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BACKGROUND: Chronic headache greatly affects the quality of life and also constitutes a significant burden on the health system. OBJECTIVE: The objective of this study was to evaluate the feasibility of telephone-based follow-up in a cohort of headache patients in India. MATERIALS AND METHODS: This was a longitudinal cohort study of patients with episodic headache with one physical visit in the neurology outpatient services in the last year. Two neurologists conducted the telephone follow up (TFU) of included patients 12 weeks apart. We evaluated the following: (1) objective characterization of headache, (2) coexistent depression and anxiety, (3) patient satisfaction, (4) treatment adherence, and (5) changes in medications. RESULTS: A total of 214 out of 274 eligible patients were included in the cohort. The mean age was 31.74 ± 7.77 years (18-45), and 164 (77%) were females. Migraine without aura was the most common diagnosis in 159 (74%). The mean disease duration was 78.01 ± 70.15 months (8-360). Concurrent depression and anxiety were noted in 87 (40.6%) and 45 (21%) of the patients, respectively. There was a significant improvement in the headache frequency (23.82 vs. 1.06, P < 0.001), severity (7.21 vs. 2.62, P = 0.032), and Headache Impact 6-item score (58.12 vs. 38.01, P = 0.014) at baseline and second follow-up. The satisfaction level to TFU in the first and second interviews was 94.4% and 97.2%, respectively. CONCLUSION: Telephone-based follow-up is a feasible alternative for repeat outpatient consultation of headache patients.
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Estudios de Factibilidad , Satisfacción del Paciente , Teléfono , Humanos , Femenino , Masculino , Adulto , Estudios Longitudinales , India/epidemiología , Adolescente , Persona de Mediana Edad , Adulto Joven , Depresión/epidemiología , Ansiedad/epidemiología , Cefalea , Estudios de Seguimiento , Trastornos de Cefalalgia/terapiaRESUMEN
BACKGROUND: The role of thymus in the immune cascade of the body is still under active clinical scrutiny. Meanwhile, there is an unravelling of myriad presentations of thymoma with effects on various organ systems. These effects arise both due to autoimmunity or a paraneoplastic process secondary to thymoma. However, an occasional patient can be a conundrum and may give no clue regarding the pathogenesis and etiology of its clinical profile. MATERIAL AND METHODS: We present an enigmatic case of a 30-year-old male presenting with superficial fungal infections followed by multiaxial neurological involvement against a background of thymoma. Extensive workup for bacterial, viral, autoimmune and paraneoplastic etiologies were negative. Thymectomy confirmed thymoma (Type AB) but failed to ameliorate the progression of symptoms. He developed recurrent episodes of severe sepsis which remitted with sensitive antibiotics only to reappear again. Immunological profile showed complete absence of peripheral circulating B-cells with reversal of CD4/CD8 ratio, findings compatible with Good's syndrome. Radiological findings showed only progressive brain atrophy without any hyperintensity. He was also treated with intravenous immunoglobulins but failed to respond with the same. RESULTS: Autopsy revealed features of panencephalitis (neuronophagia, neuronal loss, perivascular lymphocytic cuffing and microglial nodules) with virus particles detected ultrastructurally. Although the distinction between seronegative autoimmune encephalitis versus a viral encephalomyelitis often blurs histologically, we speculated viral encephalomyelitis to have happened toward the latter part of his long illness. To our knowledge, this is a case of Good's syndrome presenting with seronegative autoimmune panencephalitis superimposed with a viral infection.
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Encefalomielitis , Enfermedades del Sistema Nervioso , Timoma , Neoplasias del Timo , Adulto , Encefalomielitis/complicaciones , Encefalomielitis/tratamiento farmacológico , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Masculino , Enfermedades del Sistema Nervioso/complicaciones , Timectomía , Timoma/complicaciones , Neoplasias del Timo/complicacionesRESUMEN
Temporal lobe epilepsy (TLE) with enlargement of the amygdala (AE) is a distinct clinical entity with contrasting clinical features from TLE with hippocampal sclerosis (HS). The objectives of this systematic analysis were to study the clinical characteristics and treatment outcome of people with TLE with AE. Pubmed, Embase, Cochrane, Web of Science, Scopus, and Medline were searched using the keywords amygdala enlargement, temporal lobe epilepsy, epilepsy, and seizure in November 2020. We found 18 studies that satisfied the inclusion criteria. A total of 361 patients were included in this analysis. The mean age of onset was 36.2 years, and febrile seizure was uncommon compared to TLE with HS subjects. The type of aura and automatism was similar to TLE with HS, though less prevalent. Electroencephalography (EEG) was usually concordant with the side of AE. Anti-seizure medications (ASM), surgical, and immunotherapy were used in different studies. 86 patients underwent surgery with Engel I outcome in 69.7%. Histopathology of the resected samples was predominantly dysplasia and gliosis. A group of patients that responded well to immunotherapy with subsequent reduction of amygdala volume (AMV) purported an autoimmune etiology of AE. Heterogeneity was the main drawback that prevented comparability among the studies. The methods of measurement of AMV also differed widely in the included studies, and standardization of its method is still lacking. This analysis suggests TLE with AE as a distinctive group of patients either due to a developmental anomaly or autoimmune etiology.
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Epilepsia del Lóbulo Temporal , Amígdala del Cerebelo , Electroencefalografía , Epilepsia del Lóbulo Temporal/terapia , Hipocampo , Humanos , Recién Nacido , Imagen por Resonancia Magnética , ConvulsionesRESUMEN
BACKGROUND: In this mini review, we discuss some of the atypical neurological manifestations of dengue virus and attempt to bring them to attention to highlight the neurotropic property of the dengue virus. METHODS: Cases were chosen from retrospective hospital and outpatient records of all patients seropositive for dengue who attended the neurology referral. Seven patients have been chosen as illustrative examples of dengue-associated neurological involvement. We discuss the various central and peripheral nervous system involvement of patients and discuss the relevant findings in them. CONCLUSION: Through this case series, we wish to highlight that the dengue virus can affect the nervous system at various targets, using multiple mechanisms of pathogenesis to generate a plethora of presentations. Hence, it is vital to be aware of its presentations to be able to diagnose dengue and treat it accordingly.
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Virus del Dengue/patogenicidad , Dengue , Enfermedades del Sistema Nervioso , Dengue/diagnóstico , Dengue/fisiopatología , Diagnóstico Diferencial , Humanos , Enfermedades del Sistema Nervioso/diagnóstico , Enfermedades del Sistema Nervioso/virología , Examen Neurológico/métodosRESUMEN
We prospectively evaluated 22 consecutive patients (24 eyes) suffering from non-arteritic anterior ischaemic optic neuropathy (NAION) at a tertiary care centre in Northern India. The mean age was 51.7 years. Visual loss on awakening was noticed only in three (12.5%) eyes. Six (27.3%) patients had headache while three (9.7%) patients had retro-orbital pain at the onset of visual loss. Peripapillary haemorrhages were seen in two (8.3%) eyes. At follow up, only one eye showed improvement in visual acuity. The results of our study suggest that NAION may not always present with the classic clinical picture. Accordingly, a high index of suspicion remains the key to correct diagnosis.
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We aimed to evaluate the effect of sleep quality on memory, executive function, and language performance in patients with refractory focal epilepsy and controlled epilepsy and compare these with healthy individuals. We prospectively enrolled 37 adolescent and adult patients with refractory focal epilepsy (Group 1) and controlled epilepsy (Group 2) in each group. History pertaining to epilepsy and sleep were recorded, and all patients underwent overnight polysomnography. Language, memory, and executive function assessments were done using Western Aphasia Battery, Post Graduate Institute (PGI) memory scale, and battery of four executive function tests (Trail Making Test A & B, Digit symbol test, Stroop Task, and Verbal Fluency Test), respectively. Forty age- and sex-matched controls were also included in the study. Significant differences were noted in both objective and subjective sleep parameters among all the groups. On polysomnography, parameters like total sleep time, sleep efficiency, sleep latency, and rapid eye movement (REM) latency were found to be significantly worse in Group 1 as compared with Group 2. Cognitive and executive parameters were significantly impaired in Group 1. Shorter total sleep time, poorer sleep efficiency, and prolonged sleep latencies were observed to be associated with poor memory and executive function in patients with refractory epilepsy. Our study strongly suggests that sleep disturbances, mainly shorter total sleep time, poor sleep efficiency, and prolonged sleep latencies, are associated with impaired memory and executive function in patients with refractory focal epilepsy and to a lesser extent, among those with medically controlled epilepsy.
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Epilepsia Refractaria/complicaciones , Epilepsias Parciales/complicaciones , Función Ejecutiva/fisiología , Lenguaje , Memoria/fisiología , Trastornos del Sueño-Vigilia/complicaciones , Sueño/fisiología , Adolescente , Adulto , Epilepsia Refractaria/fisiopatología , Epilepsia Refractaria/psicología , Epilepsias Parciales/tratamiento farmacológico , Epilepsias Parciales/fisiopatología , Epilepsias Parciales/psicología , Femenino , Humanos , Masculino , Pruebas Neuropsicológicas , Polisomnografía , Estudios Prospectivos , Trastornos del Sueño-Vigilia/fisiopatología , Trastornos del Sueño-Vigilia/psicología , Prueba de Secuencia Alfanumérica , Adulto JovenRESUMEN
Aspergillus is a rare but important opportunistic pathogen to invade the central nervous system (CNS). It is a significant pathogen of not only immunocompromised but also immunocompetent patients. Its manifestations are pleiotropic, reflecting multiple mechanisms of pathogenesis and host interactions. Despite significant advances in diagnostic methods and therapeutic options, the mortality remains high. Several advances have been made in medications and surgical management, yet, current treatment practices lack uniformity. Patient woes are further heightened by the high costs of treatment and prolonged duration of therapy. In view of the challenging aspects of this disease, we present a short review of four challenging cases touching on the varied aspects of management of CNS aspergillosis covering pathogenesis, diagnostic pitfalls, surgical and medical options and evidence-based guidelines for the management of the same.
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Antifúngicos/uso terapéutico , Aspergillus/patogenicidad , Encéfalo/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Neuroaspergilosis/diagnóstico , Neuroaspergilosis/tratamiento farmacológico , Adulto , Aspergillus/efectos de los fármacos , Encéfalo/microbiología , Encéfalo/patología , Bases de Datos Factuales , Manejo de la Enfermedad , Estudios de Seguimiento , Humanos , Huésped Inmunocomprometido , Masculino , Neuroaspergilosis/epidemiología , Estudios Retrospectivos , Medición de Riesgo , Muestreo , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
The allocation of healthcare resources is reliant upon accurate information generated through clinical coding. Several factors contribute to coding inaccuracies, one of which is interpreting medical documentation. A lack of awareness among medical staff of the clinical coding process and the importance of detailed documentation exacerbates this problem. To investigate this further, 1 month of inpatient clinical coding data from a single hospital ward was reviewed by clinicians experienced in the coding and auditing process. If the reviewing clinician identified inaccuracies in the initial clinical coding, Healthcare Resource Group (HRG) codes were changed. Education sessions were then provided both to junior clinicians working on the hospital ward and to clinical coding staff and a further month of clinical coding data was again reviewed to assess for any difference after the sessions. HRG changes were made in 58.5% of 94 cases initially. Following the educational sessions, 20.5% of HRGs changed in 73 cases (p<0.0001), indicating more accurate initial clinical coding. There were also statistically significant reductions in the extent to which the primary and secondary diagnoses were changed. This study demonstrates that targeted education sessions for both junior clinicians and clinical coding staff can improve the accuracy of inpatient clinical coding.
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BACKGROUND: IIH is a severe form of headache that often has superimposed migraine and often it is very difficult to distinguish the two forms of headache. Intracranial hemodynamics is a relatively unexplored means of distinguishing between the two forms of headache. OBJECTIVES: We aimed to study intracranial flow dynamics using Transcranial Doppler in patients with IIH, migraine, and normal controls. MATERIALS AND METHODS: It was a hospital-based observational study that included 51 people with IIH, 87 people with migraine, and 101 healthy controls and all were subjected to TCD study after detailed clinical examination. RESULTS: Mean age of patients in three groups were similar with the mean age in IIH being 33.41 ± 10.75 (age in years ± SD). Vision loss was present in 66.67% of patients with IIH, and most common field defect was generalized constriction (27.5%). Neuroimaging was abnormal in 94.11% of patients of IIH with mean CSF pressure was 31.27±5.32â¯cm of water. Of all the TCD-measured velocities, mean flow velocity (MFV) showed a significant difference in all three groups with (p-value <0.001). The pulsatility index, both for middle cerebral arteries as well as ophthalmic arteries showed a significant difference in the three groups with the highest values in IIH patients (p-value<.001). The mean VMR in IIH (1.11±0.32) was lower than the mean VMR in migraine (1.34±0.43) as well as controls (1.49±0.46). CONCLUSION: TCD parameters like MFV and PI are useful parameters that show considerable variation and can be used to differentiate between IIH and migraine.
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Trastornos Migrañosos , Ultrasonografía Doppler Transcraneal , Humanos , Ultrasonografía Doppler Transcraneal/métodos , Trastornos Migrañosos/diagnóstico por imagen , Trastornos Migrañosos/fisiopatología , Masculino , Adulto , Femenino , Persona de Mediana Edad , Circulación Cerebrovascular/fisiología , Adulto Joven , Arteria Cerebral Media/diagnóstico por imagen , Arteria Cerebral Media/fisiopatología , Velocidad del Flujo Sanguíneo/fisiologíaRESUMEN
Background: Rheumatoid Arthritis (RA) is a common systemic inflammatory disease that can present with a plethora of extraarticular manifestations. Many patients with RA from low- and middle-income countries do not get timely and adequate treatment with disease-modifying therapies. This results in the perpetuation of a chronic inflammatory state. Focus: Rheumatoid vasculitis (RV) is one of the most aggressive complications of RA resulting from a prolonged proinflammatory milieu. Usually, it has the involvement of multiple organ systems, with cutaneous manifestations being the most common. Neurological presentation is uncommon but severe when present. Highlight: We present a case of severe RV presenting with an unexpected neurological complication consisting of cranial and peripheral neuropathy with small vessel disease and intracerebral haemorrhage. We intend to highlight the morbidity and long-term consequences of inadequately treated RA, the most common inflammatory disease of the connective system especially in light of the neurological presentation.
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Rheumatoid arthritis activity is generally evaluated by using DAS-28 score. But this does not reflect the extent of functional hand impairment, a decisive parameter for patient wellbeing as well as for work disability. Several questionnaires to quantify the hand involvement in RA have been elaborated, amongst which SACRAH has been popular since 2003. But this requires evaluating 23 questions on a visual analogue scale. The questions were reduced to 12 in modified-SACRAH (M-SACRAH) and to only five questions in short form SACRAH (SF-SACRAH) so as to make it easily applicable in daily clinical practice. A study was planned to compare M-SACRAH (already validated) to SF-SACRAH in Indian population as no Indian data are available on the same. A total of 100 patients of RA were evaluated for disease activity using DAS-28 score and hand functions using M-SACRAH and SF-SACRAH. The M-SACRAH and SF-SACRAH were then compared based on DAS-28 scores; also M-SACRAH was compared to SF-SACRAH using Spearman's correlation coefficient. The mean value of DAS-28 score was 3.15 ± 0.86. The mean value of SF-SACRAH was 8.065 ± 7.44, and mean value of M-SACRAH was 201.7 ± 201.1008. The correlation of DAS-28 score to SF-SACRAH and M-SACRAH was significant in moderate and high disease activity but insignificant in remission and low disease activity state. The correlation between M-SACRAH and SF-SACRAH showed a spearman's coefficient of 0.998 with a P value of <0.001 (significant correlation). Correlation was significant for all disease activity states and for remission. The study suggests that the disease activity of rheumatoid arthritis (as assessed by DAS-28 score) has a poor correlation with hand functions (as assessed by M-SACRAH and SF-SACRAH) especially in low disease activity and remission states. Further, M-SACRAH and SF-SACRAH are significantly correlated. Therefore, it is suggested that RA patients should be assessed by SF-SACRAH (which includes five questions only) in addition to DAS-28 scoring for better evaluation of hand functions, a detrimental factor in day to day performance of RA patients.
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Artritis Reumatoide/diagnóstico , Evaluación de la Discapacidad , Articulaciones de la Mano/fisiopatología , Mano/fisiopatología , Adulto , Artritis Reumatoide/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Dimensión del Dolor , Índice de Severidad de la Enfermedad , Encuestas y CuestionariosRESUMEN
Tolosa-Hunt syndrome (THS) remains a challenging diagnosis for many neurologists. Often believed to be a rare presentation, the classical presentation is known to involve cranial nerves and tissues surrounding the cavernous sinus. Traditionally, a diagnosis of THS is considered when all secondary conditions have been ruled out. Yet, newer findings have elaborated a complex pathogenetic process with some overlap from the IgG4 spectrum of disorders, with which it shares many phenotypic similarities. In this narrative review, we present an updated picture of the condition focusing on the latest developments in the pathogenesis, diagnosis, and clinical management of these two conditions and use illustrative examples to highlight the salient features of this rare presentation.
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Background: One of the major challenges is to deliver adequate health care in rural India, where more than two-thirds of India's population lives. There is a severe shortage of specialists in rural areas with one of the world's lowest physician/population ratios. There is only one neurologist per 1.25 million population. Stroke rehabilitation is virtually nonexistent in most district hospitals. Two innovative solutions include training physicians in district hospitals to diagnose and manage acute stroke ('Stroke physician model') and using a low-cost Telestroke model. We will be assessing the efficacy of these models through a cluster-randomized trial with a standard of care database maintained simultaneously in tertiary nodal centers with neurologists. Methods: SMART INDIA is a multicenter, open-label cluster-randomized trial with the hospital as a unit of randomization. The study will include district hospitals from the different states of India. We plan to enroll 22 district hospitals where a general physician manages the emergency without the services of a neurologist. These units (hospitals) will be randomized into either of two interventions using computer-generated random sequences with allocation concealment. Blinding of patients and clinicians will not be possible. The outcome assessment will be conducted by the blinded central adjudication team. The study includes 12 expert centers involved in the Telestroke arm by providing neurologists and telerehabilitation round the clock for attending calls. These centers will also be the training hub for "stroke physicians" where they will be given intensive short-term training for the management of acute stroke. There will be a preintervention data collection (1 month), followed by the intervention model implementation (3 months). Outcomes: The primary outcome will be the composite score (percentage) of performance of acute stroke care bundle assessed at 1 and 3 months after the intervention. The highest score (100%) will be achieved if all the eligible patients receive the standard stroke care bundle. The study will have an open-label extension for 3 more months. Conclusion: SMART INDIA assesses whether the low-cost Telestroke model is superior to the stroke physician model in achieving acute stroke care delivery. The results of this study can be utilized in national programs for stroke and can be a role model for stroke care delivery in low- and middle-Income countries. (CTRI/2021/11/038196).
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Myxomas are the most common cardiac tumors and present clinically with cardiac manifestations, systemic constitutional symptoms, and embolic events. Posterior circulation involvement occurs in approximately 20 percent of cerebral ischemic events.The endovascular technique is an established life-saving therapy for eligible patients upto 24 hours from symptom onset. However, the role of endovascular management in embolic atrial myxoma remains unknown with no international consensus guidelines for the management of stroke in such patient population. Here, we present a case report of an embolic posterior circulation stroke in a young female treated with mechanical thrombectomy at 23 hours from symptom onset. To the best of our knowledge, this is the first thrombectomy case in posterior circulation with embolism from myxoma. Further workup confirmed an atrial myxoma which was resected. We also review the previous cases with mechanical thrombectomy done in such cases.
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Embolia , Neoplasias Cardíacas , Mixoma , Accidente Cerebrovascular , Embolia/diagnóstico por imagen , Embolia/etiología , Embolia/cirugía , Femenino , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Humanos , Mixoma/complicaciones , Mixoma/diagnóstico por imagen , Mixoma/cirugía , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/cirugía , TrombectomíaRESUMEN
Though infections account for a significant proportion of patients with ocular motor palsies, there is surprising paucity of literature on infectious ophthalmoplegias. Almost all types of infectious agents (bacteria, viruses, fungi and parasites) can lead to ocular motor palsies. The causative infectious agent can be diagnosed in most cases using an orderly stepwise approach. In this review we discuss how to approach a patient with ophthalmoplegia with main focus on infectious etiologies.
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Oftalmoplejía , Humanos , Oftalmoplejía/diagnóstico , Oftalmoplejía/etiologíaRESUMEN
BACKGROUND: Loss of nigrosome-1 on 3T and 7T magnetic resonance imaging (MRI) is a recently explored imaging biomarker in the diagnosis of neurodegenerative parkinsonism. OBJECTIVES: This study was undertaken to evaluate the utility of imaging of nigrosome in the diagnosis of neurodegenerative parkinsonism on 3T MRI. METHODS: An institution-based prospective case-control study was conducted at a tertiary care center in North India. 3T venous blood oxygen level-dependent (VenoBOLD) and high-resolution susceptibility-weighted imaging (SWI) imaging sequences in MRI were performed in 100 patients with parkinsonism (56 with idiopathic Parkinson's disease [IPD], 30 with young onset Parkinson's disease [YOPD], 12 with progressive supranuclear palsy, and 2 patients with multiple system atrophy) and 15 controls. Grading of nigrosome was done in both the sequences. Each patient underwent 18F-DOPA positron emission tomography (PET), detailed neurological examination including Hoen and Yahr (H&Y) staging and Movement Disorder Society-Sponsored Revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) scoring. RESULTS: The diagnostic sensitivity and specificity of the detection of loss of nigrosome-1 on VenoBOLD and SWI sequence at 3T MR imaging were 90% and 66.7% and 94% and 80%, respectively. A weak negative correlation was found between the grading of the nigrosome and clinical parameters (H&Y and UPDRS III). There was no correlation between the side of nigrosome loss and clinical asymmetry. However, nigrosome imaging was not able to differentiate between Parkinson's disease and atypical parkinsonism. CONCLUSIONS: The loss of nigrosome-1 on 3T MRI on SWI and VenoBOLD sequences may serve as a potential imaging marker in the diagnosis of degenerative parkinsonian syndromes. However, it cannot differentiate between idiopathic Parkinson's disease and atypical parkinsonian syndromes.
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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.
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COVID-19 , Accidente Cerebrovascular , Control de Enfermedades Transmisibles , Humanos , India/epidemiología , Pandemias , Estudios Retrospectivos , SARS-CoV-2 , Accidente Cerebrovascular/epidemiología , Accidente Cerebrovascular/terapia , Tiempo de TratamientoRESUMEN
BACKGROUND: Presence of truncal dystonia usually points to a secondary cause of dystonia like exposure to dopamine receptor blockers or neurodegenerative illness. Rarely, it can occur as an idiopathic focal or segmental dystonia. METHODS: Retrospective review of medical records and videos of patients of truncal dystonia presenting in the Botulinum Toxin Clinic of Department of Neurology at Post Graduate Institute of Medical Education and Research, Chandigarh between May 2016 and February 2019. RESULTS: A total of 16 patients with predominant truncal dystonia were recruited. There were ten males and six females with mean age of 49.1 ± 15.1 years (range 22-70). Extensor truncal dystonia was the most common (12/16) followed by camptocormia (4/16). Various etiologies included Idiopathic Parkinson's disease (4/16), Tardive dystonia (5/16), Neurodegeneration with brain iron accumulation (genetically confirmed) (2/16) and idiopathic (5/16). All patients were refractory to a combination of oral medications tried over a period of 1.82 ± 1.93 years. All patients received electromyographic-guided botulinum toxin in paraspinals or rectus abdominis muscles depending upon the type of dystonia. The mean dose of abobotulinum toxin used was 286.7 ± 108.6 units (range 200-500 units) for paraspinals and 297.5 ± 68.5 (range 200-350) for rectus abdominis muscles per session. Average subjective response after botulinum toxin injection session was 31.2 ± 21.5% (range 0-70). No adverse effects were reported. CONCLUSION: Botulinum toxin is an acceptable alternative to patients presenting with medically refractory truncal dystonia and may offer modest benefit.
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Chronic granulomatous disease (CGD) is an uncommon genetic immunodeficiency disorder affecting neutrophil function, characterized by recurrent bacterial and fungal infections. X-linked carriers of CGD have an increased risk of autoimmune disorders, in particular lupus like disorders. We describe the case of a 37 years old female carrier of X-linked CGD, who presented with clinical features and serology consistent with a definite diagnosis of Systemic lupus erythematosus (SLE), with rare immune mediated neurological manifestations including secondary central nervous system (CNS) vasculitis and Longitudinally extensive transverse myelitis (LETM), responsive to immunomodulation. These neurological manifestations have not been described previously in carriers of CGD. We recommend early diagnosis of these immune mechanisms, especially in X-linked carriers of CGD, and appropriate immunomodulation in order to improve life expectancy and improve neurological outcome.
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Enfermedad Granulomatosa Crónica/complicaciones , Lupus Eritematoso Sistémico/complicaciones , Adulto , Antiinflamatorios/uso terapéutico , Femenino , Enfermedad Granulomatosa Crónica/tratamiento farmacológico , Enfermedad Granulomatosa Crónica/genética , Heterocigoto , Humanos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Metilprednisolona/uso terapéutico , Mielitis Transversa/etiología , Vasculitis del Sistema Nervioso Central/etiologíaRESUMEN
BACKGROUND AND PURPOSE: The cavernous sinus is a unique region owing to anatomical factors and the pathologies affecting it. The diagnosis of cavernous sinus syndrome (CSS) predominantly relies on clinicoradiological correlation. We studied the utility of computed tomographic (CT) scan versus magnetic resonance imaging (MRI) in the diagnosis of CSS. METHODS: A prospective observational study was conducted in a tertiary care center in north India. All patients presenting with a clinical syndrome of cavernous sinus involvement with radiologically confirmed lesions were enrolled in the study. MRI and CT scan with cavernous sinus cuts were done and reviewed by experienced neuroradiologists for cavernous sinus lesions and compared with the final diagnosis. Sensitivity and specificity were calculated. RESULTS: We included 48 patients in our study. A final diagnosis was achieved in 41 out of 48 (85.6%) patients. Fungal infections (16 (33.3%)) constituted the commonest cause of CSS, followed by neoplastic involvement (13 (27.1%)) and Tolosa-Hunt syndrome (12 (25%)). Vascular involvement was seen in three (6.3%) patients. Other rare causes were seen in four (8.3%) patients. CT scan had an overall sensitivity of 14.6% in achieving a final diagnosis, whereas MRI had an overall sensitivity of 70.7%, with a statistically significant difference (p < 0.001). CONCLUSIONS: Although CT scan is a relatively cheap and accessible resource, its role in CSS diagnosis and management is limited because of poor yield. Hence, it is prudent to do an MRI as an initial investigation in cases of CSS.