Demonstrating a Potential for Response Assessment to Radiochemotherapy: Preliminary Results.

Purpose: The aim of this study was to evaluate the diagnostic potential of 68 Ga-pentixafor PET/CT for in vivo CXCR4 receptors imaging in glioma and its possible role in response assessment to radiochemotherapy (R-CT).

Methods: Nineteen (12 men, 7 women) patients with glioblastoma multiforme (GBM) underwent 68 Ga-pentixafor PET/CT, contrast-enhanced MR, and MR spectroscopy. Patients were divided in to 2 groups, that is, group I was the presurgical (n = 9) group in which the scanning was done before surgery, and PET findings were correlated with CXCR4 receptors' density.

Immunohistochemical Surrogates for Molecular Stratification in Medulloblastoma.

Background: The WHO classification of central nervous system neoplasms (2016) recognized 4 histologic variants and genetically defined molecular subgroups within medulloblastoma (MB). Further, in the 2021 classification, new subtypes have been provisionally added within the existing subgroups reflecting the biological diversity. YAP1, GAB1, and β-catenin were conventionally accepted as surrogate markers to identify these genetic subgroups.

Profile of 193 pediatric cancer patients managed with radiation therapy: Challenges and lessons learned.

Background: Series on radiotherapy (RT) practice in pediatric malignancies are limited in India as only a few centers practice pediatric RT, particularly under anesthesia. We aimed to study the clinical profile of pediatric cancer patients treated with RT and to analyze various challenges in pediatric RT under anesthesia.

Materials And Methods: The data were prospectively maintained in Microsoft Excel spreadsheets.

Reelin Signaling Pathway and Mesial Temporal Lobe Epilepsy: A Causative Link.

Introduction: Mesial temporal lobe epilepsy (MTLE) is the most frequent form of partial epilepsy. Granule cell dispersion, resulting from aberrant neuronal migration in the hippocampus, is pathognomonic of MTLE. Reelin, a secreted neurodevelopmental glycoprotein has a crucial role in controlling the radial migration of neurons.

Double-hit and double-expressor primary central nervous system lymphoma: Experience from North India of an infrequent but aggressive variant.

Background: High-grade non-Hodgkin B-cell lymphoma is an aggressive mature B-cell lymphoma that depicts poor treatment response and worse prognosis. The presence of MYC and B-cell lymphoma 2 (BCL2) and/or B-cell lymphoma 6 (BCL6) rearrangements qualifies for triple-hit and double-hit lymphomas (THL/DHL), respectively. We attempted to explore the incidence, distribution, and clinical characteristics of the primary high-grade B-cell lymphoma of the central nervous system (CNS) in our cohort from North India.

Coxsackievirus and Adenovirus Receptor (CAR) Expression in Autopsy Tissues: Organ-Specific Patterns and Clinical Significance.

Coxsackievirus and adenovirus receptor (CAR) homologs have been identified in many species, and their proteins appeared to be highly conserved in evolution. While most of the human studies are about pathological conditions, the animal studies were more about the physiological and developmental functions of receptors. The expression of CAR is developmentally regulated, and its tissue localization is complex.

Role of vitreous cytology in the diagnosis of primary central nervous system lymphoma: A report of an unusual presentation.

Primary central nervous system lymphoma (PCNSL) is a rare subtype of non-Hodgkin lymphoma confined to the brain, spinal cord, meninges, intraocular compartment, and cranial nerves. Intraocular lymphoma (IOL) is a rare subtype of PCNSL. Intravitreal involvement by a PCNSL is an infrequent but potentially fatal event.

Transcription Factor Immunohistochemistry in the Classification of Pituitary Neuroendocrine Tumor/Adenoma: Proposal in a Limited-Resource Setting.

Context.—: Pituitary neuroendocrine tumors/adenomas are common intracranial tumors that require accurate subtyping because each tumor differs in its biologic behavior and response to treatment. Pituitary-specific transcription factors allow for improved lineage identification and diagnosis of newly introduced variants.

Radiation-induced intracranial rhabdomyosarcoma- A rare complication: Report of a case with literature review.

Radiation-induced sarcoma (RIS) of the central nervous system is an uncommon late complication of radiation therapy. We report a case of a 47-year-old male patient who underwent surgery followed by irradiation and chemotherapy with temozolomide for a frontal lobe gliosarcoma and presented 43 months later with a recurrent tumor in the same location with interval growth in the size of the lesion. Histology from surgical resection of the recurrent tumor revealed embryonal rhabdomyosarcoma (RMS).

PERK inhibitor, GSK2606414, ameliorates neuropathological damage, memory and motor functional impairments in cerebral ischemia via PERK/p-eIF2ɑ/ATF4/CHOP signaling.

The protein kinase R-like endoplasmic reticulum kinase/eukaryotic initiation factor 2ɑ (PERK/eIF2α), the branch of unfolded protein response (UPR), is responsible for transient arrest in translation to counter the enhanced levels of misfolded or unfolded proteins in the endoplasmic reticulum (ER) following any acute condition. In neurological disorders, overactivation of PERK-P/eIF2-P signaling, leads to a prolonged decline in global protein synthesis resulting in synaptic failure and neuronal death. Our study has shown, PERK/ATF4/CHOP pathway gets activated following cerebral ischemia in rats.

Immunological aspects of host-pathogen crosstalk in the co-pathogenesis of diabetes and latent tuberculosis.

Introduction: Diabetes is a potent risk factor for the activation of latent tuberculosis and worsens the tuberculosis (TB) treatment outcome. The major reason for mortality and morbidity in diabetic patients is due to their increased susceptibility to TB. Thus, the study was conducted to understand the crosstalk between and its host upon latent tuberculosis infection and under hyperglycemic conditions or diabetes.

Molecular Diagnosis of Encephalitis/Meningoencephalitis Caused by Free-Living Amoebae from a Tertiary Center in India.

Background: Pathogenic free-living amoeba (FLA) such as spp., , and are causative agents of fatal amoebic encephalitis/meningoencephalitis. The diagnosis of such infections is challenging due to a lack of clinical suspicion and expertise in microscopic identification.

Uncommon histopathological features of cytomegalovirus encephalitis and measles inclusion body encephalitis on autopsy in two patients with primary immunodeficiency.

Purpose: To describe the neuropathological findings in two patients with primary immunodeficiency who had fatal viral encephalitis.

Materials And Methods: Severe combined immunodeficiency (SCID) was confirmed in case 1 by genetic testing, while case 2 had features suggestive of combined immunodeficiency; however, whole exome sequencing showed no pathogenic variants. Autopsies were performed in both cases after an informed consent.

Topical Gentamicin 0.1% Promotes Collagen 7 Expression in Recessive Dystrophic Epidermolysis Bullosa.

Background: Currently, there is no cure for epidermolysis bullosa (EB) but few studies have explored the role of aminoglycosides in promoting collagen 7 expression in recessive dystrophic EB (RDEB).

Materials And Methods: Consecutive patients aged >1 year with a confirmed diagnosis of dystrophic EB (DEB) were advised to apply 0.1% w/w gentamicin cream in a collagen base (Derbriment G™) twice daily on a representative area on right lower limb (RLL) and paraffin gauze dressings on the corresponding opposite side on the left lower limb (LLL).

Phosphorylation of β-catenin at Serine552 correlates with invasion and recurrence of non-functioning pituitary neuroendocrine tumours.

Non-functioning pituitary tumours (NF-PitNETs) are common intracranial benign neoplasms that can exhibit aggressive behaviour by invading neighbouring structures and, in some cases, have multiple recurrences. Despite resulting in severe co-morbidities, no predictive biomarkers of recurrence have been identified for NF-PitNETs. In this study we have used high-throughput mass spectrometry-based analysis to examine the phosphorylation pattern of different subsets of NF-PitNETs.

Bullous pemphigoid in India: Review of cases registered in an autoimmune bullous disease clinic.

Background Information on bullous pemphigoid in an Indian context is scarce. Aim To report clinico-demographic profile, associated comorbidities and prescription pattern of bullous pemphigoid patients in India. Methods This was a retrospective study, where past records of all bullous pemphigoid patients diagnosed and treated between November 2013 and October 2019 were accessed and analysed.

Imaging CXCR4 receptors expression for staging multiple myeloma by using Ga-Pentixafor PET/CT: comparison with F-FDG PET/CT.

Objectives: Ga-Pentixafor positron emission tomography (PET) imaging targets CXCR4 expression which is overexpressed in multiple myeloma (MM). In this study, we evaluated the diagnostic utility of Ga-Pentixafor PET/CT for imaging CXCR4 expression in MM and compared results with F-fluorodeoxyglucose (F-FDG) PET/CT.

Methods: 34 (21M; 13F; median age = 57.