Idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure (ICP) without an identifiable intracranial lesion, yet its underlying pathophysiology remains poorly defined. Emerging evidence suggests that immune-mediated mechanisms may contribute to disease onset and progression. We conducted a prospective case-control study including 100 adult patients with IIH and 100 age- and sex-matched controls at a tertiary care center in India. The study received Institutional Ethics Committee approval (IEC-03/2019-1141) and was registered with the Clinical Trials Registry of India (CTRI/2020/03/023920). Serum and cerebrospinal fluid (CSF) samples were analyzed for key pro- and anti-inflammatory cytokines (IL-2, IL-4, IL-6, IL-10, IL-17A, TNF-α, IFN-γ) using BD™ Cytometric Bead Array technology. Cytokine profiles were evaluated in relation to disease severity, treatment response, and clinical outcomes. Distinct inflammatory alterations were observed in patients with IIH, suggesting a disease-associated immune signature. These findings support a potential role of immune dysregulation in IIH pathogenesis and highlight cytokine profiling as a promising approach for identifying biomarkers of disease activity and prognosis.
Summary: Background: Data of Central Nervous System (CNS) demyelinating disorders from India has been published from limited centres. The Indian Multiple Sclerosis And Allied Demyelinating Disorders Registry and Research Network (IMSRN) is a multicentric database for multiple sclerosis and allied demyelinating disorders in the Indian subcontinent. This study aimed to describe the demographic, clinical, laboratory, treatment, and follow-up details of patients in the IMSRN and summarise the distribution of major disease phenotypes in a real-world cohort. Methods: This was a prospective, observational, registry-based analysis of patients with CNS demyelinating disorders enrolled in the IMSRN between 16 August 2021 and 25 October 2025. Data were collected in a predefined case record form at recruitment and periodically every six months on a secure database. We performed descriptive and comparative analyses, including temporal trends, treatment patterns, and longitudinal follow-up. Findings: As of 25th October, 2025, 4976 patients have been recruited including radiologically isolated syndrome (RIS), 15 (0.30%); clinically isolated syndrome (CIS), 200 (4.02%); multiple sclerosis (MS), 2479 (49.82%); neuromyelitis optica spectrum disorder (NMOSD), 793 (15.94%); myelin oligodendrocyte antibody associated disease (MOGAD), 698 (14.03%); acute disseminated encephalomyelitis (ADEM), 76 (1.53%); chronic relapsing inflammatory optic neuritis (CRION), 34 (0.68%); chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), three (0.06%), and others, 562 (11.29%). The mean (SD) age of the entire cohort at enrolment is 34.62 (12.16) years, at disease onset was 29.12 (11.80) years and median (IQR) disease duration at recruitment is 2.79 (0.51–7.22) years; longest for MS. There were 65.33% females and 34.67% males, with a female-to-male ratio of 1.9:1; highest in NMOSD (3.58:1). Most patients are from urban areas and educated. Optic nerve and spinal cord involvement were the dominant areas at first presentation. The median (IQR) number of relapses per patient over follow-up was 2 (1–3) highest in the NMOSD population; dominated by optic nerve and spinal cord symptoms. The median (IQR) EDSS was 2 (1–4.5). Rituximab was the commonest disease modifying therapy (DMT) used. Longitudinal trends reflect improvement in time-to-diagnosis and significant shift in the pattern of DMT use in MS towards oral DMTs and B-cell inhibitors. Interpretation: The paper describes a cohort of patients with MS and allied disorders. The key disease characteristics of the MS population seem similar to those reported in international MS registries. The data adds to the existing literature to ascertain disease patterns, response to treatment, and long-term outcome. Funding: The IMSRN registry is funded by the Indian Council of Medical Research (ICMR) vide Grant number 5/4-5/192/NeuroTF/2019-NCD-1.
OBJECTIVE:Cavernous sinus syndrome (CSS) has diverse causes and correctly identifying the underlying pathology is difficult. The study aimed to determine the incremental value of whole-body 18 F-fluorodeoxyglucose (FDG)-PET/computed tomography (CT) in detecting extra-cranial involvement in CSS, to guide biopsy sites; and characterizing cavernous sinus pathologies based on metabolic activity. METHODS:Participants with treatment-naive CSS after clinical assessment and MRI brain were recruited prospectively from July 2022 to December 2023. All the participants underwent whole-body 18 F-FDG-PET/CT, and images were analyzed for the presence, site, extent, and standardized uptake value (SUV max ) and feasibility of biopsy from cavernous sinus and extracranial lesions. Reference standards were histopathology, definite MRI findings, or established diagnostic criteria for immunoglobulin G 4 -related disease, neurosarcoidosis and Tolosa-Hunt syndrome. RESULTS:Data of 54 patients (mean age: 44.8 years) was analyzed. Cavernous sinus lesion was detected in all 54 patients, and 26 (48.1%) patients had extra-cranial lesions. PET-directed biopsies were done from the local extension of FDG-avid cavernous sinus lesions in 20 (37%) and extra-cranial lesions in 12 (22.2%) patients. The final etiological diagnoses were infection in 18 (33.3%), inflammatory disorders in 18 (33.3%), benign neoplasms in 11 (20.5%), and malignancy in seven (12.9%) cases. PET/CT demonstrated significantly lower FDG-avidity in benign cavernous sinus neoplasms (median SUV max : 5.5, IQR: 4.5-8.0) as compared to the infective, inflammatory, and malignant conditions involving the cavernous sinus (median: 15.0, IQR: 10.7-22.4; P < 0.001). Bony erosions and contiguous paranasal involvement were more frequently associated with infective CSS ( P < 0.001). CONCLUSION:Whole-body 18 F-FDG-PET/CT effectively identifies extracranial disease in CSS, enables less invasive biopsy site selection, and enhances diagnostic yield while minimizing high-risk intracranial biopsies. 18 F-FDG-PET/CT should be considered in imaging of CSS, especially when definite diagnosis is not established with conventional workup.
Abstract Sarcoidosis is a multisystem granulomatous disorder that often mimics inflammatory, demyelinating, or neoplastic neuro-ophthalmic conditions. We describe a 39-year-old woman with two episodes of progressive visual loss in the right eye over 6 months, with partial responsiveness to steroid. Examination revealed optic disc edema and mild proptosis. Extensive evaluation for demyelinating, autoimmune, infectious, and neoplastic causes was unremarkable. A diagnosis of optic nerve sheath meningioma was initially made at a peripheral center based on the presence of a “tram-track” appearance along the optic nerve sheath on imaging. However, associated orbital fat stranding, lacrimal gland enlargement, and pachymeningeal enhancement prompted further systemic evaluation, which revealed mediastinal lymphadenopathy and omental deposits. Biopsy demonstrated noncaseating granulomas with Schaumann bodies, confirming sarcoidosis after exclusion of alternative granulomatous disorders, including tuberculosis. The patient was treated with immunosuppressive therapy. This case highlights that atypical features, responsiveness to steroid, and extraneural imaging findings should prompt systemic evaluation to enable timely diagnosis and appropriate immunosuppressive therapy.
OBJECTIVE:To evaluate prescription patterns, referral reasons, patient profiles, diagnostic accuracy, treatment adequacy, and satisfaction in headache patients referred to tertiary care centre. METHODS:A prospective observational hospital-based study was conducted from March 2019 to May 2023. A detailed clinician-administered questionnaire assessed headache characteristics, medication adherence, depression, and anxiety. Referral slips and previous medical records were reviewed. All patients were clinically assessed by two neurologists, provided appropriate treatment, and followed up after 8 weeks. RESULTS:A total of 274 referred patients were included (mean age 32.1 ± 7.8 years; 74.8% female). Treatment non-response (67.9%) was the most common cause of referral. Analgesic was prescribed in 82.8% of patients and migraine prophylactics were used in 27.4%. Primary care physicians showed low discernment for diagnosis of migraines and classification-based headache subtypes. Triptan and beta-blocker use was uncommon. 8 Follow up showed significant reduction in severity and impact of headache and improved medication adherence. CONCLUSION:Primary care management of headache showed overuse of analgesics and less discernment for ICHD-3 based classification. Referred patients were mostly chronic daily headache patients with significant pill burden and other neuropsychiatric comorbidities.
Background & objectives Sudden death, defined as death occurring within one hour of symptom onset in witnessed cases or within 24 h of last being seen alive in unwitnessed cases, remains a major public health concern. This study aimed to evaluate the incidence, causes, and risk factors associated with sudden death in young adults. Methods A cross-sectional study was conducted over one year at a tertiary care centre in New Delhi. Cases meeting the definition of sudden death were included, excluding trauma, suicide, homicide, and drug abuse. Each case underwent whole-body imaging, autopsy, and histopathological examination. A multidisciplinary team comprising forensic experts, pathologists, radiologists, and clinicians determined the cause of death. Comparative analysis was performed between sudden death in young adults (18-45 yr) and older adults (46-65 yr). Results Out of 2214 autopsies, 180 cases (8.1%) met the criteria for sudden death. Sudden death in young accounted for 103 (57.2%) cases. Mean age was 33.6 yr in sudden death in young (IQR=10) and 53.8 years in sudden death in old, with male predominance in both. Cardiovascular causes were most common in young (n=40, 42.6%), followed by respiratory causes (n=20, 21.3%) and sudden unexplained deaths (n=20, 21.3%), where no pathology was identified. Other causes included gastrointestinal, central nervous system, and genitourinary pathologies. Smoking and alcohol intake was equally prevalent in those who died young and at old age. Interpretation & conclusions Sudden death in young adults is a significant concern requiring targeted public health strategies. Coronary artery disease remains the leading cause. Respiratory and unexplained deaths warrant further investigation.
Purpose of the Study:This study compared striatal dopamine metabolism and cerebral glucose metabolism in Parkinson's disease patients with and without freezing of gait (FOG) using Fluorodeoxyglucose positron emission tomography (FDG-PET) and F-DOPA PET. We also compared the two groups' cognitive, affective, and autonomic functions. Methods:Patients were divided into two groups: those with and without FOG. All patients underwent neuropsychological evaluation. Subsequently, both groups underwent FDG-PET and FDOPA-PET. The FDOPA PET/computed tomography images evaluated dopaminergic expression in the basal ganglia. Cortex-ID-based processing of FDG images was visually analyzed for any abnormal hypermetabolism or hypometabolism in the various regions of the brain. Results:A total of 30 patients were recruited, 15 in each group. The mean age of participants was 59.33 ± 9.12 years in the freezing of the gait-negative group and 61.13 ± 9.38 years in the freezing of the gait-positive group. FDOPA PET scan analysis revealed symmetrical loss of basal ganglia uptake in the freezers compared to the nonfreezers, which had more asymmetrically reduced uptake (P = 0.025). In FDG PET, a general trend of greater metabolic reduction in the basal ganglia was observed in freezers. Our study showed bilateral involvement of frontoparietal cortices in both freezers and nonfreezers, with more widespread areas of hypometabolism reported in the freezer group. Conclusion:Freezers exhibited a more symmetrical loss of dopaminergic uptake on FDOPA PET, whereas nonfreezers showed greater asymmetry. In addition, FDG PET revealed a trend toward greater basal ganglia hypometabolism in freezers, suggesting a more profound striatal dysfunction in patients with freezing of gait.
Non-motor symptoms, including depression, anxiety, sleep disturbances, pain and cognitive dysfunction, are a much more important predictor of quality of life than the severity of dystonia. To assess the effect of Botulinum toxin on non-motor symptoms and quality of life in patients with adult-onset idiopathic focal dystonia. Patients aged > 18 years diagnosed with idiopathic focal dystonia were recruited in this longitudinal cohort study. The severity of dystonia, non-motor symptoms, and quality of life were evaluated using the BFMDRS, DNMSQuest, and EQ-5D at baseline and 1 and 3 months after botulinum toxin. 65 patients were recruited with a median age of 59 years. Blepharospasm was the most common phenomenology. 49.2
BACKGROUND:Primary central nervous system lymphoma (PCNSL) is an aggressive form of non-Hodgkin's lymphoma primarily restricted to the brain, spinal cord, eyes, and leptomeninges without any evidence of systemic spread. It is often challenging to diagnose PCNSL due to its clinical and radiological overlap with various central nervous system (CNS) inflammatory conditions and other brain tumors. Cytokines and chemokines in cerebrospinal fluid (CSF) have shown potential as biomarkers for PCNSL, but the conclusion on their diagnostic utility is unavailable. This study intended to assess the role of CSF cytokines and chemokines in differentiating PCNSL from other CNS disorders. METHODS:We searched electronic databases extensively to find studies that examined the sensitivity and specificity of CSF cytokine or chemokine levels in PCNSL-synthesizing results involved standardizing the units of measurement where necessary and verifying data accuracy. Pooled effect estimates were computed by meta-analyses utilizing random-effects models. RESULTS:A total of 12 studies were included. The increased levels of CSF interleukin (IL)-10 and C-X-C motif Chemokine Ligant 13 (CXCL 13) were significantly associated with a positive diagnosis of PCNSL. Diagnostic accuracy measures demonstrated the promising discriminatory power of CSF IL-10 and IL-10:IL-6 ratio. CONCLUSION:Our findings revealed considerable diversity in the diagnostic accuracy of individual biomarkers. Notably, CSF IL-10 and CSF IL-10:IL-6 ratios exhibited the highest pooled specificity and sensitivity, suggesting their promising potential as a diagnostic marker for PCNSL.
BACKGROUND:Parkinson's disease (PD) phenotype may vary with genetic, ethno-geographic, cultural, and environmental factors. OBJECTIVES:The aim was to develop a clinical database of PD in India and assess the influence of age-at-onset (AAO), gender, and motor subtype on the clinical profile of PD. METHODS:A cross-sectional study of PD was conducted across 18 Indian hospitals. Standardized assessments were performed by movement disorder specialists. Data were collected using uniform questionnaires during the recruitment visit. A total of 3300 age- and gender-matched case-control pairs were analyzed for environmental exposures, habits, and co-morbidities. RESULTS:We recruited 7918 PD cases with a mean AAO of 54.2 ± 11.8 years and a median disease duration of 5 years (interquartile range: 2-9). Subgroup analyses based on AAO, gender, and motor phenotype revealed significant differences in motor and non-motor symptoms, exposures, habits, and co-morbidities. Except coffee consumption, previously known associations were observed for exposure to insecticides/pesticides/fungicides (odds ratio [OR]: 1.67), head injury (OR: 3.11), coffee consumption (OR: 1.73), diabetes (OR: 1.48), hypertension (OR: 1.73), and smoking (OR: 0.74) in the Indian population. CONCLUSIONS:This large pan-Indian study highlights the clinical characteristics, environmental exposures, habits, and comorbid diseases associated with PD, which were broadly similar to those observed in European populations. The earlier AAO in Indian PD patients suggests a potentially higher genetic risk, warranting further investigation. A nationwide, community-based, epidemiological study is needed to achieve a comprehensive understanding of all risk factors for PD in India and to validate the risk factors identified in this hospital-based study. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
BACKGROUND:Non-motor symptoms have a more significant impact on the quality of life in Parkinson's disease than motor symptoms. OBJECTIVES:To evaluate the frequency of non-motor symptoms in Parkinson's disease and study their impact on quality of life. METHODS:A cross-sectional study was conducted on 100 patients with idiopathic Parkinson's disease. All patients underwent a detailed history and neurological examination, Hoehn and Yahr staging, MDS UPDRS scoring, NMSS scoring, and PDQ-39 scoring to assess their quality of life. RESULTS:All patients presented with at least one non-motor symptom. The most frequently affected non-motor symptom was sleep/fatigue (95%), followed by urinary (79%) and gastrointestinal dysfunction (76%). The total NMSS score significantly correlated with disease severity and quality of life. CONCLUSION:Non-motor symptoms are quite prevalent in patients with Parkinson's disease and significantly impact their quality of life.