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1.
Lab Invest ; 2022 Jan 14.
Artigo em Inglês | MEDLINE | ID: mdl-35031693

RESUMO

Pleomorphic xanthoastrocytomas (PXAs) are rare tumors accounting for less than 1% of astrocytomas. They commonly occur in young patients and have relatively favorable prognosis. However, they are well known to have heterogenous morphology and biological behavior with the potential to recur and disseminate throughout the central nervous system, especially their anaplastic counterparts. Recent advances in the molecular characterization have discovered BRAFp.V600E mutations in conjunction with CDKN2A/B deletions and TERTp mutations to be the most frequent alterations in PXAs. These tumors can present a diagnostic challenge as they share overlapping histopathological, genomic as well as methylation profile with various other tumor types, particularly epithelioid glioblastomas (eGBs). This review provides the spectrum of evolution of PXAs from their genesis to recent molecular insights and attempts to review pathogenesis and relationship to other tumors that they mimic especially eGB. It is postulated based on evidence from literature that PXA and eGB are possibly related and not distinct entities, being two ends of a continuous spectrum of malignant progression (grade 2-grade 4) with anaplastic PXA (grade 3) lying in between. Future WHO classifications will have to possibly redefine these tumors using more confirmatory data from larger studies.

3.
Epilepsy Behav Rep ; 17: 100516, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34957386

RESUMO

COVID-19 caused devastating effects of human loss and suffering along with disruption in clinical research, forcing reconceptualization and modification of studies. This paper attempts to outline the steps followed and detail the modifications undertaken to deal with the impacts of the pandemic on the first ongoing randomized controlled trial on effectiveness of neuropsychological rehabilitation in adult patients with drug-resistant epilepsy in India. All modifications were based on evolving guidelines and circumstantial context and were planned, reviewed and approved by important stakeholders. Results obtained from the trial need to be interpreted and analysed within this context. These modifications have implications for wider outreach of neuropsychology services in India.

4.
World Neurosurg ; 2021 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-34871801

RESUMO

Subependymal giant cell astrocytoma(SEGA) is a WHO grade1 neoplasm which due to its dubious morphological features, may be misdiagnosed as a high grade tumor at times. This tumor shows binary immuno-expression including both glial and neural markers, leading to a state of diagnostic quandary. Recent evidences have surmised the diagnostic utility of thyroid-transcription-factor1(TTF-1), spurring us to study the practicality of this marker in distinguishing SEGAs from its mimics. In this study, TTF-1 immunohistochemistry using clone 8G7G3/1(1:50) was performed in thirty-eight cases of SEGA, thirty-cases of central neurocytoma, ten-cases each of intraventricular glioblastoma and ependymoma along with five-cases of cortical tubers. Additionally, BRAFV600E mutation, a common genetic alteration in pediatric low-grade-glial tumors with neuronal-differentiation was analysed using Ventana immunohistochemistry platform. TTF-1 immunopositivity was seen in all 38 cases(100%) of SEGAs, with twenty-cases(52.6%) showing diffuse(>50% of tumor area) expression while focal(<50%) immunopositivity was seen in eighteen-cases(47.3%). None of the cases demonstrated BRAFV600E immunolabelling. Barring two-cases of neurocytoma(6.6%), all other cases including ependymoma, glioblastoma and cortical tubers were immunonegative for TTF-1. The congruous finding of TTF-1 expression in SEGA and cells of the developing neuroepithelium in the medial ganglionic eminence hint towards a primogenitor cell with neoplastic potential in presence of impelling factors.

5.
Epileptic Disord ; 2021 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-34750091

RESUMO

Different sleep stages exert differential effects on interictal discharges, neural synchrony and seizure threshold. We sought to assess the relationship between localization of the epileptogenic focus and seizure distribution in sleep versus wakefulness among patients with refractory epilepsy. We conducted a retrospective chart review-based study. Video-electroencephalography of patients with refractory epilepsy, planned for resective surgery, were reviewed for seizure localisation and occurrence relative to stage of sleep/wakefulness. Demographic/clinical data, including details of surgery, were also recorded. Bivariate analysis was conducted using the chi-square test for proportions and unpaired t-test/ANOVA to compare the means within groups. We enrolled 175 patients (107 males) with a mean age of 26.1 + 9.8 years (range: 4-53 years). We analysed 1,282 seizures, of which 916 (71.5%) were temporal, 95 (7.4%) frontal, 144 (11.2 %) central/ parietal and 19 (1.5%) arose from the occipital lobe. Temporal lobe onset seizures were more frequent during wakefulness (77.7%) compared to extra-temporal localization (65%) (p<0.0001). Amongst temporal lobe onset seizures, those during wakefulness arose more frequently from the lateral temporal (88.6%) compared to the mesial temporal lobe (75.5%) (p=0.0003). A higher proportion of seizures evolved into secondary generalisation during sleep (23.5%) versus 8.7% during wakefulness (p<0.0001). Our study demonstrates that lobar location of epileptogenic foci is associated with a predilection of seizures to occur, as well as secondarily generalise, during sleep/wakefulness. Seizures with lateral temporal lobe as well as extratemporal lobe onset were more likely to occur during wakefulness. Overall, sleep related seizures were more likely to be of extratemporal lobe onset, though.

6.
Ann Indian Acad Neurol ; 24(4): 586-588, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34728955

RESUMO

Outcomes of pallidal stimulation in KMT2B dystonia have been infrequently reported prospectively. We report the six-month outcomes of bilateral GPi DBS in an Asian Indian patient with early-onset generalized dystonia associated with a novel heterozygous variant in the KMT2B gene.

7.
Ann Indian Acad Neurol ; 24(4): 580-585, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34728954

RESUMO

Introduction: Stroke is the most common cause of epilepsy in the adult population. Post-stroke seizures (PSSs) are classified into early-onset seizures (ES) and late-onset (LS). ES can significantly affect the clinical outcome and occurrence of LS. Methods: We analyzed data from a prospective cohort of acute ischemic stroke patients between June 2018 and May 2020 in a neurology unit at a tertiary hospital. We screened all acute stroke patients and included consecutive patients older than 18 years of age, presenting with acute, first-ever neuroimaging-confirmed ischemic stroke. We excluded patients with a previous stroke, transient ischemic attacks, hemorrhagic stroke, cerebral venous thrombosis, prior history of seizures, or any other epileptogenic comorbidity. ES were classified as spontaneous seizures occurring within 1 week of the stroke. The main outcome assessed was the occurrence of ES. The secondary outcome was to determine predictors of ES and create an ES prediction score. Results: We screened 432 patients; of them, 291 were enrolled. ES occurred in 37 patients (12.7%). Cortical location (OR: 4.2), large artery disease subtype (OR: 2.9), mRS at presentation (OR: 1.4), use of anticoagulants (OR: 2.6), and hypertension (OR: 0.3) were significantly associated with the occurrence of ES. Patients with ES had a statistically significant worse clinical outcome at 3 months follow-up (P = 0.0072). Conclusion: We could formulate an ES prediction tool using the following components: (a) cortical location, (b) large vessel stroke, (c) mRS at admission, (d) anticoagulant use, and (e) presence of hypertension. This tool might help in treating patients at high risk for ES with prophylactic ASD, thereby preventing seizures and their complications.

9.
Cureus ; 13(9): e17770, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34659981

RESUMO

COVID-19 produces pulmonary symptoms in the majority of symptomatic patients. However, demyelinating illnesses of the central and peripheral nervous system are reported in few patients. We report a case of multifocal demyelination involving the peripheral nerves as well as the optic nerve who developed these symptoms while on corticosteroid therapy, which was being given for treating the COVID-19-related inflammatory state. He improved with a short course of steroids. This is the first reported case of multifocal demyelinating neuropathy in relation to COVID-19 to the best of our knowledge. Though there have been reports of COVID-19-associated central nervous system and peripheral nervous system demyelinating illness, whether COVID-19 is causative or just an association is yet to be discerned.

10.
Neuroradiology ; 2021 Oct 14.
Artigo em Inglês | MEDLINE | ID: mdl-34648046

RESUMO

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.

11.
Neurol India ; 69(4): 837-841, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34507398

RESUMO

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.


Assuntos
Epilepsia , Hemimegalencefalia , Hemisferectomia , Criança , Eletroencefalografia , Epilepsia/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética , Resultado do Tratamento
12.
Neurol India ; 69(4): 925-930, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34507414

RESUMO

Background: Screw diameters are currently available based on the literature available for the Western population. No data are available in such a large number of patients for the Indian population. Objective: The aim of this study was to define the average pedicle size available and to determine the angulation for placement of screws for C2 pedicle in the Indian population. We also try to determine the pedicle screw diameter which can be used universally in most Indian patients, considering the pedicle dimensions. Material and Methods: Retrospective morphometric analysis of CT scans of 247 patients (152 M, 95 F) was done to calculate the C2 pedicle width (PW), pedicle height (PH), pedicle length (PL), pedicle transverse angle (PTA) and the craniocaudal angulation (CCA) of the C2 pedicle. Results: Mean PW in females was 5.3 mm and in males it was 5.9 mm. This difference was statistically significant. The mean PH in the study population was 8.9 mm, while the mean PL was 29.7 mm. The mean PTA was 40.0 degrees and the CCA was 28.4 degrees. Conclusion: This is a morphometric analysis of the C2 pedicle which provides information for the surgeons to determine the safe site of entry and trajectory for the screw implantation. We conclude that a screw diameter of 2.7 mm can be safely implanted in a vast majority of the patient population.


Assuntos
Parafusos Pediculares , Vértebras Cervicais , Feminino , Humanos , Masculino , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
13.
Neuropathology ; 41(5): 412-421, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34558124

RESUMO

Ependymoma is a relatively rare glial tumor of the central nervous system that arise from the cells lining the ventricles and central canal of the spinal cord. Ependymosarcoma (ES) is a newly introduced tumor entity of uncertain prognosis characterized by a rare phenomenon of a malignant mesenchymal transition arising within an ependymoma. ESs are surgically challenging tumors for diagnosis and therapy with a high incidence of morbidity and mortality. Here, we report two diagnostically challenging cases of primary ES in a 25-year-old female and a 17-year-old male. Both the cases presented with progressive and sequential neurological deficits over a period of five to eight months, and histological examination revealed a biphasic gliomesenchymal architecture comprised of anaplastic ependymomatous and sarcomatous components. Molecular genetic analysis revealed the presence of type 1 C11orf95:RELA fusion transcript. To date, 22 cases of ES have been reported in the literature, and only one case harbored type 1 C11orf95:RELA fusion transcript.

14.
World Neurosurg ; 2021 Sep 20.
Artigo em Inglês | MEDLINE | ID: mdl-34555573

RESUMO

BACKGROUND: Cervical pedicle screws (CPS) in the subaxial cervical spine (C3-C6) are faced with high incidence of perforating the lateral pedicular cortex endangering the vertebral artery (VA). The present study analyzes the pedicle width (PW) from C3 to C6 and defines the relation of VA with regard to pedicle and transverse foramen (TF) to determine the safety margin in cases of lateral pedicular breach. METHODS: Computed tomography angiograms of 500 patients were retrospectively studied to identify the pedicle width (PW), VA area, TF area, and the lateral pedicle to vertebral artery distance (LPVA). Occupancy ratio (OR; the percentage area of transverse foramen occupied by VA) and safety margin (SM; permissible displacement of VA in TF), along with LPVA were considered to be protective parameters against VA injury if lateral pedicle breach happens. RESULTS: PW was 4.7 mm at C3, 4.83 mm at C4, 5.26 mm at C5 and 5.41 mm at C6. Mean LPVA at different levels was between 0.97 mm and 1.15 mm, OR was around 20% at all levels, and mean SM was between 2.34 mm and 2.92 at various levels. Sex differences were statistically significant for PW but not for LPVA, SM, or OR. CONCLUSIONS: This study gives us an idea about the various parameters in placement of cervical pedicle screw in subaxial cervical spine and the probable reasons there are few vertebral artery injuries despite high incidence of cervical pedicle breach. LPVA, safety margin, and OR may explain why even very significant CPS misplacement does not lead to vascular injury or neurologic deficit.

15.
BMJ Case Rep ; 14(9)2021 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-34580133

RESUMO

Progressive multifocal leukoencephalopathy can complicate the course of a patient with sarcoidosis. Here we present a rare case of a 35-year-old patient with pulmonary sarcoidosis whose course was complicated by progressive multifocal leukoencephalopathy involving the cerebellum. Neuroimaging and cerebrospinal fluid PCR played a crucial role in the diagnosis.


Assuntos
Leucoencefalopatia Multifocal Progressiva , Sarcoidose Pulmonar , Adulto , Cerebelo , Humanos , Leucoencefalopatia Multifocal Progressiva/complicações , Leucoencefalopatia Multifocal Progressiva/diagnóstico , Imageamento por Ressonância Magnética , Neuroimagem , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/diagnóstico por imagem
16.
J Neurol Sci ; 428: 117583, 2021 09 15.
Artigo em Inglês | MEDLINE | ID: mdl-34375915

RESUMO

BACKGROUND: As the health systems around the world struggled to meet the challenges of COVID-19 pandemic, care of many non-COVID emergencies was affected. AIMS: The present study examined differences in the diagnosis, evaluation and management of stroke patients during a defined period in the ongoing pandemic in 2020 when compared to a similar epoch in year 2019. METHODS: The COVID stroke study group (CSSG) India, included 18 stroke centres spread across the country. Data was collected prospectively between February and July 2020 and retrospectively for the same period in 2019. Details of demographics, stroke evaluation, treatment, in-hospital and three months outcomes were collected and compared between these two time points. RESULTS: A total of 2549 patients were seen in both study periods; 1237 patients (48.53%) in 2019 and 1312 (51.47%) in 2020. Although the overall number of stroke patients and rates of thrombolysis were comparable, a significant decline was observed in the month of April 2020, during the initial period of the pandemic and lockdown. Endovascular treatment reduced significantly and longer door to needle and CT to needle times were observed in 2020. Although mortality was higher in 2020, proportion of patients with good outcome were similar in both the study periods. CONCLUSIONS: Although stroke admissions and rates of thrombolysis were comparable, some work flow metrics were delayed, endovascular stroke treatment rates declined and mortality was higher during the pandemic study period. Reorganization of stroke treatment pathways during the pandemic has likely improved the stroke care delivery across the globe.


Assuntos
COVID-19 , Acidente Vascular Cerebral , Controle de Doenças Transmissíveis , Humanos , Índia/epidemiologia , Pandemias , Estudos Retrospectivos , SARS-CoV-2 , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/terapia , Tempo para o Tratamento
18.
Ann Indian Acad Neurol ; 24(2): 204-210, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34220064

RESUMO

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.

19.
Ann Indian Acad Neurol ; 24(2): 186-191, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34220061

RESUMO

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an astrocytopathy with a predilection for the optic nerve, spinal cord, and brainstem. In this ambispective study, we evaluate clinical characteristics, responses to therapy, and disability outcomes in patients with NMOSD. Methods: Patients diagnosed as NMOSD and following up for at least 1 year at a tertiary care center in India were recruited. Patient data were collected ambispectively from January 2012 until December 2018. Results: A total of 106 patients (29M/77F) with NMOSD were evaluated. The mean age of onset was 29 (±11.6) years. About 77 patients (72.64%) were positive for the AQP4 antibody. Age of onset was higher for those presenting with an opticospinal syndrome (34.2 years) as compared to either isolated longitudinally extensive transverse myelitis (LETM) (30 years) or optic neuritis (ON) (25.3 years). The most common syndrome at onset was LETM in 57 patients (53.77%) followed by ON in 31 patients (29.24%). Azathioprine was the most common immunotherapy (83.96%) prescribed followed by rituximab (7.54%) and mycophenolate mofetil (1.88%). There was a significant decrease in the number of relapses post-azathioprine (P < 0.001). Out of 67 patients with ON, 21 (31.34%) had complete recovery while 17 (25.37%) patients had a severe deficit at a 3-month follow-up. Out of 92 patients with a motor deficit, 49 (53.26%) patients had a partial motor deficit at a 6-month follow-up. The severe visual deficit at baseline and female gender predicted poor visual and motor recovery, respectively. Conclusion: This is the largest descriptive study on patients with NMOSD from India. Relapse rates were similar irrespective of the clinical presentation, age, gender, and disease course. Treatment with immunosuppressive treatment significantly affected the disease course.

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