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Background: Empty sella is a commonly described imaging entity in patients with idiopathic intracranial hypertension (IIH). Though menstrual and hormonal disturbances have been associated with IIH, available literature lacks systematic analysis of pituitary hormonal disturbances in IIH. More so, the contribution of empty sella in causing pituitary hormonal abnormalities in patients of IIH has not been described. We carried out this study to systematically assess the pituitary hormonal abnormalities in patients with IIH and its relation to empty sella. Methods: Eighty treatment naïve patients of IIH were recruited as per a predefined criterion. Magnetic resonance imaging (MRI) brain with detailed sella imaging and pituitary hormonal profile were done in all patients. Results: Partial empty sella was seen in 55 patients (68.8%). Hormonal abnormalities were detected in 30 patients (37.5%), reduced cortisol levels in 20%, raised prolactin levels in 13.8%, low thyroid-stimulating hormone (TSH) levels in 3.8%, hypogonadism in 1.25%, and elevated levels of gonadotropins were found in 6.25% of participants. Hormonal disturbances were independent and were not associated with the presence of empty sella (p = 0.493). Conclusion: Hormonal abnormalities were observed in 37.5% patients with IIH. These abnormalities did not correlate with the presence or absence of empty sella. Pituitary dysfunction appears to be subclinical in IIH and responds to intracranial pressure reduction, not requiring specific hormonal therapies.

Tuberculosis (TB) is a global health problem with the major brunt of disease occurring in developing countries. The cornerstone of treatment of TB is anti-tubercular therapy (ATT), which includes rifampicin, isoniazid, pyrazinamide and ethambutol. Because of emerging drug resistance, treatment failures, defaulters and increasing incidence of disseminated and extrapulmonary TB, the guidelines have been modified in some countries. Ethambutol is prescribed for longer times (in some cases8 months) and hence the incidence of ethambutol-induced optic neuropathy (EtON) is expected to rise. The fundamental question which needs explanation is why only a small subset of patients on ethambutol are prone to develop loss of vision. This review focuses on available genetic studies which provide evidence that mitochondria are the likely substrates involved in the final pathway of reactive oxidative damage of the papillo-macular bundle. Genetic analysis of mitochondrial mutations encoding genes involved in oxidative phosphorylation pathways may help in isolating the subset of patients who are genetically susceptible. If the groups having high risk of developing EtON are recognised then prolonged duration of ethambutol treatment can be avoided in these susceptible individuals. A better understanding of the pathophysiology will also pave the way for the development of management strategies in this condition.

There is ongoing debate about whether the oculomotor (III), trochlear (IV), or abducens (VI) nerve paresis in patients with migraine is directly attributable to migraine (ophthalmoplegic migraine [OM]) or is due to an inflammatory neuropathy (recurrent painful ophthalmoplegic neuropathy [RPON]). As migraine is associated with elevated serum calcitonin gene-related peptide (CGRP) levels, we studied serum CGRP levels among patients with OM/RPON to determine whether they are elevated during and between attacks. This is the first study assessing CGRP levels in the serum of patients with OM/RPON.The aim of this case-control study was to assess serum CGRP levels in patients with ophthalmoplegia and a headache consistent with migraine according to ICHD-3 criteria. Serum CGRP levels were measured during the ictal and interictal phases in 15 patients with OM/RPON and compared with age-matched and sex-matched controls without migraine (12 patients).The median serum CGRP levels were significantly elevated (P = 0.021) during the ictal phase (37.2 [36.4, 43.6] ng/L) compared with controls (32.5 [30.1, 37.3] ng/L). Serum CGRP levels during the attack correlated with the total duration of ophthalmoplegia. A CGRP level of 35.5 ng/L in the ictal phase of the attack had a sensitivity of 86.7% and specificity of 75.0% in diagnosing a patient with OM/RPON.Elevated serum CGRP levels during the ictal phase of OM/RPON favor migraine as the underlying cause of episodic headache with ophthalmoplegia.

Therapeutic plasma exchange (TPE) is indicated in the acute presentation of neuromyelitis optica spectrum disorders (NMOSD) as category II indication as per American Society for Apheresis guidelines 2016.To assess the efficacy of TPE in symptomatic patients of NMOSD not responding to high dose intravenous steroids.We retrospectively reviewed the record of TPE procedures in NMOSD patients over a period of 3 years (2013-2016). TPEs were done on Cobe® spectra (Terumo BCT, Lakewood Co., USA) using 5% human serum albumin or fresh frozen plasma as replacement fluid. Clinical improvement was recorded post-procedure and adverse reactions if any was noted.We performed TPE in 11 clinically diagnosed patients of NMOSD. Three (27%) patients had only visual symptoms, five (46%) had both visual as well as muscular symptoms, whereas three (27%) patients presented with only muscular symptoms. Out of seven tested, three patients were aquaporin-4-immunoglobulin-G-positive (AQP4-IgG positive). Visual symptoms improved from no vision pre-TPE therapy to finger counting or 6/24 post-therapy.The muscle power of the limbs improved by grade one to grade two post-therapy. Adverse events were observed in 8% (5/62) of the procedures; allergic reactions to replacement fluid as the most common event (n = 3) followed by hypotension (n = 2). Follow-up was available in 81% (9/11) patients. Two patients died at 3 and 12 months of follow-up. One patient had relapsed and underwent a second TPE cycle.The addition of TPE as a part of therapy is effective and safe in acute exacerbations of NMOSD.

Ocular manifestations form an important clinical component of several movement disorders. Both hypokinetic and hyperkinetic movement disorders can involve the eye. Ophthalmological manifestations can arise due to dysfunction at the level of retina, optic nerves, oculomotor system, or subcortical or visual cortex. Ophthalmological findings help in differentiating various movement disorders and give a clue about their severity. These follow or may precede the diagnosis of movement disorders. Basal ganglia and its various nuclei especially substantia nigra pars reticulata play an important role in the genesis of eye movements through its connections with the superior colliculus. Eye movement abnormalities using quantitative recording techniques are now being considered as noninvasive biomarkers not only for the diagnosis but also to track the progression of disease as well as to study the effects of therapies in various movement disorders. Moreover, there is ample evidence of presence of retinal degeneration in various neurodegenerative diseases evaluated using optical coherence tomography. Various patterns of retinal thinning have been described in different movement disorders and have been found to have a correlation with the stage and severity of the disorder. In this review, we discuss ophthalmological findings of common hypokinetic and hyperkinetic movement disorders.

Neurovirulence of Coronavirus-2 (SARS-CoV-2) was established soon after the Coronavirus disease-19 (COVID-19) pandemic broke. While the initial reports from Chinese cohorts suggested that around 8 to 36% patients of COVID-19 develop neurological complications, upcoming literature revealed the various neurological presentations this newly emergent virus could have. Data on neurological manifestations of COVID-19 is still accumulating. Despite an immense flooding of recent databases, gender-specific issues of this novel virus still remain elusive. Increased mortality has been noted in elderly male patients, and immediate risk of death is a major concern. As per the available literature, women are more likely to recover from the virus, but little is described about the medical and social repercussions. Gender-based analysis should help delineate specific problems in women in the rapidly changing scenario. This article discusses the relevant neurological aspects of COVID-19 with specific reference to women.

Purpose: To describe and analyze the relationship between the downward eccentric eye movement/positioning encountered in patients undergoing ophthalmic surgeries under general anesthesia (GA) with fluctuation in the depth of anesthesia (DOA) Design: observational case-seriesMethods. Patients undergoing ophthalmic surgeries under GA without non-depolarizing muscle relaxant (NDMR) between January2018-August2021 who witnessed a sudden tonic downward movement of eyes were included. DOA at the time of eccentric movement were noted in terms of MAC (minimum alveolar concentration) with/without BIS (bispectral index).Results. A total of 12/249 patients (14 events) were enrolled in this study with a median (IQR) age of 1.05years (0.8-1.57). All cases were performed with sevoflurane as an inducing agent. Downward movement /down-positioning of eyes was seen before the start of surgery in 7, during surgery in 6, and end of surgery in 1 case. It was preceded by an eccentric upward drift of eyes in 6 cases. A strong negative correlation was seen between DOA and eccentric eye position (r=-0.77, p=0.000). Median (IQR) MAC and eye position/score in 14 events at the time of eccentric down-rolling was 1.55(1.40-1.83) and -3(-4 to -2.0) respectively while median (IQR) MAC at the time of centralized position was 1.25(1.18-1.40). BIS values were inconclusive in 2 cases but corroborated elevated and downward eye positions with lighter and deeper DOA respectively in 2 cases. Conclusions. Tonic-down-rolling/downward eccentric positioning of eyes is not an uncommon entity in children under GA without NDMR, and fluctuations in DOA should be avoided to circumvent inadvertent complications during ocular surgery.

Background. Unexpected tonic down-rolling and eccentric down-positioning of eyes during general anesthesia can have serious implications in ocular surgeries.Objectives. To summarize abrupt eccentric eye positioning in downgaze following the downward movement of eyes encountered in patients undergoing ophthalmic surgeries under general anesthesia (GA) and analyze the relationship between the fluctuation in the depth of anesthesia (DOA) and eccentric eye positioning. Subjects and Methods. Patients undergoing ophthalmic surgeries under GA without nondepolarizing muscle relaxant between January 2018-December 2019 in a tertiary-eye-care who witnessed a sudden tonic downward movement of eyes were included in the retrospective case series. Results. A total of 9 patients out of 199 were enrolled in this study with a median (IQR) age of 1.1years (0.79-1.5). All cases were performed under GA with sevoflurane as an inducing agent. Downward movement was seen before the start of surgery in 4 cases and during surgery in 5 cases. Downward drift of eyes appeared tonic as a strong tug was felt in an extreme downward eccentric position. It was preceded by an eccentric upward drift of eyes in 5 cases Downward movement was quick but return movement of eyes to the central position was gradual (median 1.5 minutes). Median (IQR) MAC and eye position/score in 9 cases at the time of eccentric movement in downgaze was 1.62 (1.4-1.8) and -4 (-4 to -2.5) respectively while median (IQR) MAC at the time of centralized position was 1.3 (1.2-1.4). BIS values in two cases corroborated elevated and downward eye positions with lighter and deeper DOA respectively. A strong negative correlation was seen between DOA and eccentric eye position (r=-0.89, p=0.000).Conclusions. Tonic-downward movement of eyes or its eccentric positioning in downgaze is not an uncommon entity in children under GA without muscle-relaxant and fluctuations in DOA should be avoided to circumvent inadvertent complications during ocular surgery.

As a response to the coronavirus disease (COVID-19) pandemic, several vaccines have been developed globally and are being rolled out at a fast pace for public immunization. Drug controller general of India has approved use of CovaxinTM (BBV152, manufactured by Bharat Biotech) and CovishieldTM (Oxford-AstraZeneca COVID-19 vaccine, manufactured by Serum Institute of India) for restricted use in emergency situation.1 We describe three cases of CovishieldTM induced minor cutaneous adverse effects.

Idiopathic intracranial hypertension (IIH) is defined as a syndrome of raised intracranial pressure with normal imaging of the brain and cerebrospinal fluid (CSF) composition. There are many controversies and myths that surround IIH. Although patients of IIH may present "typical" symptoms and signs of raised intracranial pressure, clinical scenarios often vary. A typical clinical and radiological finding poses significant problems in diagnosis and management of patients with IIH. We have tried to resolve these controversies and provide a comprehensive update on different aspects of IIH. In this article, we review the common problems encountered while dealing with patients of IIH.

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.

Acute nontraumatic weakness (ANTW) is defined as acute onset of weakness in any part of the body. The weakness occurs due to interruption at any point along the motor pathway. The motor pathway originates from upper motor neuron cells in the cerebral cortex and traverses through the brainstem till lower motor neurons in the spinal cord. The axon of a lower motor neuron is known as the peripheral motor nerve that synapses with muscle. ANTW is of varied etiology and presentation that may be immediately life-threatening if respiratory muscles or autonomic nervous system is involved. Involvement of respiratory muscles may be associated with respiratory failure that may require mechanical ventilation. The weakness may be localized to one limb or generalized involving several muscle groups. When bulbar muscles are involved, weakness leads to problem in swallowing and coughing that endangers the patient's airway. Similarly, the course of the disease also varies, and these patients may worsen rapidly. Hence, a comprehensive history, systematic evaluation, and a detailed neurological examination are performed to localize the disorder. There are specific clinical features peculiar to the particular location of the lesion in the body. Hence, it is possible to anatomically localize these lesions based on the clinical features. Initial laboratory tests and appropriate neuroimaging should be obtained as indicated by history and examination. The time-sensitive emergencies should be addressed immediately, as the delay in management may lead to either permanent neurological damage or may worsen the overall outcome in such conditions. The initial management should always include care of airway, breathing, and circulation (ABC). The imaging should be obtained only after initial stabilization of ABC. The definitive treatment should be done as per the etiology.

The importance of ovarian teratoma as a cause of opsoclonus myoclonus ataxia syndrome (OMAS) and other paraneoplastic syndromes continues to be underestimated. A strong suspicion and appropriate diagnosis remain keys to successful outcome of paraneoplastic OMAS with ovarian teratoma. We report a 14-year-old girl with paraneoplastic OMAS in association with an ovarian teratoma who improved completely following resection of tumour as well as immunotherapy and review the literature briefly.

Objectives. To summarize abrupt eccentric eye positioning in downgaze following the downward movement of eyes encountered in patients undergoing ophthalmic surgeries under general anesthesia (GA) and analyze the relationship between the fluctuation in the depth of anesthesia (DOA) and eccentric eye positioning in downgaze. Subjects and Methods. Patients undergoing ophthalmic surgeries under GA without nondepolarizing muscle relaxant between January 2018-December 2019 in a tertiary-eye-care who witnessed a sudden tonic hypo-tropic movement of eyes were included in the retrospective, cross-sectional study. Results. A total of 8 patients out of 199 were enrolled in this study with an average age of 1.13±0.40years. All cases (3 pediatric cataracts, 4 strabismus, and 1 pseudophakia with posterior capsular opacification) were performed under GA with sevoflurane as an inducing agent. Downward movement was seen before the start of surgery in 4 cases and during surgery in 4 cases. Downward drift of eyes appeared tonic as the strong tug was felt in an extreme downward eccentric position. It was preceded by an eccentric upward drift of eyes following which sevoflurane concentration was increased to optimize DOA when this downward drift was encountered (mean minimal alveolar concentration/MAC 1.63±0.25). Downward movement was quick but return movement of eyes to the central position was gradual (mean 1.55±0.48minutes) when DOA was decreased (mean MAC 1.3±0.09).Conclusions. Tonic-downward movement of eyes or its eccentric positioning in downgaze is not an uncommon entity in children under GA without muscle-relaxant and fluctuations in DOA should be avoided to circumvent inadvertent complications during ocular surgery.

Background The primary role of neuroimaging in idiopathic intracranial hypertension (IIH) is to exclude secondary causes of raised intracranial pressure. Recently, a few imaging markers have been described which may suggest diagnosis of IIH in atypical cases. We carried out this study to assess the prevalence and accuracy of these neuroimaging signs in predicting the diagnosis of IIH. Methods Eighty treatment-naive patients with IIH and 30 controls were recruited as per a predefined criterion. Magnetic resonance imaging (MRI) brain with detailed sella imaging was done in all patients. Results The most common abnormality noted was optic nerve tortuosity in 82.5% of patients, followed by posterior scleral flattening in 80%, perioptic subarachnoid space (SAS) dilatation in 73.8% and partial empty sella in 68.8% of patients. The presence of optic nerve tortuosity was the most sensitive sign on neuroimaging, though the highest specificity was seen for posterior scleral flattening and perioptic SAS dilatation. The presence of more than three neuroimaging features correlated with severity of vision loss. Conclusion In suggestive clinical scenarios, posterior scleral flattening, perioptic SAS dilatation and optic nerve tortuosity are highly sensitive and specific signs in IIH. This study also highlights the utility of MRI as a valuable tool for prognosis of visual outcome in patients with IIH.

AIMS AND OBJECTIVES: High dose methotrexate (HDMTx) based chemotherapy forms the backbone of therapy for patients with Primary Central Nervous system Lymphoma (PCNSL). However, delivering HDMTx in resource constrained settings, especially without therapeutic drug monitoring, is difficult. We share our experience of treatment of patients with PCNSL at our center over a 10-year period with local adaptations made to deliver HDMTx. MATERIALS AND METHODS: We retrospectively analysed the case records of patients diagnosed with a PCNSL over the course of 10 years from 2010 to 2020. RESULTS: Fifty-five patients received therapy for newly diagnosed PCNSL. Thirty-six patients received Modified De-Angelis protocol ± Rituximab with curative intent. Fourteen of these patients were unable to complete the protocol with the most common cause being development of methotrexate toxicity. Patients unable to complete the designated 5 cycles of HDMTx had a poorer PS and higher probability of having a high IELSG score at baseline. Nineteen patients were given non HDMTx based therapy either due to advanced age or poor performance status. Twenty-nine patients (52.7%) were able to achieve a complete response. The most common cause of mortality was relapse/progressive disease. The Median EFS and OS of the cohort was 29 months and 40 months respectively. CONCLUSION: All attempts should be made to have therapeutic drug level monitoring for administration of HDMTX based therapy for the patients with PCNSL, more so in patients who have poor performance status and a high IELSG score. If it is imperative to give HDMTx without access to TDM facility then a possible risk of higher toxicity should be explained to all patients, beforehand

Supplemental material, sj-pdf-1-neu-10.1177_19714009211000623 for Sensitivity and specificity of neuroimaging signs in patients with idiopathic intracranial hypertension by Nandita Prabhat, Shivani Chandel, Dr Aastha Takkar, Chirag Ahuja, Ramandeep Singh, Soundappan Kathirvel and Vivek Lal in The Neuroradiology Journal

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 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.

Background: The pathway underlying ocular convergence is less clear. Convergence weakness is usually a clinical sign of dorsal midbrain pathology. An orbital tumor causing such phenomenon is unknown. We describe its occurrence in patients with proptosis due to orbital tumors. Materials and Methods: Series of six patients with axial proptosis secondary to orbital tumors that demonstrated varying forms of convergence weakness (deficiency to complete failure). Results: Three had weakness of convergence with drifting away of proptosed eyeball on attempted near vision, while the other three had failure. One of these had convergence weakness in nonproptosed eye. MRI excluded a central etiology in all. Conclusions: The article highlights an interesting phenomenon of deficient convergence in the presence of intact medial rectus function in patients with proptosis. The symmetry of orbital contents possibly plays a significant role in the process of convergence.

The scientific basis of intracranial aneurysm (IA) formation, its rupture and further development of cerebral vasospasm is incompletely understood. Aberrant protein expression may drive structural alterations of vasculature found in IA. Deciphering the molecular mechanisms underlying these events will lead to identification of early detection biomarkers and in turn, improved treatment outcomes. To unravel differential protein expression in three clinical subgroups of IA patients: (1) unruptured aneurysm, (2) ruptured aneurysm without vasospasm, (3) ruptured aneurysm who developed vasospasm, we performed untargeted quantitative proteomic analysis of aneurysm tissue and serum samples from three subgroups of IA patients and control subjects. Candidate molecules were then validated in a larger cohort of patients using enzyme-linked immunosorbent assay. A total of 937 and 294 proteins were identified from aneurysm tissue and serum samples, respectively. Several proteins that are known to maintain structural integrity of vasculature were found to be dysregulated in the context of aneurysm.

Leber hereditary optic neuropathy (LHON) is a maternally inherited disease resulting in irreversible visual loss usually in patients belonging to the age group of 15-35 years. Clinically, the patients present with sequential or bilateral, painless, progressive visual loss with central (or ceco-central) scotomas. Although the three mutations, namely, G11778A, T14484C, and G3460A contribute to95% of LHON cases globally, the relative frequency of each mutation varies.We aimed to assess the clinical and genetic profile of patients with mutation-positive LHON at a north Indian tertiary care center.One hundred sixty-one patients (61 prospective and 100 retrospective) presenting with the clinical diagnosis of LHON were screened for the three known mitochondrial mutations (G1178A, G3460A, T14448C). Patients were assessed for detailed clinical, ophthalmological, and neurological examinations. Five milliliter of blood sample was taken to assess the three known mutations using DNA isolation and Sanger sequencing.Clinical profile of 83 patients with both positive and negative mutations was analyzed. Twenty-three out of 161 patients (14.3%) tested positive for either of the three mutations. The majority of the patients harbored G11778A mutation (56.52%) followed by T14484C (34.78%) and G3460A (8.69%). No statistical difference could be noted between the clinical profiles of mutation-negative and -positive patients.