Systemic lupus erythematosus presenting with holocord myelitis.

This is a report of a case of a 25-year-old woman, who presented with a rapidly progressive sensory-motor flaccid quadriparesis which had developed over a few days along with bladder and bowel involvement. She had a past history of photosensitive rash and joint pains along with mild-to-moderate grade fever; for which she had never been evaluated. Serological markers for systemic lupus erythematosus (SLE) were strongly positive and helped in establishing the diagnosis of SLE-related holocord myelitis. High-dose intravenous glucocorticoid followed by intravenous pulse cyclophosphamide was used to treat her and there was a significant improvement. In this case report, the diagnosis of SLE was made for the first time in a patient presenting with holocord myelitis.

Stroke-related education to emergency department staff: An acute stroke care quality improvement initiative.

BACKGROUND: Acute ischemic stroke therapy is time sensitive, and optimum treatment is missed due to pre-hospital and/or in-hospital delay. MATERIALS AND METHODS: A prospective observational (before and after) study was conducted for 1 year.The study period was defined as phase-I or pre-education phase, phase-II or immediate post-education phase, and phase-III or delayed post-education phase, with each phase lasting for 4months. All consecutive stroke patients presenting within 12 hours of stroke onset were enrolled. Baseline and outcome data including acute stroke care quality matrices and functional outcomes were collected. RESULTS: A total of 264 patients were enrolled. All acute stroke care quality matrices improved significantly (P ≤ 0.01) with a median door to imaging time (DTI) of 114, 35, and 47 minutes in the three phases consecutively. In phase-II, proportions of patients imaged within 25 minutes of arrival increased by 35%. Mean door to needle (DTN) time were 142 ± 49.7,63.7 ± 25.1, and 83.9 ± 38.1 minutes in the three consecutive phases. Patients with DTN < 60 minutes of arrival increased by 63%. Modified Rankin score (mRS) at 3 months improved significantly in all ischemic stroke patients (P = 0.04) and patients with mRS of 0-2 increased by 22%. CONCLUSIONS: Stroke education to emergency department (ED) staff is an effective method to improve acute stroke care.

Clinical predictors of mechanical ventilation in Guillain-Barré syndrome.

BACKGROUND: Patients with Guillain-Barré syndrome (GBS) require assisted ventilation frequently. However, no single factor can predict ventilator requirement. AIMS: To identify clinical variables which could predict the need for mechanical ventilation in GBS. SETTINGS AND DESIGN: Tertiary hospital-based retrospective and prospective study. MATERIALS AND METHODS: One hundred and thirty-eight GBS patients studied were divided into two groups ventilated (Group 1) and non-ventilated (Group 2). Parameters assessed included age, gender, associated illness(es), antecedent events, first symptom at onset, time from onset to bulbar involvement, confinement to bed and peak disability, upper limb power and reflexes at nadir, presence of facial weakness, neck muscle weakness and autonomic dysfunction. STATISTICAL ANALYSIS: Multivariate predictors of ventilation were assessed using logistic regression analysis. RESULTS: There were 53 patients in Group 1 and 85 in Group 2. The mean age in the two groups was comparable. On bivariate analysis, simultaneous weakness of upper (UL) and lower (LL) limbs as the initial symptom (P<0.001); UL power less than Grade 3/5 at nadir (P<0.001); presence of neck and bulbar weakness (P<0.001); shorter duration from onset to bulbar weakness and confinement to bed (P=0.001) and bilateral facial involvement (P<0.01) were more frequently associated with the need for ventilation. Preserved reflexes in UL at nadir was significantly associated with absence of the need for mechanical ventilation (P<0.01). On multivariate analysis, factors independently associated with the need for mechanical ventilation included simultaneous motor weakness in UL and LL as the initial symptom (P=0.02), UL power<3/5 (Medical Research Council grade) at nadir (P=0.013) and presence of bulbar weakness (P<0.001). Preserved reflexes in the UL at nadir was independently associated with a lesser need for ventilation (P=0.001). CONCLUSIONS: Comprehensive assessment of clinical features may predict the need for mechanical ventilation in patients of GBS.

Epilepsy surgery in a pediatric population: a retrospective study of 129 children from a tertiary care hospital in a developing country along with assessment of quality of life.

PURPOSE: To assess the outcome of a pediatric population operated for drug-resistant epilepsy from a large tertiary care center in India. METHODS: Retrospectively: quality of life (QOL); prospectively: preoperative assessment included interictal EEG, MRI (as per epilepsy protocol), video-EEG. Ictal SPECT (with subtraction) and PET were performed when required. QOL scores were assessed using the HASS or SSQ for seizure severity, Quality of Life in Childhood Epilepsy (QOLCE) for QOL, and Child Behavior Check List (CBCL) for behavior. RESULTS: 142 were operated from January 2000 to June 2011 by the senior author. 118 patients with at least 1 year of follow-up were included in the study. Mean age at surgery was 9.8 ± 4.3 years. In addition, 40 patients underwent QOL assessment prospectively both before and after surgery. Mean duration of epilepsy was 5.3 ± 3.3 years. A class I outcome (Engel's) was seen in 79.5% patients, class II in 8.6%, class III in 10.7%, and class IV in 1 patient. As per surgical procedures, class I outcome in patients who underwent temporal resection, hemispherotomy and extratemporal resection was 76, 87 and 72%, respectively. QOL scores correlated with duration of seizures, epileptic encephalopathy and outcome of surgery, but not with side of surgery, age and sex. CONCLUSIONS: This study, the largest reported from India, has demonstrated satisfactory results for epilepsy surgery in children.

Association of allergic patients' phenotypes with IgE reactivity to recombinant pollen marker allergens.

BACKGROUND: During the last decade allergen molecules from several allergen sources have been produced by recombinant DNA technology. The aim of this study was to investigate whether IgE reactivity to recombinant pollen allergens with broad and narrow cross-reactivity is associated with clinical phenotypes of allergic sensitization. METHODS: Serum IgE reactivity to a panel of six recombinant birch and grass pollen allergens was measured by ELISA in pollen sensitized patients from Central Europe to define groups of patients with exclusive IgE reactivity to rBet v 1, with exclusive reactivity to major grass pollen allergens (rPhl p 1, rPhl p 2, rPhl p 5) and with IgE reactivity to cross-reactive pollen allergens (rBet v 2, rPhl p 7). Patients' clinical phenotypes were recorded. IgE responses to tree, grass and weed pollen as well as plant food extracts were evaluated in vitro by CAP-FEIA and clinical sensitivities were confirmed in vivo by skin prick testing. RESULTS: IgE reactivity to the recombinant major birch pollen allergen, rBet v 1, was associated with sensitization to pollen from birch, taxonomically related trees and to certain plant-derived food. Reactivity to the recombinant timothy grass pollen allergens, rPhl p 1, rPhl p 2, rPhl p 5, indicated sensitization to pollen from grasses. Patients reacting with the highly cross-reactive allergen rPhl p 7 were polysensitized to pollen from unrelated trees, grasses and weeds and rBet v 2-positive patients were polysensitized to pollen and plant-derived food from unrelated plants. CONCLUSIONS: IgE reactivity to recombinant marker allergens is associated with clinical phenotypes of allergic sensitization and may be useful for the selection of treatment strategies.

Surgery for medically intractable epilepsy due to postinfectious etiologies.

Medically intractable epilepsy (MIE) resulting from postinfectious etiologies requiring surgery have been uncommonly reported. A series of 28 cases are presented (hospital prevalence 5.7%). The mean duration of epilepsy prior to surgery was 8.2 +/- 2.1 years. The mean time of onset of epilepsy after central nervous system infection was 1.4 +/- 0.9 years (range 0-19 years). The pathologies included postpyogenic meningitic/encephalitic sequel (8), neurocysticercosis (6), tuberculomas/posttuberculous etiology (4), postpyogenic abscess of otogenic etiology (4), posttraumatic abscess-related gliosis (2), and gliosis of unknown infectious etiology (4) cases. Surgery included mesial temporal (11), lateral temporal (4), frontal (9), parietal (2) resections and hemispherotomy (1). Hippocampal sclerosis was seen in nine cases (4 neurocysticercosis) and this occurred in younger persons as compared to neocortical epilepsies. Good outcome (Engel class I and II) was seen in 23 of 28 cases (Engel class I in 17).

Idiopathic hypertrophic cranial pachymeningitis and dural sinus occlusion: two patients with long-term follow up.

Idiopathic hypertrophic cranial pachymeningitis (IHPM) is a clinicopathological entity characterized by thickening and fibrosis of the dura mater with resultant clinical symptoms. It is generally steroid responsive and has a tendency to remit and relapse. We present here two patients with IHPM with associated dural sinus occlusion and describe their clinicoradiological features and long-term course and outcome.

Corticosteroids for managing tuberculous meningitis.

BACKGROUND: Tuberculous meningitis, a serious form of tuberculosis that affects the meninges covering the brain and spinal cord, is associated with high mortality and disability among survivors. Corticosteroids have been used as an adjunct to antituberculous drugs to improve the outcome, but their role is controversial. OBJECTIVES: To evaluate the effects of corticosteroids as an adjunct to antituberculous treatment on death and severe disability in people with tuberculous meningitis. SEARCH STRATEGY: In September 2007, we searched the Cochrane Infectious Diseases Group Specialized Register, CENTRAL (The Cochrane Library 2007, Issue 3), MEDLINE, EMBASE, LILACS, and Current Controlled Trials. We also contacted researchers and organizations working in the field, and checked reference lists. SELECTION CRITERIA: Randomized controlled trials comparing a corticosteroid plus antituberculous treatment with antituberculous treatment alone in people with clinically diagnosed tuberculosis meningitis and which include death and/or disability as outcome measures. DATA COLLECTION AND ANALYSIS: We independently assessed search results and methodological quality, and independently extracted data. We analysed the data using relative risks (RR) with 95% confidence intervals (CI) and the fixed-effect model. We also conducted complete-case and best-worst case analyses. MAIN RESULTS: Seven trials involving 1140 participants (with 411 deaths) met the inclusion criteria. All used dexamethasone or prednisolone. Overall, corticosteroids reduced the risk of death (RR 0.78, 95% CI 0.67 to 0.91; 1140 participants, 7 trials). Data on disabling residual neurological deficit from three trials showed that corticosteroids reduce the risk of death or disabling residual neurological deficit (RR 0.82, 95% CI 0.70 to 0.97; 720 participants, 3 trials). Adverse events included gastrointestinal bleeding, bacterial and fungal infections and hyperglycaemia, but they were mild and treatable. AUTHORS' CONCLUSIONS: Corticosteroids should be routinely used in HIV-negative people with tuberculous meningitis to reduce death and disabling residual neurological deficit amongst survivors. However, there is not enough evidence to support or refute a similar conclusion for those who are HIV positive.

Hyperacute thrombolysis with IV rtPA of acute ischemic stroke: efficacy and safety profile of 54 patients at a tertiary referral center in a developing country.

BACKGROUND: Given the constraints of resources, thrombolysis for acute ischemic stroke (AIS) is under evaluation in developing countries. Prothrombin time (PT), platelet count and activated partial thromboplastin time (aPTT) may not be feasible within the time window. AIM: To evaluate the safety and efficacy of thrombolysis in selected patients without the coagulation profile. DESIGN: Open, nonrandomized, observational study. MATERIALS AND METHODS: Fifty-four stroke patients were classified using TOAST criteria (large artery atherosclerotic = 13; cardioembolic = 12; small vessel occlusion = 22; other determined etiology =three; undetermined etiology = four). The mean time to reach emergency was 2.4h (1.15-3.4), the mean door to CT, 24 min (10-47) and the door to recombinant tissue plasminogen activator (r-tPA) injection, 26.8 min (25-67). The NIHSS scores ranged from 11 to 22 (mean = 15.5 +/- 2.7). Patients with history of liver or renal disease or those on anticoagulants were excluded. The PT, aPTT and platelet count were not done. Recombinant tissue plasminogen activator was administered at a dosage of 0.9 mg/Kg. RESULTS: Thirty-five patients (65%) significantly improved on NIHSS at 48 h (> or =4 points) (mean change = 10; range= 4-17). At one month, 43 (79%) improved on Barthel Index (mean change = 45%). One each developed small frontal lobe hemorrhage and recurrent stroke; one died of aspiration; and eight showed no improvement. CONCLUSIONS: Hyperacute thrombolysis was found useful and safe in selected patients with AIS even without the coagulation studies.

Dengue fever with papilledema: a case of dengue-3 virus infection in central nervous system.

BACKGROUND AND OBJECTIVES: Neurological manifestations of dengue fever are rarely reported during acute illness and clinical presentation commonly observed is of acute encephalitis or one of the post-infectious immune mediated manifestations. We describe a case of dengue fever having mild encephalopathy and papilledema at presentation. CASE REPORT: Twenty-year-old female presented with fever, headache and vomiting. On examination she did not have classical signs of dengue fever and was found to have bilateral papilledema on fundus examination. Detailed work-up did not reveal any other cause of papilledema. Diagnosis of dengue fever was established by blood IgM antibody test on day 7 of illness. Retrospective analysis of CSF (drawn on day 5 of illness) by RT-PCR assay showed a characteristic band of dengue-3 virus. Papilledema was transient and subsided following symptomatic treatment. The patient recovered from acute illness and follow-up was unremarkable. CONCLUSION: Especially in dengue endemic areas, in the patients having acute febrile illness with subtle signs and symptoms suggestive of CNS involvement, dengue virus infection should also be ruled out early in the clinical course.

Mapping of the antigenic and allergenic epitopes of Lol p VB using gene fragmentation.

The recombinant proteins of Lol p VA and Lol p VB expressed in E. coli reacted with IgE antibodies from sera of allergic patients and mAbs FMC A7 and PpV1. Cross-absorption analyses using these recombinant proteins showed that Lol p VA and Lol p VB possess both similar and unique IgE binding determinants. Gene fragmentation was utilized to localize the antigenic and allergenic determinants of Lol p VB. When full-length cDNA of Lol p VB was digested into three fragments and expressed as the fusions from the glutathione transferase of pGEX vectors, fragments Met1-Val196 and Asp197-Val339 bound IgE while fragment Met1-Pro96 did not. The data suggest that there are at least two IgE binding determinants in Lol p VB. In addition, only fragment Met1-Val196 reacted with mAb PpV1. The localization of these determinants was further resolved using random fragment expression libraries. The mAb PpV1 determinant was near the N-terminal region of Lol p VB molecule. The IgE binding determinants were distributed in the central region: region I (amino acids 111-195) and II (199-254). These IgE binding determinants are conserved in Lol p VA.

Immunological relationships among group I and group V allergens from grass pollen.

Specific IgE antibodies have been affinity-purified from recombinant grass pollen allergens, and used to identify isoforms of the two major allergens of rye-grass pollen, Lol p I and Lol p V and cross-reactive allergens in other grasses. Lol p I-specific IgE (affinity-purified from the recombinant protein expressed by clone 13R which encodes amino acids 96-240 of Lol p I) identified four isoforms of the allergen. The same probe recognized cross-reactive epitopes in pollen proteins from 14 out of 16 grasses. The allergens identified by Lol p V-specific IgE (affinity-purified from the recombinant protein expressed by clones 12R or 19R which encode the full Lol p V protein) varied more in their physicochemical characteristics than the Group I isoforms. At least eight isoforms of Lol p V were identified by the Lol p V-specific IgE. The same probe recognized cross-reactive epitopes in pollen protein from 13 out of 16 grasses. Group I proteins were identified in grasses from two sub-families of the Poaceae, while the Group V allergens were only identified in pollen of grasses from one sub-family, the Pooideae.

Molecular basis of IgE-recognition of Lol p 5, a major allergen of rye-grass pollen.

Grass pollen, especially of rye-grass (Lolium perenne). represents an important cause of type I allergy. Identification of IgE-binding (allergenic) epitopes of major grass pollen allergens is essential for understanding the molecular basis of interaction between allergens and human IgE antibodies and therefore facilitates the devising of safer and more effective diagnostic and immunotherapy reagents. The aim of this study was to identify the allergenic epitopes of Lol p 5, a major allergen of rye-grass pollen, immunodissect these epitopes further so that the amino acid residues critical for antibody binding can be determined and investigate the conservation and nature of these epitopes within the context of the natural grass pollen allergens. Peptides, 12-13 amino acid residues long and overlapping each other by 4 amino acid residues, based on the entire deduced amino acid sequence of the coding region of Lol p 5, were synthesised and assayed for IgE-binding. Two strong IgE-binding epitopes (Lol p 5 (49-60) and (265-276), referred to as peptides 7 and 34, respectively) were identified. These epitopes were further resolved by truncated peptides and amino acid replacement studies and the amino acid residues critical for IgE-binding determined (Lol p 5 (49-60) residue Lys57 and (265-276) residue Lys275). Sequences of these epitopes were conserved in related allergens and may form the conserved allergenic domains responsible for the cross-reactivity observed between pollen allergens of taxonomically related grasses. Furthermore, due to its strong IgE-reactivity, synthetic peptide Lol p 5 (265-276) was used to affinity-purify specific IgE antibodies which recognised proteins of other clinically important grass pollens. further indicating presence of allergenic cross-reactivity at the level of allergenic epitope. Moreover, Lol p 5 (265 276) demonstrated a strong capacity to inhibit IgE-binding to natural rye-grass pollen proteins highlighting the antibody accessibility to these sequences within the context of the natural allergens. Strong IgE-binding epitopes of Lol p 5 have been identified down to single critical amino acid residues and are shown to occur as linear or continuous domains in the natural conformation of natural Lol p 5 and other group 5 grass pollen allergens. The fact that such an allergenic synthetic epitope has the capacity to strongly inhibit IgE-binding to natural allergens highlight its potential for use as a candidate in future therapeutics to treat pollen-associated allergies.

Study of the outcome of modified shoelace repair for midline incisional hernia.

AIMS AND OBJECTIVES: To study the efficacy and short-term sequelae of modified shoelace repair for midline incisional hernias. MATERIALS AND METHODS: A prospective non-randomized study of 30 cases of midline incisional hernias was carried out at a public hospital from May 2009 to Oct 2012. Patients underwent modified shoelace repair which comprises reconstruction of linea alba along with the use of polypropylene mesh to reinforce the facial layer. A proforma was maintained for each patient documenting patient details, nature of previous surgery and complications if any, postoperative course, and complications postincisional hernia repair. RESULTS AND DISCUSSION: Thirty patients underwent this repair with no complications and no recurrence for minimum follow-up period of 12 months. Our technique is a simple extra-peritoneal procedure with no extensive tissue dissection and avoids the potential complications of bowel injury and adhesions with the mesh. The principle involved is that during straining, the recti shorten by tonic contraction and approximate toward the midline. An incisional hernia weakens the midline and causes the recti to move laterally with contraction as medial pull is lost. By reforming a strong new linea alba, there is restoration of medial pull on recti. This is further buttressed by the mesh. CONCLUSION: Modified shoelace repair is a simple and safe extra-peritoneal procedure and can be used for all midline incisional hernias.