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Background and Objectives: Early initiation and maintenance of anticoagulation appears to be the mainstay of treatment of cerebral venous thrombosis (CVT), but the evidence supporting the intensity and duration of anticoagulation is limited. Methods: We retrospectively collected data of patients admitted with CVT over a 5-year period, who had a minimum of 6 months of clinical follow-up and three or more prothrombin time international normalized ratio (INR) values spread over 6 months. Data collected included demographic, clinical, and radiologic parameters, anticoagulation status during the follow-up, complications, and clinical status at the last follow-up. Results: We identified 204 patients, and the mean age was 34.4 ± 11.1 years. The majority had a provoked etiology (194, 95.1%) for CVT. After initial anticoagulation with unfractionated heparin, all patients transitioned to acenocoumarol or warfarin and this was maintained for a mean duration of 16.02 ± 11.2 months. Time in therapeutic range of INR 2–3 was only 5.1 ± 11.8 percent days and time spent in an INR of 1–1.5 was 68.7 ± 31.8 percent days. The average INR over 6 months was 1.37 ± 0.33. Duration of follow-up was 18.9 ± 13.25 months, and a good outcome was noted in 183 (89.7%) patients. Complications were seen in 29 (14.2%) patients. Multivariate analysis showed only the CVT grading scale score to be an independent predictor of good outcome. Conclusions: Maintenance of an intensive level of anticoagulation may not be required in patients with CVT and may be particularly true when a transient and treatable risk factor is the provoking etiology.

Objectives:. To assess the effect of gait training with robotic exoskeleton-assisted rehabilitation systems on gait parameters, balance, and overall disability in motor incomplete myelopathy. Design:. Prospective pre-post study. Setting:. Neurological rehabilitation unit in a tertiary university teaching hospital. Participants:. Thirty-four motor incomplete myelopathy patients [22 males, mean age 36.7 y (12.7)] were included in this prospective, pre-post study. Twenty-two had nontraumatic etiology, and 17 had tetraplegia. American Spinal Injury Association impairment scale suggested 23 as C and 11 as American Spinal Injury Association impairment scale-D. The mean duration of the lesion was 333 days. Intervention:. Twenty-four sessions of robotic gait training (1-h session, 5–6 sessions/wk) for 4–6 weeks. Outcome measures:. Gait speed was assessed using a 10-meter walk test (10MWT), endurance with a 2-minute walk test (2MWT) and 6-minute walk test (6MWT), walking ability by Walking Index in Spinal Cord Injury-walking index for spinal cord injury II, functionality using Spinal cord Independence Measure-SCIM III, and balance by Timed-up-and-go (TUG) test. Results:. At baseline, 20 patients were nonwalkers-NWB, and 14 were walkers-WB. The walking index for spinal cord injury II and SCIM III suggested significant improvement in both groups by the end of the study (P

Objective: Motor imagery-based brain–computer interface (MI-BCI) is a promising novel mode of stroke rehabilitation. The current study aims to investigate the feasibility of MI-BCI in upper limb rehabilitation of chronic stroke survivors and also to study the early event-related desynchronization after MI-BCI intervention. Methods: Changes in the characteristics of sensorimotor rhythm modulations in response to a short brain–computer interface (BCI) intervention for upper limb rehabilitation of stroke-disabled hand and normal hand were examined. The participants were trained to modulate their brain rhythms through motor imagery or execution during calibration, and they played a virtual marble game during the feedback session, where the movement of the marble was controlled by their sensorimotor rhythm. Results: Ipsilesional and contralesional activities were observed in the brain during the upper limb rehabilitation using BCI intervention. All the participants were able to successfully control the position of the virtual marble using their sensorimotor rhythm. Conclusions: The preliminary results support the feasibility of BCI in upper limb rehabilitation and unveil the capability of MI-BCI as a promising medical intervention. This study provides a strong platform for clinicians to build upon new strategies for stroke rehabilitation by integrating MI-BCI with various therapeutic options to induce neural plasticity and recovery.

Background: Idiopathic intracranial hypertension (IIH) typically manifests with headache, accompanied by papilledema and visual loss, and has a higher prevalence in females. In recent years, ocular sonography, particularly, measurement of optic nerve sheath diameter (ONSD), is being investigated for diagnosis of IIH. Methods: A total of 35 patients over the age of 18 years, fulfilling the modified Dandy's criteria for diagnosis of IIH were included. Patients underwent assessment with magnetic resonance imaging, lumbar puncture, and ocular sonography to measure ONSD and ocular arterial indices. Results: The mean ONSD values (in centimeters) in the right eye of patients with IIH was 0.57 ± 0.13, while it was 0.48 ± 0.03 in controls. In the left eye, the mean ONSD value (cm) was 0.59 ± 0.13 in patients with IIH and 0.48 ± 0.03 in controls. ONSD was significantly higher in cases compared to controls (P < 0.001, Welch test). Pulsatility index of the central retinal artery was significantly higher in cases compared to controls (P < 0.001, Welch test). Resistance index of the ophthalmic artery was statistically significant (P < 0.005, Welch test). Receiver operating characteristic curve analysis revealed a cutoff value of 5.1 mm on the right side and 5 mm on the left side had a sensitivity and specificity of more than 80% for IIH diagnosis. Conclusion: Our study provides insights into the utility of optic nerve sheath measurements and arterial indices in the diagnosis of IIH in a South Indian cohort. Further research is needed to fully understand the longitudinal relationship of these parameters and treatment outcomes in IIH.

Background: Deep cerebral venous thrombosis (DCVT) can have long-term functional and cognitive sequelae. Although literature exists on cognitive impairment after arterial stroke, cognitive sequelae after cerebral venous thrombosis (CVT) are much less studied. Methods: Clinical records of 29 patients diagnosed with DCVT were reviewed. The Modified Telephonic Interview for Cognitive Status (TICS-M) was adapted and validated in the regional language (Kannada) and applied to 18 patients with DCVT, at a mean follow-up duration of 5.32 years. Screening for depression was done via telephonic Patient Health Questionnaire-9 (PHQ-9)-Kannada version, and functional status was screened by applying the modified Rankin Scale (mRS). Results: DCVT had a mortality rate of 10.34% due to acute complications. mRS scores of 0–1 were achieved at follow-up in all patients who survived. Receiver operating characteristic (ROC) analysis revealed a cutoff of ≤44.5 (maximum score of 49) for the diagnosis of cognitive impairment via TICS-M (Kannada version) in DCVT patients. Evidence of cognitive dysfunction was seen in eight patients (42.10%), and three patients (16.66%) had evidence of depression. Conclusions: Survivors of acute DCVT can potentially have long-term cognitive sequelae. Screening for cognitive dysfunction, depression, and functional status can be effectively done using telephonically applied scales that are adapted to the local language. Neuropsychological evaluation and early cognitive rehabilitation can be initiated for patients in whom deficits are identified on cognitive screening.

Introduction Microglia exert a crucial role in homeostasis of white matter integrity and several studies highlight the role of microglial dysfunctions in neurodegeneration. Primary microgliopathy are disorders where the pathogenic abnormality of the microglia causes white matter disorder and leads to a neuropsychiatric diseases. Triggering Receptor Expressed on Myeloid Cells (TREM2), TYRO protein tyrosine kinase binding protein (TYROBP), Colony-stimulating factor 1 receptor (CSF1R) are genes implicated in primary microgliopathy. Clinical manifestations of primary microgliopathy are myriad ranging from neuropsychiatric syndrome, motor disability, gait dysfunction, ataxia, pure dementia, frontotemporal dementia, Alzheimer dementia and so on. It becomes imperative to establish diagnosis of microgliopathy masquerading as degenerative dementia, especially with promising therapies on horizon for the same. We aim to describe a case series of subjects with dementia harboring novel genes of primary microgliopathy, along with their clinical, neuropsychological, and cognitive profile and radiological patterns. Methods: The prospective study was conducted in a university referral hospital in South India, as a part of an ongoing clinic-genetic research on Dementia subjects and was approved by the institutional ethics committee. All patients underwent detailed assessment including socio demographic profile, clinical and cognitive assessment, pedigree analysis and comprehensive neurological examination. Subjects consenting for blood sampling underwent genetic testing by Whole exome sequencing (WES). Results: 100 patients of dementia underwent genetic analysis using whole exome sequencing and three pathogenic variants, one each of TREM2, TYROBP and CSF1R and two variants of uncertain significance in CSF1R were identified as cause of primary microgliopathy .TREM 2 and TYROBP presented as frontotemporal syndrome whereas CSF1R presented as frontotemporal syndrome and Alzheimer dementia. Conclusion :WES has widened the spectrum of underlying neuropathology of degenerative dementias and diagnosing primary microglial dysfunction with emerging therapeutic options is of paramount importance. Cases of primary microgliopathy due to Novel mutations in TREM2, TYROBP and CSF1R with phenotype of degenerative dementia are being first time reported from Indian cohort. Our study enriches the spectrum of genetic variants implicated in degenerative dementia, and provides the basis for exploring complex molecular mechanisms like microglial dysfunction, as underlying cause for neurodegeneration.

Abstract Objective To observe the effect of overground gait training with ‘Mobility Assisted Robotic System-MARS’ on gait parameters in patients with stroke. Patients & methods This prospective pre-post study was conducted in a tertiary teaching research hospital with 29 adult stroke patients, with age up to 65 years. Patients fulfilling the inclusion criteria were divided in 2 groups based on the duration of stroke (≤ 6 months-sub-acute & > 6 months-chronic stroke) and provided overground gait training with MARS robot for 12 sessions (1 h/session) over a period of 2–3 weeks. Primary outcome measures were; 10-Meter walk test-10MWT, 6-min’ walk test-6MWT and Timed up & Go-TUG tests. Secondary outcome measures were Functional Ambulation Category-FAC, Modified Rankin Scale-MRS and Scandinavian Stroke Scale-SSS. Results No adverse events were reported. Twenty-five patients who were able to perform 10-MWT at the beginning of study were included in the final analysis with 12 in sub-acute and 13 in chronic stroke group. All primary and secondary outcome measures showed significant improvement in gait parameters at the end of the training (p

Aberrant alterations in any of the two dimensions of consciousness, namely awareness and arousal, can lead to the emergence of disorders of consciousness (DOC). The development of DOC may arise from more severe or targeted lesions in the brain, resulting in widespread functional abnormalities. However, when it comes to classifying patients with disorders of consciousness, particularly utilizing resting-state electroencephalogram (EEG) signals through machine learning methods, several challenges surface. The non-stationarity and intricacy of EEG data present obstacles in understanding neuronal activities and achieving precise classification. To address these challenges, this study proposes variational mode decomposition (VMD) of EEG before feature extraction along with machine learning models. By decomposing preprocessed EEG signals into specified modes using VMD, features such as sample entropy, spectral entropy, kurtosis, and skewness are extracted across these modes. The study compares the performance of the features extracted from VMD-based approach with the frequency band-based approach and also the approach with features extracted from raw-EEG. The classification process involves binary classification between unresponsive wakefulness syndrome (UWS) and the minimally conscious state (MCS), as well as multi-class classification (coma vs. UWS vs. MCS). Kruskal-Wallis test was applied to determine the statistical significance of the features and features with a significance of p < 0.05 were chosen for a second round of classification experiments. Results indicate that the VMD-based features outperform the features of other two approaches, with the ensemble bagged tree (EBT) achieving the highest accuracy of 80.5% for multi-class classification (the best in the literature) and 86.7% for binary classification. This approach underscores the potential of integrating advanced signal processing techniques and machine learning in improving the classification of patients with disorders of consciousness, thereby enhancing patient care and facilitating informed treatment decision-making.

Background and Purpose: Cerebral venous thrombosis (CVT) presenting as vision loss is uncommon. Raised intracranial tension in CVT is proposed as one of the mechanisms (13.2%). There are still unknown underlying mechanisms to explain vision loss in CVT. The safety and outcome of the surgery (optic nerve sheath fenestration [ONSF] or theco-peritoneal shunt [TPS]) to reduce intracranial hypertension and prevent vision loss has not been studied. Methods: A retrospective case record review of CVT patients with impending vision loss who underwent ONSF/TPS from 2007 to 2019 was performed from the stroke registry. All patients had formal neuro-ophthalmological evaluation and documentation of visual acuity, supplemented by visual field assessments by perimetry in a subset of patients. Safety and outcomes were assessed based on vision improvement and adverse effects after the surgery. Results: Among approximately 1400 patients with CVT admitted in the stroke ward over 12 years, surgery for rescuing vision was done in 18. Among these, the males were 6, and the females were 12. The mean age of presentation was 24 (range 18–52 years). All of them had headaches and progressive blurring of vision with papilledema. The number of patients who underwent TPS was 13, ONSF was 1, and both were 4. In the TPS group (26 eyes), vision improved in 15 eyes (57.7%), remained status-quo in 8 eyes (30.7%), and worsened in 3 eyes (11.5%). Four patients underwent both surgeries; three eyes improved, two remained status quo, and three worsened. One patient underwent ONSF, and his vision remained status quo (no perception of light). Three patients (17.6%) of the TPS group had minor complications (low-pressure headache, subdural hygroma), and five (29.4%) had major complications like subdural hemorrhage, abdominal wound infection, and meningitis. Conclusion and Implications: In patients with CVT, adequate vision monitoring is mandatory. Shunt surgeries (especially TPS) may help in stabilizing/improving vision in CVT patients with impending vision loss, despite adequate anti-edema measures (53.8% improved). Early diagnosis and precise decisions in referring for surgery are crucial.

Diagnosis and treatment of cerebral venous sinus thrombosis (CVT) associated with subdural hematoma (SDH) is challenging with an increased risk of rebleeding on using anticoagulation. There are no guidelines at present due to its rare presentation. In this report we describe three patients who presented with non-traumatic SDH and CVT over the last 3 years. Clinical assessment, investigations including neuroimaging, and management were reviewed both at time of admission and follow-up. These patients presented with varied CVT syndromes -isolated raised intracranial pressure (ICP), focal and diffuse encephalopathy. Neuroimaging helped in diagnosing CVT and SDH. Cases 1 and 3 had SDH alone, while case 2 had SDH along with intraparenchymal hemorrhage. Management of these patients was tailored individually as per mechanism of CVT. Case 1 was clinically stable, however, she had rebleeding after starting anticoagulation, requiring its discontinuation. Cases 2 and 3 underwent immediate neurosurgical intervention in view of deteriorated sensorium. Although CVT manifesting as SDH is rare, clinicians should have a high index of suspicion to accurately diagnose and manage these challenging cases. The decision regarding use of anticoagulation and apt time for neurosurgical intervention needs to be individualized depending on patients condition and response to treatment.

Posterior Cortical Atrophy (PCA) and Logopenic progressive aphasia (LPA) are nonamnestic variants of Alzheimer's disease (AD). Language deficits are a hallmark of LPA but not PCA. Studies have revealed the presence of language deficits in PCA similar to LPA, however it has not been a consistent observation. Only alexia and agraphia are the language deficits listed in the latest consensus criteria for classification of PCA. In this case report we present the clinical, cognitive-linguistic, and neuroimaging features of a patient presenting with an unusual overlap of LPA-PCA. Although the diagnostic characteristics for LPA is fulfilled, the probable progression of the disease to exhibit most of the characteristics similar to PCA is highlighted. Thus, it contributes to the notion of a continuum between the two atypical variants of AD. The possibility of patients with PCA to exhibit language deficits with the progression of the disease similar to that of LPA that primarily involves the phonological short-term memory deficits is emphasized. The need to not undermine the language deficits irrespective of the stage of the disease in PCA is weighed upon for a better identification and management via speech-language intervention programs.

Objectives: Frontotemporal dementia (FTD) syndromes are a complex group of disorders characterised by profound changes in behaviour and cognition. Many of the observed behavioural abnormalities are now recognised to be due to impaired social cognition. While deficits in emotion recognition and empathy are well-recognised in behavioural-variant (Bv)FTD, limited information exists about the nature of social cognitive impairment in the language variant primary progressive aphasia (PPA) that includes progressive non-fluent aphasia (PNFA) and semantic dementia (SD), and in the motor variants FTD amyotrophic lateral sclerosis (FTD-ALS) and FTD progressive supranuclear palsy (FTD-PSP). This prospective study sought to explore the nature and profile of social cognition deficits across the spectrum of FTD. Methods: Sixty patients on the FTD spectrum, i.e., classical (16 with BvFTD and 20 with PPA) and overlap FTD syndromes (13 with FTD-ALS and 11 with FTD-PSP) were evaluated by means of the social cognition tasks, the Interpersonal Reactivity Index (IRI) for empathy, and pictures of facial affect (POFA) for emotion recognition. General cognition and behaviour were also assessed. Results: A significant impairment in emotion recognition and empathy was detected in both the classical and overlap FTD syndromes. The recognition of positive emotions was relatively preserved compared to that of negative emotions. Among the FTD subtypes, maximal impairment of empathy was demonstrated in FTD-PSP. Conclusion: Social cognition impairment is pervasive across the spectrum of FTD disorders, and tests of emotion recognition and empathy are clinically useful to identify the nature of behavioural problems in both classical and overlap FTD. Our findings also have implications for understanding the neural basis of social cognition in FTD.

Objectives: The growing prevalence of dementia, especially in low- and middle-income countries (LMICs), has raised the need for a unified cognitive screening tool that can aid its early detection. The linguistically and educationally diverse population in India contributes to challenges in diagnosis. The present study aimed to assess the validity and diagnostic accuracy of the Indian Council of Medical Research-Neurocognitive Toolbox (ICMR-NCTB), a comprehensive neuropsychological test battery adapted in five languages, for the diagnosis of dementia.Methods: A multidisciplinary group of experts developed the ICMR-NCTB based on reviewing the existing tools and incorporation of culturally appropriate modifications. The finalized tests of the major cognitive domains of attention, executive functions, memory, language, and visuospatial skills were then adapted and translated into five Indian languages: Hindi, Bengali, Telugu, Kannada, and Malayalam. Three hundred fifty-four participants were recruited, including 222 controls and 132 dementia patients. The sensitivity and specificity of the adapted tests were established for the diagnosis of dementia.Results: A significant difference in the mean (median) performance scores between healthy controls and patients with dementia was observed on all tests of ICMR-NCTB. The area under the curve for majority of the tests included in the ICMR-NCTB ranged from 0.73 to 1.00, and the sensitivity and specificity of the ICMR-NCTB tests ranged from 70 to 100% and 70.7 to 100%, respectively, to identify dementia across all five languages.Conclusions: The ICMR-NCTB is a valid instrument to diagnose dementia across five Indian languages, with good diagnostic accuracy. The toolbox was effective in overcoming the challenge of linguistic diversity. The study has wide implications to address the problem of a high disease burden and low diagnostic rate of dementia in LMICs like India.

Background: Aphasia is a common consequence of stroke. To optimize recovery, it becomes critical as there are early identification and treatment of language deficits. The rising burden of stroke aphasia and lack of screening tools in the Indian context necessitates the need for a screening tool. Objective: We aimed to adapt and validate the Frenchay Aphasia Screening Test (FAST) to the Indian context in two widely spoken Indian languages, Telugu and Kannada, for the literate and illiterate population. Methods: A systematic process of adaptation and culturally appropriate modifications of the original FAST were done in 116 healthy controls and 115 patients. The validity of the adapted test was established. Results: The optimum cut-off values for detecting aphasia in our sample ranged from 25 to 25.5 (literate) and 13.5 to 15.5 (illiterate) with high sensitivity and specificity. There was also a significant correlation between aphasia scores for adapted FAST and the Western Aphasia Battery (WAB), establishing good convergent validity. Discussion: Results of the adaptation and validation of two Indian versions of FAST, suggest that it is an easy-to-use screening measure for detecting stroke-related language disabilities. The psychometric properties of the Indian version of FAST met the standardised requirements for adaptation and validation. Conclusions: The Indian version of FAST was found to be a reliable and valid bedside screening tool for aphasia in stroke patients. We aim that this study will facilitate the use of the test across other Indian languages and a large clinical population in the future.

Introduction: In Guillain Barre syndrome (GBS), worsening of weakness or disability after initial period of recovery or stabilization is described as treatment-related fluctuations (TRF). Aim: This study aims to describe the clinical characteristics and outcome of six patients with GBS and TRF. Patients and Methods: Six patients with GBS fulfilling NINCDS criteria, evaluated at a tertiary care university hospital during 2008–2017, were diagnosed to have TRF. They form the basis of this report. Results: All patients were men and their mean age was 40 years. At presentation, mean duration of illness was 15 days; the illness had plateaued in three and progressive in other three patients. Two of the four patients had variant GBS. Initially, five patients were treated with large volume plasmapheresis (LVPP) and one patient with methyl prednisolone. At 17–28 days after disease onset, three patients developed new neurologic deficits (bilateral facial paresis in two; paralytic ileus in one). Other three patients with worsening of limb weakness (medical research council sum score of >5) and disability (Hughes disability grade by ≥1) fulfilled Kleyweg's criteria for TRF. All the six patients were treated with the completion of five cycles or additional cycles of LVPP. Conclusion: Awareness about TRF is essential for correct diagnosis and management of patients with GBS.

Disorders of lipid metabolism can cause variable clinical presentations, often involving skeletal muscle, alone or together with other tissues. A 19-year-old boy presented with a 2-year history of muscle pain, cramps, exercise intolerance and progressive weakness of proximal lower limbs. Examination revealed skeletal markers of Marfan syndrome in the form of increased arm span compared with height, Kyphoscoliois, moderate pectus excavatum, high arched palate and wrist sign. He also had mild neck flexor weakness and proximal lower limb weakness with areflexia. Pathologic findings revealed lipid-laden fine vacuoles in the muscle fibers. Possibility of carnitine deficiency myopathy was considered and the patient was started on carnitine and Co Q. The patient made remarkable clinical improvement over the next 2 months. This case is reported for rarity of the association of clinical markers of Marfan syndrome and lipid storage myopathy and sparse literature on lipid storage myopathy in the Indian context.