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Primary CNS Vasculitis (PCNSV) is a rare, diverse, and polymorphic CNS blood vessel inflammatory condition. Due to its rarity, clinical variability, heterogeneous imaging results, and lack of definitive laboratory markers, PCNSV diagnosis is challenging. This retrospective cohort analysis identified patients with histological diagnosis of PCNSV. Demographic data, clinical presentation, neuroimaging studies, and histopathologic findings were recorded. We enrolled 56 patients with a positive biopsy of CNS vasculitis. Most patients had cerebral hemisphere or brainstem symptoms. Most brain MRI lesions were bilateral, diffuse discrete to confluent white matter lesions. Frontal lobe lesions predominated, followed by inferior cerebellar lesions. Susceptibility-weighted imaging (SWI) hemorrhages in 96.4% (54/56) of patients, either solitary microhemorrhages or a combination of micro and macrohemorrhages. Contrast-enhanced T1-WIs revealed parenchymal enhancement in 96.3% (52/54 patients). The most prevalent pattern of enhancement observed was dot-linear (87%), followed by nodular (61.1%), perivascular (25.9%), and patchy (16.7%). Venulitis was found in 19 of 20 individuals in cerebral DSA. Hemorrhages in SWI and dot-linear enhancement pattern should be incorporated as MINOR diagnostic criteria to diagnose PCNSV accurately within an appropriate clinical context. Microhemorrhages in SWI and venulitis in DSA, should be regarded as a potential marker for PCNSV., (© 2024. The Author(s).)

Background: Ulnar nerve is frequently involved in mononeuropathies of the upper limb. Ulnar neuropathies have been diagnosed conventionally using clinical and electrophysiological findings. Physicians opt for nerve imaging in patients with ambiguous electrophysiological tests to gain additional information, identify etiology and plan management., Objectives: The aim of this study was to describe the electrophysiological and the magnetic resonance neurography (MRN) findings in patients with nontraumatic ulnar neuropathy., Methods: All consecutive patients with suspected nontraumatic ulnar mononeuropathy were recruited; clinical assessment and electrophysiological studies (EPSs) were done in all. After EPS, patients with localization of lesion along the ulnar nerve underwent MRN., Results: All 39 patients recruited had clinical findings suggestive of ulnar neuropathy; Electrophysiological confirmation was possible in 36/39 (92.30%) patients. Localization of ulnar nerve lesion to elbow and wrist was possible in 27 (75%) and 9 (25%) patients, respectively. MRN was done in 22 patients; a lesion was identified in 19 of 22 (86.36%) ulnar nerves studied. Thickening and hyperintensity in T2 W/short TI inversion recovery images of ulnar nerve at the level of olecranon, suggesting ulnar neuropathy at elbow, was the commonest (8/22) imaging finding., Conclusions: MRN acts as a complimentary tool to EPS for evaluating nontraumatic ulnar neuropathy. By identifying the etiology, MRN is likely to modify the management decision., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: We evaluate the potential utility of F-18 FDG-PET in addition to MRI in the diagnostic work-up of patients with autoimmune epilepsy (AE) and propose the inclusion of functional imaging in the antibody prevalence in epilepsy (APE) scoring system., Methods: This was a retrospective analysis in 60 patients, diagnosed and treated for AE, of whom 40 were antibody negative (presumed AE) and 20 were antibody positive (definitive AE). All patients had undergone a dedicated brain and whole body FDG-PET in the department of Nuclear Medicine., Results: In the antibody negative group, MRI supported a diagnosis of AE in 23 patients. Both MRI and PET were indicative in 12 cases, and standalone PET was positive in 8. While MRI alone was diagnostic in 57% (23/40), the combined yield of both modalities was 77% (31/40). When PET scores were added to assign the APE score in MRI negative cases, average APE score was 5.4. In the antibody positive group, MRI supported the diagnosis of AE in 7 patients. Both MRI and PET were positive in 4 patients and standalone PET was positive in 5 patients. While MRI alone was diagnostic in 35% (7/20), the combined yield of both modalities was 60% (12/20). When PET scores were added to assign the APE score in MRI negative cases, average APE score was 6.1., Conclusion: The inclusion of metabolic information from PET distinctly improved (the sensitivity of) APE scores to predict autoimmune origin even in antibody negative cases. A larger prospective study of similar type could justify adoption of FDG-PET into the standard diagnostic procedure., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objectives: Posttherapy changes in treated glioma patients cannot be reliably differentiated from tumor recurrence. We evaluated the role of Tc-methionine SPECT/CT for the detection of recurrent glioma and compared the same with F-FDG PET/CT and contrast-enhanced MRI (CeMRI)., Methods: Forty-four patients with histologically proven, previously treated glioma and clinical suspicion of recurrence were prospectively enrolled in the study. Of these 44 patients, 39 (28 male and 11 female subjects; age, 38.05 ± 9.7 years) underwent Tc-methionine SPECT/CT, F-FDG PET/CT, and CeMRI of the brain and were included for final analysis. Combination of repeat imaging, biopsy, and/or clinical follow-up (6-36 months) was taken as reference standard. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were calculated. Diagnostic values among modalities were compared., Results: Positive predictive value and negative predictive value for Tc-methionine SPECT/CT, F-FDG PET/CT, and CeMRI were 95.6% and 56.2%, 92.3% and 61.5%, and 79.4% and 42.9%, respectively. Sensitivity and specificity for the 3 modalities were 75.9% and 90%, 82.8% and 80%, and 87.1% and 30%. Specificity of Tc-methionine SPECT/CT was significantly higher than that of CeMRI (P < 0.0001) but not of F-FDG PET/CT (P = 0.36). No significant difference was seen between the modalities for sensitivity and accuracy., Conclusions: Tc-methionine is a promising tracer for detection of recurrent glioma. Diagnostic values of Tc-methionine SPECT/CT are similar to F-FDG, although it is more specific than CeMRI. So it may be used as a cost-effective alternative and also where PET/CT is not available.

Motivated by recent interest in identification of functional brain networks, we develop a new multivariate approach for functional brain network identification and name it as Multivariate Vector Regression-based Connectivity (MVRC). The proposed MVRC method regresses time series of all regions to those of other regions simultaneously and estimates pairwise association between two regions with consideration of influence of other regions and builds the adjacency matrix. Next, modularity method is applied on the adjacency matrix to detect communities or functional brain networks. We compare the proposed MVRC method with existing methods ranging from simple Pearson correlation to advanced Multivariate Adaptive Sparse Representation (ASR) methods. Experimental results on simulated and real fMRI dataset demonstrate that MVRC is able to extract functional brain networks that are consistent with the literature. Also, the proposed MVRC method is 650-750 times faster compared to the existing ASR method on 90 node network., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Till date there are no randomized trials to suggest the superiority of intra-operative magnetic resonance imaging (IOMRI) guided trans-sphenoidal pituitary resection over two dimensional fluoroscopic (2D-F) guided resections. We conducted this trial to establish the superiority of IOMRI in pituitary surgery. Primary objective was to compare extent of tumor resection between the two study arms. It was a prospective, randomized, outcome assessor and statistician blinded, two arm (A: IOMRI, n=25 and B: 2D-F, n=25), parallel group clinical trial. 4 patients from IOMRI group cross-over to 2D-F group and were consequently analyzed in latter group, based on modified intent to treat method. A total of 50 patients were enrolled till completion of trial (n=25 in each study arm). Demographic profile and baseline parameters were comparable among the two arms (p>0.05) except for higher number of endoscopic procedures and experienced neurosurgeons (>10years) in arm B (p=0.02, 0.002 respectively). Extent of resection was similar in both study arms (A, 94.9% vs B, 93.6%; p=0.78), despite adjusting for experience of operating surgeon and use of microscope/endoscope for surgical resection. We observed that use of IOMRI helped optimize the extent of resection in 5/20 patients (25%) for pituitary tumor resection in-group A. Present study failed to observe superiorty of IOMRI over conventional 2D-F guided resection in pituitary macroadenoma surgery. By use of this technology, younger surgeons could validate their results intra-operatively and hence could increase EOR without causing any increase in complications., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Purpose: High-resolution magnetic resonance imaging (MRI) of intracranial parts of sixth nerve and seventh nerve and the extraocular muscles (EOMs) in orbit to correlate the clinical characteristics in patients with two special forms of strabismus in congenital cranial dysinnervation disorders which are Duane's retraction syndrome (DRS) and Mobius syndrome., Materials and Methods: Morphological analysis by 3T MRI of orbit (using surface coils) and brain (using 32 channel head coil) was performed on 6 patients with clinical DRS (1 bilateral), 2 cases with Mobius syndrome, and 1 case with congenital sixth nerve palsy. These were compared with findings in five controls., Results: We observed absence/hypoplasia of sixth nerve in five out of seven eyes with DRS (71.42%), anomalous course in one eye, sixth and seventh nerve absence/hypoplasia in affected eyes with Mobius syndrome and bilateral absence/hypoplasia of the sixth nerve in congenital sixth nerve palsy. For EOMs we calculated maximum diameter, area, and circumference of muscles using Osirix software, and noticed significant hypoplasia of lateral rectus in comparison to controls (P < 0.001)., Conclusions: MRI gives useful information regarding confirmation of clinical diagnosis and its neurological anomalies in complex cases and helps to plan tailor made surgical management.

We report our initial experience to optimize neurosurgical procedures using high field strength intraoperative magnetic resonance imaging (IOMRI) in 300 consecutive patients as high field strength IOMRI rapidly becomes the standard of care for neurosurgical procedures. Three sequential groups (groups A, B, C; n=100 each) were compared with respect to time management, complications and technical difficulties to assess improvement in these parameters with experience. We observed a reduction in the number of technical difficulties (p<0.001), time to induction (p<0.001) and total anesthesia time (p=0.007) in sequential groups. IOMRI was performed for neuronavigation guidance (n=252) and intraoperative validation of extent of resection (EOR; n=67). Performing IOMRI increased the EOR over and beyond the primary surgical attempt in 20.5% (29/141) and 18% (11/61) of patients undergoing glioma and pituitary surgery, respectively. Overall, EOR improved in 59.7% of patients undergoing IOMRI (40/67). Intraoperative tractography and real time navigation using re-uploaded IOMRI images (accounting for brain shift) helps in intraoperative planning to reduce complications. IOMRI is an asset to neurosurgeons, helping to augment the EOR, especially in glioma and pituitary surgery, with no significant increase in morbidity to the patient., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Purpose: To determine the impact of intraoperative magnetic resonance imaging (iMRI) in epilepsy surgeries on the extent of surgical resection and seizure outcome along with its feasibility and limitations., Methods: Patients with pharmacoresistant epilepsy (PRE), who underwent surgeries in operating theater equipped with high-field 1.5-Tesla MRI, were evaluated for extent of resection, operative time, scanning time, pathologies, resultant extra resection, and seizure outcomes., Results: Thirty-nine patients with mean age of 18 (range: 3-65) years with PRE underwent surgical intervention. Mean duration of epilepsy was 10.2 years. Surgical interventions included tumor resection (31%), resection of focal cortical dysplasia (28%), mesial temporal lobe surgeries (18%), and disconnection surgeries (23%). iMRI alone, apart from navigation and electrophysiology, improved resection rates in 13% (5 out of 39) of these patients. In lesional group, iMRI modified operative strategy resulting in increased resections in 21% (5/23) patients. Complete resection was observed in 87% of patients. iMRI scanning time constituted 25% (mean: 72 ± 21 min) of time spent under anesthesia by the patient. Major and minor complications were observed in 2.5% and 7.5% of patients, respectively. The mean follow-up was 14 months. Favorable postoperative seizure control (Engel Classes I and II) was achieved in 85% and complete seizure freedom was achieved in 77% of patients (Engel Class IA) at 1-year follow-up., Conclusions: iMRI increases the extent of resection mainly in lesional epilepsy surgeries translating into good seizure outcomes but not found to be much beneficial in prototype mesial temporal sclerosis surgeries and disconnection surgeries.

Objective: To objectively mark out abnormal areas of magnetic resonance imaging (MRI), positron emission tomography (PET), and electrocorticography (ECoG) using neuronavigation so as to 1) enhance the accuracy of margins of the epileptogenic zone and 2) understand the relationships of all the three modalities with each other., Methods: A prospective study was conducted of 37 patients with intractable epilepsy due to lesional, neocortical pathologies from noneloquent areas. Prior to surgery, fusion and transfer of MRI and PET images onto a neuronavigation system was performed. At surgery, this was correlated to intraoperative ECoG using the electrode as referential points. An objective score was created for every electrode point that was correlated with MRI and PET abnormality at the point. The extent of surgical resection was mapped out using these data., Results: From a total of the data recorded from 1280 electrode points, 23.5% were located over the lesion. In addition, over the lesions, 93% of PET and 66% of ECoG points were abnormal. Over the perilesional areas, 43% of PET and 45% of ECoG points were abnormal. Using these data for surgery, both lesional and perileisonal areas were resected; 33/37 patients had good outcome (25 Engel I, 8 Engel II) (mean follow-up: 23.6 ± 3.2 months; range 18-31 months)., Conclusion: Multimodal imaging and ECoG using this method seems to provide a better objective localization of the epileptogenic foci., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Neurocysticercosis, involvement of the central nervous system by taenia solium, is one of the most common parasitic diseases worldwide. However, isolated spinal involvement by neurocysticercosis, either intramedullary or extramedullary, is uncommon. The authors report a case of C1-C2 intradural extramedullary neurocysticercosis in a young male that presented initially with signs of raised intracranial pressure followed by high cervical myelopathy. Transventricular migration of the neurocysticercal cyst along the cerebrospinal pathways leading to clinical pleomorphism as described by the authors can perplex the best of clinicians leading to a missed diagnosis as in the present case. Diagnosis was confirmed after surgical excision and the patient is doing well at six months follow up with no neurological deficits. Spinal neurocysticercosis should be considered in the differential diagnosis in high-risk populations with new symptoms suggestive of a spinal mass lesion.

Ewing's sarcomas (ES) account for approximately 10% of primary malignant bone tumors. Primary Ewing's sarcoma of the skull is a very rare entity. We report a case of Ewing's sarcoma of the sphenoid bone in a 5-year-old boy. CT scan showed heterogeneously enhancing mass with associated spiculated new bone formation along the posterolateral wall of the orbit. Magnetic resonance (MR) imaging showed markedly heterogeneously signal intensity mass lesion containing areas of hemorrhage and fluid-fluid levels. Spiculated new bone formation and fluid-fluid levels had not been reported previously in skull Ewing's sarcomas and may be helpful features for preoperative diagnosis.

We report a case of surgically proven giant neurocysticercosis (NCC). MR imaging revealed an unusually large solitary parenchymal cystic lesion showing signal intensity similar to CSF on all pulse sequences, with internal septations and a small nodule in the anterior aspect of this lesion compatible with this diagnosis. Identification of a scolex in a cystic lesion with CSF intensity plays a key role in the diagnosis of NCC. The presence of internal septations is an atypical feature.

We report three cases of spinal angiolipoma, two intramedullary and one epidural, and review their MRI features. Diagnoses were established preoperatively in all cases. Specific MRI features including the importance of postgadolinium fat-saturated T1-weighted images and presence of vascular flow-voids are discussed with histopathological correlation. A characteristic morphological appearance of intramedullary angiolipoma has been suggested.

Background: Primary intracranial Ewing's sarcoma (ES) is a type of primitive neuroectodermal tumour and is a rare malignant tumour in children and adolescents. The imaging features of ES overlap with other central nervous system embryonal tumours, making it difficult to pinpoint a specific diagnosis. We aim to explore the clinical, neuroimaging and differential diagnoses of this entity. Case presentation: We describe a 6-month-old infant who presented with complaints of enlarging the head size and poor feeding. Imaging revealed a contrast-enhancing large solid-cystic mass lesion with internal calcification, focal bone erosion and haemorrhage in the posterior fossa. Histopathological examinations, immunohistochemistry, and molecular analysis confirmed ES. Conclusions: The confirmative diagnosis of primary intracranial ES requires histological examination, immunohistochemical analysis, and genetic detection, along with radiological findings. Surgical excision followed by combined radiotherapy and chemotherapy is the treatment of choice. [ABSTRACT FROM AUTHOR]

Glial fibrillary acidic protein (GFAP) astrocytopathy is a rare autoimmune inflammatory disorder affecting the central nervous system, involving the meninges, brain parenchyma, and spinal cord. The distinctive radiologic feature observed on magnetic resonance imaging (MRI) is characterized by periventricular radial and linear contrast enhancement. This case report details a 45-year-old male who initially exhibited constitutional symptoms, followed by encephalitis, lower limb weakness, and urinary retention. The MRI findings revealed meningoencephalitis with longitudinal extensive myelitis. Notably, the cerebrospinal fluid analysis confirmed the presence of anti-GFAP antibodies. [ABSTRACT FROM AUTHOR]

Medicine has many vividly named signs. We have compiled a list of radiological cerebral signs inspired by phenomena in outer space. These range from the well- known 'starry sky' appearance of neurocysticercosis or tuberculomas, to various lesser known signs including the 'starfield' pattern of fat embolism; 'sunburst' sign of meningiomas; 'eclipse' sign of neurosarcoidosis; 'comet tail' sign of cerebral metastases; 'Milky Way' sign of progressive multifocal leukoencephalopathy; 'satellite' and 'black hole' sign of intracranial haemorrhage; 'crescent' sign of arterial dissection and 'crescent moon' sign of Hirayama disease. [ABSTRACT FROM AUTHOR]

Background: Desmoid fibromatosis (DF) is an atypical, non-malignant tumor known for its distinctive feature of exhibiting an intermediate biological behavior that falls between benign fibrous lesions and fibrosarcoma. The article aimed to outline the imaging characteristics of desmoid fibromatosis and distinguish it from its similar counterpart, nerve sheath tumors. Results: Here, we present two cases of desmoid fibromatosis whose imaging findings mimic nerve sheath tumors. The patients presented with neck swelling and radiating pain. Both cases were biopsy-proven. Imaging features characteristic of these lesions include elongated shape, split-fat sign, and fascial tail sign with mixed signal intensity on T1WIs and T2WIs depending upon the collagen content of the lesion. Conclusions: MRI is the best diagnostic modality for differentiating fibromatosis from nerve sheath tumors. Surgery continues to be the primary approach for treating these growths, but in some cases, chemoradiation is employed to achieve improved control rates. The presence of intralesional low-intensity bands in T2WIs is a characteristic feature of fibromatosis. Given its local aggressiveness and propensity for recurrence, the use of multiple imaging modalities is essential for providing informed guidance on outcomes and treatment strategies. [ABSTRACT FROM AUTHOR]

This article discusses a case study of a 12-year-old boy with THAP1-associated dystonia, a condition that mimics neurodegeneration with brain iron accumulation (NBIA). The boy presented with childhood onset, generalized dystonia that progressively worsened over time. Neurological examination revealed dystonia involving various body regions, along with bradykinesia and rigidity. Magnetic resonance imaging (MRI) showed hypointensity in the bilateral globus pallidi, a finding typically associated with NBIA. However, genetic testing revealed a pathogenic mutation in the THAP1 gene, confirming the diagnosis of DYT6 dystonia. Treatment with levodopa and other medications resulted in mild improvement. This case adds to the evidence that DYT-THAP1 should be considered as a mimic of NBIA. [Extracted from the article]

Keywords: anti-Zic4; ataxia; magnetic resonance imaging EN anti-Zic4 ataxia magnetic resonance imaging 1128 1131 4 11/07/22 20221101 NES 221101 Imaging characteristics of paraneoplastic neurological syndromes frequently mimic common pathologies like infections or demyelinating disorders (primary/secondary). A combination of these MRI findings can be a marker for anti-Zic4 associated neurological disorder. Salt and Pepper Appearance of Brain-Stem and Cerebellum in Anti-Zic4 Associated Neurological Disorder. [Extracted from the article]

Hemidystonia, unilateral limb choreoathetosis, unilateral limb tremor, faciobrachial dystonia, and myoclonus have been reported in the literature. Keywords: Rasmussen's encephalitis; Hemichorea; Movement disorder EN Rasmussen's encephalitis Hemichorea Movement disorder 542 545 4 05/13/22 20220501 NES 220501 Movement disorders in Rasmussen's encephalitis (RE) are rarely described. Hyperkinetic movement disorders except myoclonus3 have been rarely described in RE because the more predominant presentation of seizures overshadows subtle movement disorders; however, a thorough understanding of movement disorders in RE is important because these may be the only presentations of a patient with RE. [Extracted from the article]

Purpose: We planned this prospective study to evaluate PSMA expression in recurrent high-grade gliomas (rHGG), including anaplastic astrocytoma and glioblastoma using Glu-NH-CO-NH-Lys-(Ahx)-[Ga-68 (HBED-CC)]- (Ga-68 PSMA) positron emission tomography (PET), with its theranostic potential in mind. Methods: This was a prospective study enrolling patients with clinical and MRI evidence of rHGG on follow-up. Three treated cases of HGG with RN on MRI were also included as negative controls. Abnormal tracer accumulation in the brain parenchyma, more than the contralateral hemisphere was interpreted as positive study. For semiquantitative analysis, a 3D spherical region of interest (ROI) was drawn around the site of the abnormal Ga-68 PSMA uptake, and the ratio of SUVmax of tumor (T) to SUVmax of the contralateral corresponding area (TBR) was calculated. Each patients' PSMA brain PET was fused to the corresponding MRI and reviewed for concordance. Results: Thirty patients were included in the study, a total of 49 lesions were detected on MRI, and fused PET/MR images showed increased Ga-68 PSMA uptake in all these lesions. Multifocal lesions were better appreciated on fused PET-MR images, and concordance between MRI and PET was 100 % for patient and lesion-wise detection. Recurrent glioma lesions showed SUVmax and SUVmean values (median and IQR) 6.0 (4.4–8.2) and 3.3 (2.8–3.7), respectively. Lesions labeled as radiation necrosis on MRI did not show tracer accumulation. Conclusion: Ga-68 PSMA has potential utility for evaluating recurrence in HGG and its potential for theranostics would encourage its use in the evaluation of these patients. [ABSTRACT FROM AUTHOR]

The article presents radiological observations in four patients with genetically proven mitochondrial membrane protein-associated neurodegeneration (MPAN). Topics include the characteristics of MPAN such as progressive spasticity and gait abnormalities, the prominent spasticity observed in MPAN patients, and the novel radiological indicator of MPAN.

The article reports that Myelin oligodendrocyte glycoprotein‑associated disorder (MOGAD) is an autoimmune oligodendrocytopathy, whereas neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy. Topics include Cell‑based assay shows strong immunofluorescence to aquaporin and myelin oligodendrocyte protein in the serum tested on transfected cell lines; and enhancement in the medial temporal lobe following contrast administration Coronal FLuid Attenuated Inversion Recovery.

The article presents case studies related to Myelin Oligodendrocyte glycoprotein antibody syndrome and seizures. Topics include a 12‑year‑old girl presented with acute onset unsteadiness and imbalance while walking and diminution of vision from both eyes; and a 13‑year‑old boy presented with recurrent episodes of focal neurological deficits associated with pure motor paraparesis with stiffness of limbs, and horizontal binocular diplopia which were responsive to corticosteroids.

Background: The recovery of the upper-limb (UL) motor function after ischemic stroke (IS) remains a major scientific, clinical, and patient concern and it is hard to predict alone from the clinical symptoms. Objective: To determine the accuracy of the prediction of the recovery of UL motor function in patients with acute ischemic middle cerebral artery (MCA) stroke using individual clinical, transcranial magnetic stimulation (TMS) or diffusion tensor imaging (DTI) parameters or their combination. Methods and Material: The first-ever acute ischemic MCA stroke patients within 7 days of the stroke onset who had an obvious UL motor deficit underwent TMS for the presence of motor-evoked potential (MEP) and DTI to evaluate the integrity of corticospinal tracts. Multivariate logistic regression analysis was done to test for the accuracy of the prediction of the recovery of UL motor function. Results: Twenty-nine acute ischemic MCA stroke patients (21 males and 8 females) with a mean age of 51.45 ± 14.26 years were recruited. Model-I included clinical scales (Fugl-Meyer Assessment [FMA] + Motricity Index [MI]) + TMS (MEP) + DTI (fractional anisotropy [FA]) were found to be the most accurate predictive model, with the overall predictive ability (93.3%; 95% confidence interval [CI]: 0.87-0.99) and sensitivity: 94.9% (95% CI: 0.87-1.0) and specificity: 95.8% (95% CI: 0.89-1.0); respectively. Conclusion: The accuracy of UL motor recovery can be predicted through the clinical battery and their elements as well as TMS (MEP) and DTI (FA) parameters. Further, well-designed prospective studies are needed to confirm our findings. [ABSTRACT FROM AUTHOR]

Multiple cranial nerve palsies have many possible causes, including infective, inflammatory, neoplastic and infiltrative diseases of the meninges or skull base. We present the clinicopathological conference of a 27-year-old man with a smouldering course of sequential cranial nerve palsies. His imaging showed dural thickening and osteosclerosis of the skull base with otomastoiditis. Cerebrospinal fluid showed lymphocytic pleocytosis with reduced glucose and normal protein concentrations. There was a possible response to corticosteroids and anti-tubercular treatment. Initial biopsy from the thickened and enhancing dura was unremarkable. His condition deteriorated after the steroids were tapered; MR imaging showed progression of lesions and positron emission tomography showed intense hypermetabolism. Subsequently, a diagnostic test revealed the final diagnosis. This case was presented at the All India Institute of Medical Sciences' monthly clinicopathological conference series in February 2021. [ABSTRACT FROM AUTHOR]

A case study of a 38‑year‑old gentleman presented with two episodes of sudden onset right hemiparesis in the past 3 years with good recovery along with progressively increasing cognitive impairment. Topics include Cognitive functions were impaired with the pronounced impairment of frontal and temporal lobes on detailed assessment; and computed tomographic (CT) angiography showed multiple fusiform aneurysms in the bilateral distal cervical internal carotid artery.

Background: Hemispheric disconnection represents a challenging and major epilepsy surgical procedure. This procedure in experienced hands offers excellent results in terms of seizure outcomes, especially for hemispheric pathologies such as Rasmussen's encephalitis, hemispheric dysplasias, hemimegalencephaly. The technique of hemispherotomy has witnessed various modifications over the years, beginning from anatomical hemispherectomy to the current era of minimally invasive functional hemispheric disconnections.Objective: This study aimed to describe the technique of performing endoscopic vertical hemispherotomy using interhemispheric corridor developed by the senior author.Materials and Methods: A 12-year-old girl with seizure onset at the age of 10 years presented with an aura of fear and nausea followed by tonic deviation of eyes to the right and blinking with speech arrest. There were tonic-clonic movements of the right-sided limbs along with ictal spitting and occasional deviation of the angle of mouth to the right. The patient had loss of awareness for the event along with postictal confusion lasting few minutes.Results: Video electroencephalography (VEEG) revealed left parietocentral and left temporal localization. Serial magnetic resonance imaging (MRI) brain over 3 years revealed progressive left hemispheric changes suggestive of Rasmussen's encephalitis. The patient underwent left-sided endoscopic hemispherotomy. At 2 years follow-up, the patient is seizure-free (ILAE [International League Against Epilepsy] Class 1).Conclusion: Endoscopic hemispherotomy using the interhemispheric approach is an elegant, minimally invasive, reproducible, safe, and efficacious technique. [ABSTRACT FROM AUTHOR]

Background and Purpose: Cerebral small vessel disease (CSVD) markers have not been widely studied in relation to hematoma volume and growth in hypertensive intracerebral hemorrhage (ICH). The objectives to assess the relationship of white matter hyperintense lesions (WMHL), microbleeds (MBs), and cortical siderosis (CSS) with hematoma volume, hematoma expansion (HE), and 3 months outcome in patients with hypertensive ICH. Methods: All consecutive acute hypertensive supratentorial ICH presenting to the emergency were prospectively recruited. Baseline and 24 hours computed tomography (CT) to assess hematoma volume and magnetic resonance imaging (MRI) for CSVD markers were performed in all subjects. WMHL (graded using Fazekas's scale), MBs, and CSS were assessed and compared with baseline variables and outcomes. All the images were assessed by an experienced stroke neurologist/neuroradiologist. Results: One hundred and fifty-seven patients were screened and 60 were included. Mean age was 54.08 ± 11.57 years and 47 (78%) were males. Of 60, 19 (28.1%) had HE, 31 (51.6%) had major bleed (>30 ml), and 28 (47.46%) had poor 3 month outcome (mRS 4-6). On univariate analysis, high grade WMHL was associated with greater HE [odds ratio (OR): 2.65, confidence interval (CI) 1.48--4.72, P = 0.001), greater proportion with volume >30 ml (OR: 7.16, CI: 1.09--47.13, P = 0.001) and poor outcome (OR: 2.1, CI: 0.05--3.27, P = 0.001). MBs were associated with poor outcome (P = 0.029) but not with HE/volume. CSS was related to HE (P = 0.031), a large volume bleed (P = 0.023), and poor outcome (P = 0.021). On multivariate model, only WMHL independently predicted HE (P = 0.034), greater proportion with bleed volume >30 ml (P = 0.041), and poor outcome (P = 0.042). Conclusions: WMHL in MRI serves as a predictor of hematoma expansion, a large volume bleed, and poor outcome in hypertensive ICH and may be incorporated into existing prediction models. [ABSTRACT FROM AUTHOR]

A case study of a 44‑year‑old woman presented with cerebral venous sinus thrombosis presenting with features of raised intracranial tension and plaque meningeal thickening. Topics include Deep vein thrombosis and isolated cortical vein thrombosis are unusual findings of cerebral venous thrombosis; and Radiological finding of venous thrombosis includes visualization of thrombus, irregular sinus lumen manifesting partial obliteration.

A case study of a 50‑year‑old lady presented to the emergency with one day history of multiple simple partial seizures involving right arm and face without any motor deficit or loss of consciousness. Topics include neoplastic etiology was suspected and anti‑edema medicines including steroids, anti-epileptic drugs and other supportive measures; and contrast‑enhanced imaging revealed a well‑defined multi‑lobulated lesion in subcortical white matter of left posterior frontal region.

A case study of a 22‑year‑old woman who developed acute onset altered behavior in the post‑partum state and was initially misdiagnosed to have cortical vein thrombosis. Topics include Electroencephalogram (EEG) revealed periodic sharp wave complexes synchronized with the jerks; and Considering a possibility of Acute Disseminated Encephalomyelitis (ADEM) she was pulsed with steroids and discharged.

A case study of a 15‑year‑old boy presented with complaints of intellectual decline and behavioral change for last 8 months due to Cobalamin C Disease. Topics include developed concomitant stiffness of both lower limbs with transient tremulousness of feet on standing from a recumbent position; and considering the child's age, presentation with cognitive dysfunction and spastic ataxic paraparesis, a positive family history.

A case study of a 28‑year‑old male, born out of non‑consanguineous marriage from North‑West India belonging to Aggarwal community, presented with history of recurrent neurologic deficits since the last 14 years. Topics include autosomal recessive monogenic vasculitic syndrome caused by loss of function homozygous or compound heterozygous mutations; and deficiency of adenosine deaminase‑2 (DADA‑2) is recently described as an autoimmune disease caused by mutations.

Background: The management of hypothalamic hamartomas (HH) rests upon the type of presentation. These are rare congenital benign lesions presenting either with central precocious puberty (CPP), drug refractory epilepsy (DRE) or combination of both. We present here our experience in the management of these lesions from a neurosurgeon's perspective and review the pertinent literature.Objective: To present a series of HH presenting with CPP and DRE managed in the neurosurgery department at our center with an emphasis on the associated endocrine abnormalities.Materials and Methods: A prospective observational study over a period of five years included 16 patients of HH. All patients were evaluated with 3 Tesla Magnetic Resonance Imaging (MRI) brain, complete hormonal workup including gonadotrophins, testosterone (males) and estradiol (females), and video-electroencephalography (VEEG) as a part of epilepsy workup. All these patients were evaluated with postoperative hormonal workup and repeat MRI brain if repeat surgery was contemplated.Results: Among the 16 patients of HH, there were 11 male and 6 female children. All the patients presented with DRE with four of these had associated CPP. All the patients underwent robotic-guided radiofrequency ablation (RFA), with 75% seizure freedom following 1st RFA surgery. Three of the four patients with CPP achieved both clinical and biochemical normalization. One patient had just a marginal reduction in the serum gonadotrophins. One patient was reoperated twice and three underwent RFA thrice.Conclusion: The management of HH should be individualized with DRE taking the precedence requiring early surgery. A multidisciplinary approach is therefore recommended for a successful outcome. [ABSTRACT FROM AUTHOR]

We present the clinicopathologic conference of a 34-year-old lady with history of facial palsy 14 years ago who developed new deficits of mononeuritis multiplex, maculopapular rash, pancytopenia, splenomegaly, lung involvement and cognitive decline rapidly over three years. Investigations revealed pancytopenia, reversal of albumin globulin ratio, mediastinal adenopathy, ANA positivity, low C3 levels with the CSF being inflammatory and MRI showing extensive hemorrhagic lesions with mass effect. She had a rapidly progressive fatal course over three years with the disease being undiagnosed. This case was presented in the annual meeting of the Indian Academy of Neurology in September 2018. [ABSTRACT FROM AUTHOR]

Keywords: frontotemporal dementia; amyotrophic lateral sclerosis; SQSTM1; Sequestosome; p62 EN frontotemporal dementia amyotrophic lateral sclerosis SQSTM1 Sequestosome p62 800 802 3 07/21/21 20210701 NES 210701 A 43-year-old Asian Indian male presented with progressive gait difficulty for 3 years. Cerebellar Ataxia in Adults with SQSTM1-Associated Frontotemporal Dementia-Amyotrophic Lateral Sclerosis Spectrum of Disorders This is classified as a pathogenic variant associated with the phenotype of frontotemporal dementia (FTD)-ALS (OMIM*616437).1 This variant was also observed in his elder brother (currently asymptomatic) and younger sister (having bilateral lower limb hyperreflexia). [Extracted from the article]

A case study of a 42‑year‑old gentleman with prior comorbidities presented with the sub‑acute onset and progressive weakness with slurring of speech with impaired comprehension. Topics include Whole‑body positron emission tomography scan revealed areas of hypometabolism in the left fronto‑parietal and right parieto‑occipital region; and multiple well‑defined hyperintense lesions in the subcortical and deep white matter of both cerebral hemispheres with peripheral foci of satellite lesions.

Aim: To establish normative measurements of pituitary gland in Indian population. Material and Methods: In this cross-sectional study, we measured dimensions of pituitary gland in 482 (213 females and 269 males) Indian subjects with apparently normal pituitary gland function. Mid-sagittal T1-weighted image (T1-WI) on magnetic resonance imaging (MRI) was used to measure height and length of pituitary gland. Pituitary gland width was measured using coronal T1-WI and pituitary gland volume was calculated. Results: Mean height, length and calculated volume of pituitary gland was significantly higher in females compared to males (p = <0.001, P = 0.03 and P = <0.001, respectively) when all age groups were combined but pituitary gland width was not statistically different in male and female subjects. When subjects were divided into different age groups, except for 10–14 years age group where pituitary height was significantly higher in females as compared to male, no significant difference was observed between male and female in any of the parameters (height, length, width and volume). The mean pituitary gland height was 5.80 ± 1.32 mm and 5.37 ± 1.25 mm in female and male subjects, respectively. Females achieved peak pituitary gland height in 10 to 14-year age group, while males achieved their peak pituitary gland height in 15 to 19-year age group. Conclusion: Our study provides age and sex wise normative data for pituitary measurements derived from Indian population. [ABSTRACT FROM AUTHOR]

The article presents a case study related to 45-year-old lady has presented to our hospital with recurrent episodes of tingling in the right hand for the last 3 years. Topics inlcude the cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) being an autosomal dominant, and the case of CADASIL presenting with recurrent seizures evidence of cerebral cavernous malformations (CCMs) put forward a common pathophysiological association.

The article presents a case study of a 29-year-old female with complaints of vertigo. Topics include hyperintensity with an incomplete horseshoe pattern of enhancement where demyelination possibility keeping ahead of brainstem glioma; and rare involvement of the corpus callosum, the brainstem, or the spinal cord.

Objective: Resection of large Vestibular Schwannomas (VSs) can be associated with postoperative facial nerve injury. Diffusion-based tractography has emerged as a powerful tool for three-dimensional imaging and reconstruction of white matter fibers; however, tractography of the cranial nerves has not been well studied. In this prospective study, we aim to predict the position of facial nerve in large VSs (>3 cm) using Diffusion Tensor Imaging (DTI) tractography and correlate it with the intraoperative finding of the position of facial nerve.Materials and Methods: Twenty patients with a large VS (>3 cm) undergoing surgery were subjected to preoperative DTI to predict the position of the facial nerve in relation to the tumor. The surgeon was blinded to the results of the preoperative DTI tractography. A comparative analysis was then made during operation. The location of the facial nerve in relation to the tumor was recorded during surgery using facial nerve stimulator.Results: Of the 20 patients who underwent DTI tractography, it was not possible to preoperatively identify facial nerve in one patient. In another patient, although DTI tractography predicted the position of facial nerve, it was not identified intraoperatively. In the remaining 18 patients, DTI tractography accurately predicted the facial nerve position. The predicted position was in synchronization with the intraoperative facial nerve position in 16 patients (89% concordance). It was discordant in two patients (11%), but this was not found to be statistically significant (P = -0.3679).Conclusion: This study validates the reliability of facial nerve DTI-based fiber tracking for prediction of the facial nerve position in patients with large VSs. The reliable preoperative visualization of facial nerve location in relation to the VS will allow surgeons to plan tumor removal accordingly and may increase the safety of surgery. [ABSTRACT FROM AUTHOR]

The article presents a case study of 48‑year old female patient Spinal cysticercosis considered as neurocysticercosis. Topics discussed include analysis of multifocal cerebrospinal fluid (CSF) lesions through magnetic resonance imaging (MRI) in the patient; provision of corticosteroids towards the patient for treatment approaches; and recommendation for usage of albendazole in treatment conditions.

Objective. To effectively combine functional MRI (fMRI), diffusion tensor tractography (both guided by neuronavigation) along with cortical stimulation (CS) for surgery of eloquent cortex (EC) lesions. Materials and methods. Fifteen patients with lesions adjacent to the eloquent motor and sensory cortex were included. Preoperative fMRI and diffusion tensor imaging were performed and then integrated into the neuronavigation system. Intraoperative CS of sensory/motor cortex was performed to localize the EC under awake condition and this was correlated with areas active on fMRI utilizing neuronavigation. For excision of the deeper structures, CS, and tractography guided by neuronavigation were utilized. Results. A total of 127 cortical sites were evaluated with CS in 15 patients. The overall sensitivity, specificity, and accuracy of fMRI were 79%, 85%, and 82%, respectively, keeping the areas positive on CS as a referential parameter. Tractography helped in resecting the deeper areas of the tumor, but was not very accurate due to brain shift. However, it was useful in roughly assessing the deeper areas close to the long tracts. The risk of developing persistent neurological deficits was 6%. Pathologies included gliomas in ten patients, cavernous malformation in two patients, meningioma in one patient, and focal cortical dysplasia and Dysembryonic neuroepithelial tumor in one patient each. Near total excision was achieved in 7/10 (> 95% excision) gliomas and a total excision in all others lesions. Conclusions. Lesions directly over the EC present a special surgical challenge. The challenge lies in excising these lesions without producing any deficits. These goals may be achieved better by combined use of multimodal neuronavigation (fMRI and tractography) and intraoperative mapping with CS under awake conditions. [ABSTRACT FROM AUTHOR]

Astrocytomas of Conus-Cauda equina region are rare. Astrocytomas, which are intramedullary tumors, may rarely have an extramedullary component. However, primary intradural extramedullary astrocytomas are extremely rare, with very few cases reported in the literature. We describe a giant extramedullary pilocytic astrocytoma of Cauda equina in a 20-year-old male. To the best of our knowledge, this is the first report of such a case in the available literature. This case highlights the fact that astrocytomas can be primarily extramedullary and emphasizes the need to consider pilocytic astrocytoma in the differential diagnosis of cystic Cauda equina tumors. [ABSTRACT FROM AUTHOR]

Objectives: To review clinical characteristics and response to immunomodulation therapy in autoimmune encephalitis presenting with status epilepticus (SE), epilepsy, and cognitive decline. Design: Observational, prospective case series. Setting: All India Institute of Medical Sciences, New Delhi, India. Materials and Methods: Prospective analysis of 15 patients, who presented with SE, epilepsy, cognitive decline, and other neurological symptoms with positive autoantibodies. Demographic and clinical characteristics were recorded. Brain magnetic resonance imaging (MRI), cerebrospinal-fluid analysis (CSF), and tumor screening were done periodically. Treatment received and responses (categorized as per patients and treating doctor's information) were noted. Results: There were 15 (males = 10) patients of autoimmune encephalitis. The mean age of presentation was 24 years (range: 2-64 years). The most common onset was subacute (64%) and four (29%) patients presented as SE. Predominant clinical presentations were seizures (100%) almost of every semiology. CSF was done in 10 patients; it was normal in 60%. Brain MRI was done in all patients, in six (40%) it was normal, six (40%) showed T2W and FLAIR hyperintensities in bilateral limbic areas. Antibodies found were the N-methyl-D-aspartate receptor antibody in seven (50%), voltage-gated potassium channel antibody in five (36%), two of antiglutamic acid decarboxylase, and one patient with double stranded DNA (dsDNA) antibodies. None showed evidence of malignancy. Patients received immunotherapy, either steroids, intravenous immunoglobulin, or both. Follow-up showed significant improvement in majority of cases, neither further seizures nor relapse in nine (67%) cases. One death occurred, due to delayed presentation. Conclusions: Uncommon but potentially reversible causes of SE, epilepsy, and cognitive decline may be immune-related and high index of suspicion will prevent missing the diagnosis. [ABSTRACT FROM AUTHOR]

A case of visual hallucination, headache and left hemiparesis is reported. The patient had a history of recurrent attacks of similar semiology for the previous 15 years. MRI brain revealed a cortical hyperintensity on T2W, FLAIR and diffusion weighted imaging (DWI) in the right cerebral hemisphere with a normal ADC (apparent diffusion coefficient) map and MR angiogram. Detailed workup for MELAS was negative. A diagnosis of sporadic hemiplegic migraine was made and he was managed conservatively. He made a gradual complete recovery over 2 weeks. He was discharged on flunarizine for prophylaxis and has remained asymptomatic over the ensuing 4 months. This interesting condition is reviewed and discussed herein. [ABSTRACT FROM AUTHOR]