Sensitivity and specificity of phrenic nerve electrophysiology to predict mechanical ventilation in the Guillain-Barré syndrome.

INTRODUCTION/AIMS: In Guillain-Barré syndrome (GBS), the sensitivity and specificity of phrenic compound muscle action potential (CMAP) measurements to predict endotracheal mechanical ventilation are unknown. Hence, we sought to estimate sensitivity and specificity. METHODS: We performed a 10-year retrospective analysis of adult GBS patients from our single-center laboratory database (2009 to 2019). The phrenic nerve amplitudes and latencies before ventilation were recorded along with other clinical and demographic features. Receiver operating curve (ROC) analysis with area under the curve (AUC) was used to determine the sensitivity and specificity with 95% confidence interval (CI) for phrenic amplitudes and latencies in predicting the need for mechanical ventilation. RESULTS: Two hundred five phrenic nerves were analyzed in 105 patients. The mean age was 46.1 ± 16.2 years, with 60% of them being male. Fourteen patients (13.3%) required mechanical ventilation. The average phrenic amplitudes were lower in the ventilated group (P = .003), but average latencies did not differ (P = .133). ROC analysis confirmed that phrenic amplitudes could predict respiratory failure (AUC = 0.76; 95% CI, 0.61 to 0.91; P < .002), but phrenic latencies could not (AUC = 0.60; 95% CI, 0.46 to 0.73; P = .256). The best threshold for amplitude was ≥0.6 mV, with sensitivity, specificity, and positive and negative predictive values of 85.7%, 58.2%, 24.0%, and 96.4%, respectively. DISCUSSION: Our study suggests that phrenic CMAP amplitudes can predict the need for mechanical ventilation in GBS. In contrast, phrenic CMAP latencies are not reliable. The high negative predictive value of phrenic CMAP amplitudes ≥0.6 mV can preclude mechanical ventilation, making these a useful adjunct to clinical decision-making.

Management and Outcomes of COVID - 19 Associated Cerebral Venous Sinus Thrombosis.

BACKGROUND: Systemic hyper-coagulabilty leading to micro and macro thrombosis is a known complication of Coronavirus disease - 2019(COVID -19). The postulated mechanism appears to be the viral activation of endothelium, triggering the coagulation pathways. Thrombosis of the cerebral veins and sinuses (CVT), a potentially serious condition, has been increasingly reported with COVID - 19 infection. In this clinical study we attempt to describe the clinical profile, investigations and outcomes of patients with COVID- 19 associated CVT. METHODS: This is a single center prospective observational study from South India. The study included patients (aged >18 years) with concomitant COVID infection and CVT. The clinical, laboratory, imaging characteristics, management and outcomes were described and compared with COVID negative CVT patients. RESULTS: Out of 97 cases of CVT treated at our center during the first and second waves of the COVID pandemic 11/97 (11%) were COVID related CVT. Among these 11 patients, 9 (81%) had presented with only CVT related symptoms and signs and were tested positive for COVID - 19 infection during the pre-hospitalization screening. Respiratory symptoms were absent in 90% of the patients. Headache (100%) and seizures (90%) were the common presenting symptoms. The median time to diagnosis was 6 hours, from presentation to the emergency department. Transverse sinus was involved 10/11 (90%) and majority of them (9/11) had Haemorrhagic Venous Infarction (HVI). Acute inflammatory markers were elevated in comparison with non COVID CVT patients, with the mean serum D-dimer being 2462.75 ng/ml and the C-reactive protein was 64.5 mg/dl. Three patients (30%) underwent decompressive hemicraniectomy (DHC) because of large hemispheric HVI. All patients survived in the COVID CVT group while the mortality in the non COVID group was 4%. At 6 months follow up excellent outcome (modified Rankin Scale (mRS) score of 0-2) was noted equally in both groups. CONCLUSIONS: Symptoms and signs of CVT may be the only presentation of COVID-19 infection. Prompt recognition and aggressive medical management including DHC offers excellent outcomes.

Utility of stimulus induced after discharges in the evaluation of peripheral nerve hyperexcitability: Old wine in a new bottle?

Limited literature is available on stimulus induced after discharges (SIAD) in patients with peripheral nerve hyperexcitability (PNH). The aim of the study was to examine the diagnostic utility of SIAD in the diagnosis and monitoring of primary PNH disorders. In this retrospective study, we studied 26 patients who were admitted with a diagnosis of primary PNH to the department of Neurology from January 2013 to April 2019. Their clinical profile, immunological characteristics were extracted from the database and nerve conduction studies were relooked for the presence of SIAD. 76% of patients in the primary PNH cohort had SIAD with 90% of them being voltage-gated potassium channel complex antibody positive; predominantly against contactin-associated protein-2 antigen and rest being paraneoplastic. There was also resolution of SIAD following treatment indicating reversible hyperexcitability. SIAD is a sensitive marker for Primary PNH syndrome with monitoring and diagnostic implications.

Monoclonal Gammopathies of 'Neurological Significance': Paraproteinemic Neuropathies.

OBJECTIVES: To study the clinical profile and outcomes of patients with paraproteinemic neuropathy (PPN) and to explore the utility of nerve conduction studies (NCSs) to differentiate between the demyelinating subtypes. METHODS: We did a retrospective analysis of patients diagnosed with PPN between January 2010 and December 2019 in an inpatient setting. The study population consisted of patients above 16 years of age presenting with clinical features suggestive of chronic peripheral neuropathy and on evaluation was found to have PPN. RESULTS: A total of 74 patients were identified. The patients were predominantly in the 6th decade, and the majority were males. The subtypes of PPN were monoclonal gammopathy of undetermined significance (MGUS) (45.9%), POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) (24.3%), solitary plasmacytoma (17.6%), multiple myeloma (8.1%), and AL amyloidosis (4.1%). There are specific features on NCS which can help in identifying POEMS syndrome and IgM MGUS. The majority of patients with PPN tend to stabilize or improve with treatment; however, many have a severe residual disability. New terminology and classification of these entities as 'monoclonal gammopathies of neurological significance' can aid in early diagnosis and the development of effective treatment, to prevent residual disability. CONCLUSION: PPN has a heterogeneous spectrum of clinical, biochemical, and electrophysiological features. NCS can help distinguish POEMS syndrome and IgM MGUS from other demyelinating subtypes.

An Unusual Cause of Chronic Headache in an Adolescent Boy: A Case Report.

Spontaneous intracranial hypotension (SIH) is an under-diagnosed cause of headache in children and adolescents. SIH results from cerebrospinal fluid (CSF) leak due to breach in the dura mater and the etiology for dural breach is often diverse. We report an adolescent boy who presented with chronic episodic headache that later progressed to daily headache. There was a typical history of worsening of headache on upright position and relief of headache on lying down. He was treated with migraine prophylaxis in another hospital but there was no response. Marfanoid features and brisk deep tendon reflexes were observed on clinical examination. Brain magnetic resonance imaging (MRI) revealed sagging of the brain stem, pachymeningeal enhancement, and tonsillar herniation. MRI of spine myelogram confirmed multiple levels of CSF leak. He was initially managed with supportive measures and fluoroscopic-guided fibrin glue injection. Although child remained symptom-free for the next 6 months, he again developed headache. MRI and computed tomography spine myelogram revealed a meningeal diverticulum in the lumbar spine. He was managed with an autologous epidural blood patch and he has been well since then. In this report, we highlight the clinical and radiological pointers to the presence of SIH in children with recurrent headache.

EEG lateralization and seizure outcome following peri-insular hemispherotomy for pediatric hemispheric epilepsy.

OBJECTIVE: To determine whether preoperative non-lateralizing scalp electroencephalography (EEG) influences seizure outcome following peri-insular hemispherotomy (PIH) in pediatric hemispheric epilepsy. METHODS: Retrospective data was collected on all 45 pediatric patients who underwent PIH between 2005 and 2016. All underwent a basic pre-surgical evaluation consisting of detailed history and examination, neuropsychological assessment, MRI, and EEG. SPECT/PET, fRMI, or Wada testing were done in only eight patients. Seizure outcome was assessed using the Engel classification. RESULTS: Among those who underwent hemispherotomy, 20 (44%) were females. Mean age at surgery was 8 ± 4.3 years and mean duration of symptoms was 5.2 ± 3.7 years. The most common etiologies of hemispheric epilepsy were hemiconvulsion-hemiplegia epilepsy syndrome, Rasmussen encephalitis, and post-encephalitic sequelae, together comprising 27 (60%) patients. Among the 44 patients with follow-up data (mean duration 48 ± 33 months), seizure freedom (Engel class I) was attained by 41 (93.2%). Anti-epileptic medications were stopped or decreased in 36 (82%). Seventeen (38.6%) patients had non-lateralizing EEG. Seizure outcome was not related to lateralization of EEG activity. CONCLUSIONS: PIH provides excellent long-term seizure control in patients despite the presence of non-lateralizing epileptiform activity, although occurrence of acute postoperative seizures may be higher. Routine SPECT/PET may not be required in patients with a non-lateralizing EEG if there is good clinico-radiological concordance.

Thyroid dysfunction in patients on antiretroviral therapy: A perspective from southern India.

Background: Thyroid dysfunction in patients with human retroviral infection has been reported but the prevalence of thyroid function abnormalities in patients on highly active antiretroviral therapy (HAART) has not been studied. We aimed to assess the prevalence of thyroid dysfunction and autoimmunity (antithyroid peroxidase auto-antibodies [TPO-Ab]) in patients on first-line HAART, identify risk factors for thyroid dysfunction and determine any association of thyroid dysfunction with HAART. Methods: We screened and enrolled consecutive patients from the outpatient department if they were (i) diagnosed with HIV infection (enzyme-linked immunosorbent assay); (ii) aged more than 18 years; (iii) on HAART for 1 year or more; and (iv) clinically stable with no evidence of any acute illness in the past 2 months. We excluded patients who were on drugs that affect thyroid function. Thyroid function tests and CD4 counts were done. Results: A total of 159 patients on firstline HAART were included in the study. Their mean (SD) age was 43.3 (10) years and duration of HAART was 44.4 (33.54) months. The mean CD4 count was 502.8 (274.45). Forty-seven patients (29.6%) had thyroid dysfunction. TPO-Ab positivity was noted in 6 patients. No association was seen between thyroid dysfunction and any type of regimen or drug. There was a significant negative correlation between CD4 counts and thyroid-stimulating harmone (TSH) suggesting that thyroid dysfunction may be more prevalent when immunity is low. Conclusions: There is a high prevalence of thyroid dysfunction, predominantly subclinical hypothyroidism, in patients on HAART. Thyroid autoimmunity is low in this subset of patients. Lower immunity is associated with higher TSH levels. Larger longitudinal studies are required to determine the course of hypothyroidism in patients on HAART.

Accuracy and reliability of Babinski sign versus finger and foot tapping in the diagnosis of corticospinal tract lesions.

INTRODUCTION: The Babinski sign is one of the most important clinical signs for detecting corticospinal tract (CST) lesions. However, due to variations in testing and interpretation, it has been associated with low interobserver agreement rates. In this study, the diagnostic value of finger and foot tapping in detecting CST lesions was compared to that of the Babinski sign. MATERIALS AND METHODS: Three groups of participants were recruited: Group 1 - individuals having CST lesions diagnosed on the basis of clinical examination as well as neuroimaging; group 2 - individuals having a non-CST neurological illness; group 3 - normal individuals who were relatives of the patients recruited. The sensitivity and specificity of finger tapping, foot tapping, and Babinski sign were calculated. RESULTS: 375 patients, 125 in each group, were included. The overall sensitivity for Babinski sign was 49.6% and specificity was 85.8%. The overall sensitivity for finger and foot tapping was 79.5% and specificity was 88.4%. The interobserver agreement between the medical students and the neurologist was greater for finger and foot tapping (Kappa = 0.83) when compared to Babinski sign (Kappa = 0.45). CONCLUSION: Finger and foot tapping is a valid and reliable test in the clinical diagnosis of corticospinal lesions. The reliability and validity of Babinski sign is variable and thus its ability to diagnose the manifestations of corticospinal lesions is less when compared to the finger and foot tapping test.

Is Ross Syndrome an Autoimmune Entity? A Case Series of 11 Patients.

BACKGROUND: Ross syndrome is diagnosed by the presence of segmental anhidrosis, areflexia, and tonic pupils. Fewer than 60 cases have been described in literature so far. There have been reports of presence of antibodies in such patients, suggesting an autoimmune pathogenesis. METHODS: We describe the clinical profile in this case series of 11 patients with Ross syndrome and discuss the current status of autoimmunity in its pathogenesis and the management. RESULTS: Of the 11 patients with Ross syndrome there was an almost equal sex distribution (male:female ratio was 1.17:1) and the mean age of onset of symptoms was 26 years. Patients took an average of 6 years to present to a tertiary center. Sixty-three percent of the patients presented with complaints of excessive sweating, whereas only 27% had complaints of decreased sweating over a particular area of the body. Only 45% of the patients had the complete triad of Ross syndrome, which included segmental anhidrosis, tonic pupil, and absent reflexes. Eighty-nine percent of the patients had documented absent sympathetic skin response on electromyography. The various markers of autoimmunity were negative in all patients who were investigated for the same in this series. Ninety percent of the patients were managed conservatively. CONCLUSIONS: These findings suggest that, in Ross syndrome, generalized injury to ganglion cells or their projections are not purely autoimmune-mediated.

Sonothrombolysis for acute ischemic stroke - Break on through to the other side.

BACKGROUND: Intravenous (IV) tissue plasminogen activator (tPA) infusion combined with transcranial low-frequency ultrasound waves targeted on the occluded arterial segment (sonothrombolysis) can increase recanalization in large artery-acute ischemic stroke (LA-AIS). AIMS: To evaluate the benefits of sonothrombolysis in LA-AIS. SETTINGS AND DESIGNS: An open-labeled observational study done in a quaternary care teaching hospital. METHODOLOGY: Patients with LA-AIS within the window period (<4.5 h) with no contraindications for IV-recombinant tPA were sonothrombolysed. Recanalization was monitored and graded using the transcranial Doppler thrombolysis in brain ischemia (TIBI) flow criteria and also by time of flight magnetic resonance angiography using a modified thrombolysis in myocardial infarction score. Parenchymal changes were assessed using computed tomography (CT) or diffusion-weighted imaging-Alberta Stroke Programme Early CT Score. National Institutes of Health Stroke Scale (NIHSS) and modified Rankin Scale (mRS) were used to assess the outcome. RESULTS: Eighteen patients underwent sonothrombolysis and the mean onset to needle time was 138 min (range 65-256). TIBI residual flow grade of ≥2 was seen in 15 of 18 patients (83%). Immediate dramatic improvement (NIHSS score ≤3 points or improvement by ≥10 points) was seen in 6 of 18 patients (30%) and in 9 of 18 patients (50%) within the next 24 h. Two patients (one with TIBI 0, another with re-occlusion) underwent mechanical thrombectomy post-sonothrombolysis. Symptomatic hemorrhage occurred in 5.5% of the patients. At 6 months, 2 of 18 patients (11%) died and 10 of 16 patients (63%) achieved mRS ≤2. CONCLUSIONS: Sonothrombolysis appears to be a safe way to augment the effect of tPA without increasing the door to needle time with the added advantage of observing flow through the occluded artery in real time.

'Munchausen syndrome': a forgotten diagnosis in the spine.

PURPOSE: To present the case of a patient with Munchausen's syndrome who underwent multiple surgeries in the spine before the diagnosis was made and, therefore, to highlight the importance of this obscure condition that can result in unnecessary surgical treatment. METHODS: A 44-year-old businesswoman presented with multiple episodes of low back pain and weakness in both lower limbs over past 11 years. Past history consisted of multiple hospitalizations, and three surgeries on her lumbar spine at different hospitals, with dramatic improvement in symptoms being reported each time after surgery. Clinical examination showed inconsistent and nonspecific neurological findings. Imaging studies like X-rays, magnetic resonance imaging, and all neurophysiological studies were within normal limits. RESULTS: Multi-disciplinary evaluation by a team of orthopedicians, neurologist and psychiatrist and rehabilitation specialists diagnosed it as 'Munchausen syndrome'. Only one report of this fictitious disease in spine was found in review of literature (Association AP, Diagnostic and statistical manual of mental disorders: DSM-IV-TR(®), 2003). CONCLUSIONS: A history of multiple surgical interventions at multiple hospitals, often followed by dramatic improvement and then relapse, should trigger a suspicion of Munchausen syndrome, particularly in the scenario of normal imaging studies. Diagnosing this rare condition in spine is key to avoid unnecessary surgery.

Therapeutic Drug Monitoring of Levetiracetam and Lamotrigine: Is There a Need?

BACKGROUND: This study was a retrospective assessment of the therapeutic drug monitoring data collected for levetiracetam and lamotrigine from a clinical setting. The proportion of patients in relation to the therapeutic ranges for serum concentrations of lamotrigine and levetiracetam was estimated, and the influence of age and anticonvulsant comedications on their clearances were studied. METHODS: Information on levetiracetam (2011-2013) and lamotrigine (2008-2013) dose, trough concentration, age, sex, body weight, and anticonvulsant comedications prescribed was obtained from the therapeutic drug monitoring register and archived medical records. Patients were categorized into 4 groups based on anticonvulsant comedications and further divided into 3 subgroups based on age (a: <9 years; b: 9-17 years; c: ≥18 years). In each subgroup, the proportion of patients who achieved trough concentrations in the therapeutic range for levetiracetam and lamotrigine was computed. Apparent clearance (CL/F) was compared across subgroups by 1-way analysis of variance, and factors which significantly predicted CL/F were identified by stepwise multiple linear regression. RESULTS: Overall, 348 (330 patients) and 706 (493 patients) samples for levetiracetam and lamotrigine were included in the analysis. Of these, 56.9% and 72.4% were within, 43.1% and 23.9% below, 0% and 3.7% above the therapeutic range for levetiracetam and lamotrigine, respectively. A significant difference in CL/F was noted across subgroups for levetiracetam (P < 0.001) and lamotrigine (P < 0.001). Age <9 years, age ≥18 years, and inducer comedications significantly predicted CL/F for levetiracetam. For lamotrigine, inhibitor comedications, age <9 years, inducer comedications, and age 9-17 years significantly predicted CL/F. CONCLUSIONS: These findings emphasize the need to monitor relatively newer anticonvulsants, lamotrigine and levetiracetam, especially among children and when other anticonvulsant comedications are prescribed or discontinued in the treatment regimen.

Natural history of multiple sclerosis from the Indian perspective: Experience from a tertiary care hospital.

CONTEXT: Multiple sclerosis (MS) has a spectrum of heterogeneity, as seen in western and eastern hemispheres, in the clinical features, topography of involvement and differences in natural history. AIM: To study the clinical spectrum, imaging, and electrophysiological as well as cerebrospinal fluid (CSF) characteristics and correlate them with outcome. SETTINGS AND DESIGN: Retrospective analysis of MS patients during a period of 20 years. SUBJECTS AND METHODS: Cases were selected according to recent McDonald's criteria (2010), They were managed in the Department of Neurology, Christian Medical College, Vellore. STATISTICAL ANALYSIS USED: Chi-square and Fisher's exact tests were used for categorical variables. Multiple binary logistic regressions were done to assess significance. Kaplan-Meier curves were drawn to estimate the time to irreversible disability. RESULTS: A total of 157 patients with female preponderance (55%) were included. The inter quartile range duration of follow-up was 9.1 (8.2, 11) years for 114 patients, who were included for final outcome analysis. Relapsing remitting MS (RRMS) (54.1%) was the most common type of MS seen. RRMS had a significantly better outcome (odds ratio: 0.12, 95% confidence interval: 0.02-0.57, P = 0.008) compared to progressive form of MS (primary progressive, secondary progressive). The Expanded Disability Status Scale score of patients at presentation and at final follow-up was 4.4 ± 1.31 and 4.1 ± 2.31, respectively. During the first presentation, polysymptomatic manifestations like motor and sphincteric involvement, incomplete recovery from the first attack; and, during the disease course, bowel, bladder, cerebellar and pyramidal affliction, predicted a worse outcome. CONCLUSION: A high incidence of optico-spinal presentation, predominance of RRMS and a low yield on cerebrospinal fluid (CSF) studies are the major findings of our study. A notable feature was the analysis of prognostic markers of disability.

From Pseudotumor Cerebri to Neurobrucellosis: A Journey With Several Lessons.

We present a case admitted for evaluation of suspected idiopathic intracranial hypertension (IIH) with an unusual but important departure from the expected algorithm. A 31-year-old lady came with a two-week duration of a mild headache and one-week duration of double vision with no previously documented fever or any comorbidities. Clinically, she had papilledema and bilateral abducens palsy with no signs of meningeal irritation. MRI brain radiology was consistent with IIH. Her CSF study showed pleocytosis with elevated protein levels and normal glucose. Serology was positive for Brucella melitensis at low titers but CSF culture grew Brucella melitensis, confirming the diagnosis of neurobrucellosis. Her headache and abducens palsy improved over the first two weeks, and the papilledema resolved over two months with antibiotics. This clinical mimic is important for physicians (including neurophysicians) and Infectious Disease specialists. The radiological mimic comes from chinked (small) ventricles, unlike most meningeal diseases which can present with papilledema and abducens palsy including tuberculosis, cryptococcosis, and leptomeningeal carcinomatosis. A CSF study is mandatory in the workup of IIH despite massive improvements in imaging.

Emerging pattern of asymptomatic hyperparathyroidism in South India - a six-year retrospective study.

INTRODUCTION: Primary hyperparathyroidism (PHPT) is a common endocrine disease with a variable presentation. There is a recent increase in the number of asymptomatic cases due to the use of multichannel automated analyzers.

"De Novo" Hypercapnic Respiratory Failure Unmasking Neuromuscular Disorders: Experiences From a Tertiary Care Center and Review of Literature.

OBJECTIVES: Neuromuscular disorders could have respiratory involvement early or late into illness. Rarely, patients may present with a hypercapnic respiratory failure (with minimal motor signs) unmasking an underlying disease. There are hardly any studies which have addressed the spectrum and challenges involved in management of this subset, especially in the real-world scenario. METHODS: A retrospective study comprising consecutive patients hospitalized with hypercapnic respiratory failure as the sole/dominant manifestation. The clinical-electrophysiological spectrum, phrenic conductions, diaphragm thickness, and outcomes were analyzed. RESULTS: Twenty-seven patients were included, the mean age was 47.29 (SD 15.22) years, and the median duration of respiratory symptoms was 2 months (interquartile range [IQR] 1-4). Orthopnea was present in 23 patients (85.2%) and encephalopathy in 8 patients (29.6%). Phrenic nerve latencies and amplitudes were abnormal in 83.3% and 95.6%, respectively. Abnormal diaphragm thickness was noted in 78.5%. Based on a comprehensive electrophysiological strategy and paraclinical tests, an etiology was established in all. Reversible etiologies were identified in 17 patients (62.9%). These included myasthenia gravis (anti-AChR and MuSK), inflammatory myopathy, riboflavin transporter deficiency neuronopathy, Pompe disease, bilateral phrenic neuritis, and thyrotoxicosis. Respiratory onset motor neuron disease was diagnosed in 8 patients (29.6%). Despite diaphragmatic involvement, a functional respiratory recovery was noted at discharge (45%) and last follow-up (60%). Predictors for good outcomes included female sex, normal nerve conductions, and recent-onset respiratory symptoms. DISCUSSION: A good functional recovery was noted in most of the patients including respiratory onset motor neuron disease. A systematic algorithmic approach helps in proper triaging, early diagnosis, and treatment. Clinical and electrodiagnostic challenges and observations from a tertiary care referral center are discussed.

Decompressive craniectomy in cerebral venous thrombosis: a single centre experience.

BACKGROUND: Cerebral venous thrombosis (CVT) is an important cause for stroke in the young where the role for decompressive craniectomy is not well established. OBJECTIVE: To analyse the outcome of CVT patients treated with decompressive craniectomy. METHODS: Clinical and imaging features, preoperative findings and long-term outcome of patients with CVT who underwent decompressive craniectomy were analysed. RESULTS: Over 10 years (2002-2011), 44/587 (7.4%) patients with CVT underwent decompressive craniectomy. Diagnosis of CVT was based on magnetic resonance venography (MRV)/inferior vena cava (IVC). Decision for surgery was taken at admission in 19/44 (43%), within 12 h in 5/44 (11%), within first 48 h in 15/44 (34%) and beyond 48 h in 10/44 (22%). Presence of midline shift of ≥ 10 mm (p<0.0009) and large infarct volume (mean 146.63 ml; SD 52.459, p<0.001) on the baseline scan influenced the decision for immediate surgery. Hemicraniectomy was done in 38/44 (86%) and bifrontal craniectomy in 6/44 (13.6%). Mortality was 9/44 (20%). On multivariate analysis (5% level of significance) age <40 years and surgery within 12 h significantly increased survival. Mean follow-up was 25.5 months (range 3-66 months), 26/35 (74%) had 1 year follow-up. Modified Rankin Scale (mRs) continued to improve even after 6 months with 27/35 (77%) of survivors achieving mRs of ≤ 2. CONCLUSIONS: This is the largest series on decompressive craniectomy for CVT in literature to date. Decompressive craniotomy should be considered as a treatment option in large venous infarcts. Very good outcomes can be expected especially if done early and in those below 40 years.

Total Knee Arthroplasty in Paget's Disease using 3D-Printed Patient-Specific Femoral Jig - A Case Report.

Introduction: Patients with Paget's disease develop abnormal bony anatomy which can result in significantly altered lower limb alignment predisposing them to early secondary osteoarthritis. Due to the severe extra-articular deformity, total knee arthroplasty (TKA) in these patients is challenging. Conventional knee arthroplasty using intramedullary guides is not an option and can lead to erroneous limb alignment postoperatively. Patient-specific instrumentation (PSI) is a simple solution in such complex primary knee arthroplasty. Case Report: A 70-year-old male patient presented with a severe left femur deformity and left knee pain. He was diagnosed to have monostotic Paget's disease of the left femur with tricompartmental osteoarthritis of the left knee. After reduction in pathological bone turnover, the patient was planned for a total knee replacement. As a standard intramedullary femoral jig was not applicable due to the femoral deformity, a computed topography-based 3D-printed patient-specific instrument was used. This custom jig was used to define and perform the distal femur cut at 90 degrees to the mechanical axis of the femur in the coronal and sagittal plane. Postoperatively, the patient did well and achieved good function and pain relief. Conclusion: The use of a 3D-printed PSI for complex primary knee arthroplasty is an excellent option with no additional operative time than a conventional knee arthroplasty. Although a robotic or computer-navigated TKA would be an excellent option in this case, we restored the limb alignment using a cost-effective patient-specific femoral jig. This could be a viable option in centers without navigation or robotic arthroplasty.

Comparison of Phenytoin and Fosphenytoin in Treatment of Active Seizures in the Emergency Department.

Background and Objective: Ongoing seizure in the Emergency Department is a medical emergency and its aggressive management is essential. Prompt antiepileptic therapy with early cessation of seizure would minimize the morbidity and risk of recurrence. To compare time to seizure control with fosphenytoin to phenytoin protocol in the ED. Materials and Methods: We conducted an observational study on patients with active seizure in the Emergency Department comparing phenytoin versus fosphenytoin protocol over one year. Results: During the study period, we recruited 121 patients in the phenytoin group and 124 patients in the fosphenytoin group. Generalized tonic-clonic seizure (73.5% in phenytoin vs. 68.5% in fosphenytoin arm) was the most common type of seizure in both the arms. The mean time taken for cessation of seizure in the fosphenytoin arm (17.48 ± 49.24) was less than half of that in the phenytoin arm (37.20 ± 58.17) (mean difference: 19.72, P = 0.004, 95% CI: -33.27 to -6.17). There was a significant decrease in recurrence rates of seizure with phenytoin compared to the fosphenytoin arm (17.7% vs. 31.4%: OR: 0.47, P = 0.013; 95% CI: 0.26-0.86). Favorable STESS (≤2) was higher with phenytoin compared to fosphenytoin (60.3% vs. 48.4%). The overall in-hospital mortality rate in both arms was negligible (0.8%). Conclusion: The mean time for cessation of active seizure with fosphenytoin was less than half that of phenytoin. Despite its higher cost and minor adverse effects when compared to phenytoin, benefits seem to outweigh its limitation.