Significant Residual Deficits Research Articles

Are Functional Performance Test Scores Better When Compared to Baseline or Contralateral Limb Scores Following LE Injury in Adolescent Athletes?

Functional performance tests (FPT) have been used with athletes following an injury to determine readiness to return-to-play (RTP), usually using limb symmetry indices to the contralateral limb or a baseline score. There is not a consensus as to which criterion scores are best compared. This study aimed to compare common functional performance test scores from injured athletes at the time of release to RTP to both preseason baseline scores and to the contralateral limb. It was hypothesized that using baseline scores for comparison would be more responsive to residual deficits following injury than using the contralateral limb. Prospective longitudinal cohort study. High school athletes (n=395) from all varsity sports completed a battery of FPTs including the Y-Balance Test (YBT), single limb hop tests and T-Test for agility (TT) during their preseason to establish baseline data. Injured athletes (n=19) were re-tested using all FTP's again at the time of RTP. Paired t-tests were used to detect if significant (p\<0.05) residual deficits were present at time of RTP when compared to baseline and to contralateral scores on FPTs. Differences in YBT scores were found in the anterior direction only (p=0.021) when comparing RTP to preseason, but there were no differences when compared to RTP data for the contralateral limb. Differences were detected with the single leg hop test (p = 0.001) when comparing the RTP to preseason and were also detected in both the single leg hop (p= 0.001) and triple hop (p=0.018) when compared to the contralateral limb. Differences in TT scores were detected when comparing RTP to preseason for cutting first with both the unaffected (p = 0.019) and affected (p = 0.014) limbs. The YBT in the anterior direction and the TT are better able to detect residual deficits when comparing RTP to preseason scores. Hop tests are better able to detect deficits when compared to the contralateral limb. These results could make preseason testing more efficient when creating a reference for determining RTP readiness following lower extremity injury.

Guillain-Barré Syndrome.

This article summarizes the clinical features, diagnostic criteria, differential diagnosis, pathogenesis, and prognosis of Guillain-Barré syndrome (GBS), with insights into the current and future diagnostic and therapeutic interventions for this neuromuscular syndrome. GBS is an acute, inflammatory, immune-mediated polyradiculoneuropathy that encompasses many clinical variants and divergent pathogenic mechanisms that lead to axonal, demyelinating, or mixed findings on electrodiagnostic studies. The type of antecedent infection, the development of pathogenic cross-reactive antibodies via molecular mimicry, and the location of the target gangliosides affect the subtype and severity of the illness. The data from the International GBS Outcome Study have highlighted regional variances, provided new and internationally validated prognosis tools that are beneficial for counseling, and introduced a platform for discussion of GBS-related open questions. New research has been undertaken, including research on novel diagnostic and therapeutic biomarkers, which may lead to new therapies. GBS is among the most frequent life-threatening neuromuscular emergencies in the world. At least 20% of patients with GBS have a poor prognosis and significant residual deficits despite receiving available treatments. Research is ongoing to further understand the pathogenesis of the disorder, find new biomarkers, and develop more effective and specific treatments.

Intracranial invasive group A streptococcus: a neurosurgical emergency in children.

Invasive group A streptococcus (iGAS) infections are associated with a high rate of morbidity and mortality. CNS involvement is rare, with iGAS accounting for only 0.2%-1% of all childhood bacterial meningitis. In 2022, a significant increase in scarlet fever and iGAS was reported globally with a displacement of serotype, causing a predominance of the emm1.0 subtype. Here, the authors report on iGAS-related suppurative intracranial complications requiring neurosurgical intervention and prolonged antibiotic therapy. The authors performed a retrospective chart review of consecutive cases of confirmed GAS in pediatric neurosurgical patients. Five children with a median age of 9 years were treated for intracranial complications of GAS infection over a 2-month period between November 2022 and December 2022. All patients had preceding illnesses, including chicken pox and upper respiratory tract infections. Infections included subdural empyema with associated encephalitis (n = 2), extradural empyema (n = 1), intracranial abscess (n = 1), and diffuse global meningoencephalitis (n = 1). Streptococcus pyogenes was cultured from 4 children, and 2 were of the emm1.0 subtype. Antimicrobial therapy in all patients included a third-generation cephalosporin but varied in adjunctive therapy, often including a toxin synthesis inhibitor antibiotic such as clindamycin. Neurological outcomes varied; 3 patients returned to near neurological baseline, 1 had significant residual neurological deficits, and 1 patient died. Despite the worldwide increased incidence, intracranial complications remain rarely reported resulting in a lack of awareness of iGAS-related intracranial disease. Awareness of intracranial complications of iGAS and prompt referral to a pediatric neurology/neurosurgical center is crucial to optimize neurological outcomes.

Tumefactive brain parenchymal neurosarcoidosis.

Enhancing brain parenchymal disease, and especially tumefactive lesions, are an uncommon manifestation of neurosarcoidosis. Little is known about the clinical features of tumefactive lesions and their impact on management and outcomes, which this study aims to characterize. Patients with pathologically-confirmed sarcoidosis were retrospectively reviewed and included if brain lesions were: (1) intraparenchymal, (2) larger than 1cm in diameter, and (3) associated with edema and/or mass effect. Nine patients (9/214, 4.2%) were included. Median onset age was 37years. Diagnosis was confirmed by brain parenchymal biopsies in 5 (55.6%). Median modified Rankin scale (mRS) score was 2 (range 1-4) at initial presentation. Common manifestations included headache (77.8%), cognitive dysfunction (66.7%), and seizures (44.4%). Sixteen lesions were present in 9 patients. The frontal lobe (31.3%) was most affected, followed by the subinsular region (12.5%), basal ganglia (12.5%%), cerebellum (12.5%), and pons (12.5%). MRI characteristics of the dominant lesions included spherical morphology (77.8%), perilesional edema (100.0%), mass effect (55.6%), well-demarcated borders (66.7%), and contrast enhancement (100.0%; 55.6% heterogeneous). Leptomeningitis was frequently present (77.8%). All required corticosteroid-sparing treatments, and most (55.6%) needed at least a third line of treatment (infliximab used in 44.4%). All patients relapsed (median 3 relapses, range 1-9). Median last mRS was 1.0 after median follow-up of 86months, with significant residual deficits in 55.6%. Tumefactive brain parenchymal lesions are uncommon, usually affect the supratentorial brain along with leptomeningitis, and are refractory to initial treatments with a high risk of relapse. Significant sequelae were encountered despite a favorable median last mRS.

Tumefactive Neurosarcoidosis (P9-5.007)

<h3>Objective:</h3> To define and characterize tumefactive neurosarcoidosis, a unique phenotype of neurosarcoidosis distinct from the more commonly seen smaller, perivascular, subpial parenchymal lesions. <h3>Background:</h3> Enhancing brain parenchymal disease is an uncommon manifestation of neurosarcoidosis (10–27%). Intraparenchymal tumefactive lesions are even rarer (6–8%). As no studies dedicated to tumefactive lesions have been published, their clinical characteristics and impact on management and outcomes are unknown. <h3>Design/Methods:</h3> Patients were included if: 1) lesions were within brain parenchyma, 2) the lesion’s largest dimension was greater than 1 cm, 3) perilesional edema and/or mass effect were present, and 4) sarcoidosis was confirmed by neural or extraneural biopsies. <h3>Results:</h3> Nine patients were included with a median age of 37 years. Pathological confirmation came from brain parenchymal biopsies in 5 (55.6%) and extraneural tissues in the remainder. Isolated neurosarcoidosis was seen in 1 patient (11.1%). Only 1 patient (11.1%) was known to have systemic sarcoidosis previously at the time the parenchymal mass was evaluated. Median modified Rankin scale (mRS) score at nadir was 2 (range 1–4). Common manifestations included headache (77.8%), cognitive dysfunction (55.6%), and seizures (44.4%). MRI characteristics included spherical morphology (77.8%), perilesional edema (100.0%), mass effect (55.6%), well-demarcated lesions (66.7%), and contrast enhancement (100.0%; 55.6% inhomogeneous). The frontal lobe (44.4%) was most commonly affected, followed by the subinsular region (22.2%), putamen (22.2%), and internal capsule (22.2%). Leptomeningitis was a frequent neuroinflammatory accompaniment (77.8%). All patients received steroids and required steroid-sparing treatments. 55.6% of patients required second- and third-line treatments. Infliximab was needed in 44.4%. After a median follow-up period of 86 months, the median last mRS was 1.0, with significant residual deficits in 44.4% of patients. <h3>Conclusions:</h3> Tumefactive neurosarcoidosis most commonly affects the supratentorial brain in patients without a known history of sarcoidosis, is typically associated with leptomeningitis, and often is refractory to initial treatments. <b>Disclosure:</b> Dr. Bou has nothing to disclose. Dr. El Sammak has nothing to disclose. Dr. Chien has nothing to disclose. Dr. Cavanagh has nothing to disclose. Dr. Hutto has nothing to disclose.

CASE OF TRANSVERSO-SIGMOID THROMBOPHLEBITIS WITH BILATERAL SUBDURAL EMPYEMA

Subdural empyema is a life-threatening infection of the intracranial space, with very high rates of mortality if not appropriately diagnosed and treated. When managed well, with surgery and antibiotics, patients usually recover without significant residual deficits. The route of spread of infection and the organism involved needs to be investigated for complete eradication of the disease.&#x0D; We present the case of a young girl, without any co-morbidities, initially diagnosed as having Cerebral Venous Sinus Thrombosis, and treated accordingly, who later suffered a cascade of complications that proved almost fatal. She was managed successfully at our institution, and recovered completely in the end, and is currently leading a normal life. A pathophysiological analysis of her disease process was made retrospectively, and indicated how a seemingly innocuous dental infection spread over time to produce a life-threatening subdural empyema, involving a fastidious opportunistic commensal organism. We present an account of the same.

Ocular Manifestations of Miller Fisher Syndrome: a Case Report

Background:Miller Fisher syndrome (MFS) is a variant of Guillain-Barré syndrome and is characterised by a clinical triad of ophthalmoplegia, ataxia and areflexia.Objectives:This report presents an atypical case of MFS characterized by ocular and gastrointestinal involvement, and anti-ganglioside antibody-positivity.Methods:A 17-year old boy was referred to our ophthalmology emergency room with signs and symptoms of diplopia and upper lid ptosis of the right eye. He underwent a complete ophthalmologic examination with special reference to strabologic status, as well as a neuropediatric examination with serum antiganglioside antibody panel.Results:Strabologic examination showed horisontal diplopia (near and far), ptosis of the upper eyelid on the right and bilateral ophthalmoplegia (limited elevation). Orthoptic examination revealed esotropia of 8 prism dioptres (PD) at near and 18 PD at far distance. A pediatric neurologist found normal limb power, deep tendon reflexes and flexor plantar responses, but attenuated right patellar reflex. Serum anti-GQ1b IgG (+++), anti-GQ1b IgM (++) and anti-GD1a IgM(++) were positive. Positivity of anti-GQ1b IgG antibody confirmed the existence of incomplete MFS. We treated the patient with systemic intravenous immunoglobulins for five days, and after five months of follow-up, all symptoms resolved.Conclusion:MFS can present itself as a wide range of clinical features and its timely recognition is important. Despite the alarming nature of the disease, patients with MFS tend to have a good recovery of presented symptoms, and without any significant residual deficit.

Post-ARDS Pulmonary Manifestations in COVID-19 Patients: Assessment of Risk Factors and Treatment Approaches

Background: Mixed radiological pulmonary manifestations with persistent and even progressive dyspnea are the possible consequences of COVID-19 and ARDS and the most worrying long-term complications. Such patients are at high risk for pulmonary fibrosis and non-reversible lung dys-function. The aim of this study was the assessment of risk factors and possible approaches to the treatment of such residual complications in COVID-19 and ARDS patients. Methods: Our study was randomized and controlled. Ninety seven patients were assessed at pul-monary and critical care departments of University Hospital, Baku city 6 weeks after discharge from COVID-19 hospitals. All patients have been treated in ICU for acute respiratory distress syndrome (ARDS) related to severe COVID-19 infection. All patients had persistent, nonimproving symptoms, particularly dyspnea. Depending on residual fibrotic and non-fibrotic changes in lungs all patients have been divided in two groups: 1) 39 patients with lung involvement (50-75%); 2) 58 patients with lung involvement more than 75%. Results: At 6 weeks after discharge all patients reported persistent symptoms: dyspnea 100,0% (97/97), cough 42,3% (41/97), chest pain 51,5% (50/97), fever 29,0% (28/97) and hemoptysis 18,5% (18/97) were assessed. Pulmonary abnormalities were found in all subjects; however, most intensive abnormalities were noticed in intubated patients with severe ARDS (OR 3,75[0,91-8,44] 95% CI; p﹤0,001). In all patients persistent lung function impairment were evaluated. Severity of lung function deficiency was depending on severity of ARDS (p﹤0,001). Follow-up CT chest was done following negative PCR result after 12 weeks to assess degree of recovery and residual fibrotic and non-fibrotic changes. Pulmonary fibrosis has been seen in all patients with lung involvement more than 75% (OR 2,45 [0,81-6,29] 95% CI; p﹤0,001), and organ-izing pneumonia although seen commonly in patients with massive lung involvement (OR 4,24 [0,94-9,26] 95% CI; p﹤0,001). Traction bronchiectasis was higher in patients with massive lung involvement compared to non-massive (OR 2,11 [0,72-5,01] 95% CI; p﹤0,004). CT chest finding like ground glass opacities, consolidation, vascular and bronchial thickening were similar in both group patients (p﹥0,05). Lack of use dexamethasone in acute phase of the disease was higher in patients with lung involvement more than 75% (p﹤0,004) and lack of use prone position ventilation also was one of risk factors for excessive lung involvement (p﹤0,01). Invasive mechanical ventilation incidence was higher in massive lung involvement ((OR 2,84 [0,76-6,44] 95% CI; p﹤0,002). Lack of use HFNC also was associated with higher incidence of more massive lung involvement and development of pulmonary fibrosis (p﹤0,01) and organizing pneumonia (p﹤0,001).Conclusions: Following post-ARDS COVID-19 disease the patients have significant residual radiological inflammatory lung disease, persistent ventilation and functional deficit. There were several risk factors associated with more severe lung fibrotic and non-fibrotic residual changes. Treatment with corticosteroids at the time of ICU admission associated with decrease of extensity of radiological changes and functional impairment.

Promising recovery biomarkers after first event acute demyelinating optic neuritis

BackgroundAcute demyelinating optic neuritis is often a first episode of subsequent CNS demyelinating disease, most frequently multiple sclerosis (MS). The majority of the patients have substantial recovery of the high contrast visual acuity (HCVA) and 30-2 perimetry, but reduced contrast sensitivity persists in 56% of eyes after an episode of optic neuritis. Cross sectional studies of MS patients show low contrast visual acuity (LCVA) correlates modestly with RNFL thickness and macula ganglion cell +IPL layer (GCL) thickness. Considering the potential severity of the vision deficits at onset, and GCL thinning at outcome, we hypothesized 10-2 perimetry and LCVA deficits would be frequent following an episode of optic neuritis. MethodsWe prospectively studied 32 eyes of 32 patients (9 men, 23 women, age 34 years ± 10) with first time acute optic neuritis. We measured LCVA 2.5% (# letters seen), GCL thickness and loss, and 10-2 mean deviation (MD in decibels, dB) within 15 days from symptoms onset and at six months. The 10-2 threshold perimetry, a novel assessment in the MS research field, included to further characterize central vision function. We used correlation analysis to assess associations between GCL thickness and thinning and 10-2 perimetry as well as LCVA 2.5% at six months. ResultsCompared to fellow eyes we found significant residual LCVA deficits in 28/32 study eyes at 6 months with 12.6 ± 15.8 letters seen, p=0.001). Similarly, 10-2 MD threshold perimetry in the study eyes at 6 months showed significant difference relative to the fellow eyes, p=0.01.In regards to GCL changes at 6 months, we found statistically significant reduction of the GCL thickness in the study eyes relative to the fellow eyes (study eyes 69.6 ± 9.6 µm, fellow eyes 82.7 ± 4.7 µm, p=0.001), with thinning present in 29/32 eyes. The mean GCL thinning at 6 months in the study eyes was 12.4 ± 8.4 µm.Correlation analysis of associations between primary outcome measures at 6 months showed significant relationship between GCL thickness and 10-2 MD threshold perimetry (0.43, p = 0.015) but not with LCVA. At six months, the mean GCL thinning strongly correlated with the 10-2 MD (-0.60, p=0.01) and moderately with LCVA (-0.46, p=0.008). ConclusionsGCL thickness is the best structural and LCVA and 10-2 MD are sensitive functional measures for determining residual deficits due to optic neuritis. The 10-2 MD correlates best with the outcome GCL thickness and loss. Our findings also suggest that 10-2 threshold perimetry and macular GCL can provide functional and structural promising biomarker signals to explore neuro-protective research.

Randomized trial of argatroban plus recombinant tissue-type plasminogen activator for acute ischemic stroke (ARAIS): Rationale and design

Previous studies have implied the efficacy and safety of argatroban plus recombinant tissue-type plasminogen activator (r-tPA) in patients with acute ischemic stroke. Further trials are needed to establish convincing conclusions in a large sample size. Argatroban plus r-tPA for Acute Ischemic Stroke (ARAIS) trial is a multicenter, prospective, randomized, open-label, and blind-end point trial. The trial proposes to randomize 808 patients with acute ischemic stroke National Institutes of Health Stroke Scale (NIHSS score≥ 6 at the time of randomization) within 4.5 hours of symptom onset to receive argatroban (100 μg/kg bolus followed by an infusion of 1.0 μg/kg per minute for 48 hours) plus r-tPA or r-tPA alone. The primary end point is the proportion of patients with an excellent outcome of no clinically significant residual stroke deficits (modified Rankin scale 0-1) at 90 days. Secondary end points include the proportion of patients with a good outcome (modified Rankin scale 0-2) at 90 days, early neurological improvement (NIHSS score ≥2-point decrease) at 48 hours, early neurological deterioration (NIHSS score ≥4-point increase) at 48 hours, decrease in the NIHSS score from baseline to 14 days, and stroke recurrence or other vascular events at 90 days. Safety end points include symptomatic intracerebral hemorrhage, parenchymal hematoma type 2, and major systemic bleeding. ARAIS trial will evaluate whether argatroban plus r-tPA is superior to r-tPA alone in improving functional outcomes in acute ischemic stroke patients in a large sample population.

Abstract WP145: Safety of Intra-Arterial Mesenchymal Stem Cells in a Large Animal Model of Stroke: A Dose Escalation Study

Background: Despite the efficacy of thrombectomy for large vessel occlusion acute ischemic stroke (AIS) , ~50% of patients have significant residual deficits. Pre-clinical data on intra-arterially (IA) administered mesenchymal stem cells (MSCs) in stroke are promising and this approach is attractive for clinical application. While there is a concern for micro-occlusion with IA delivery due to the large size of MSCs, a dose of 1 x 10 5 MSCs given 24-48 hr in a rodent reperfusion middle cerebral artery occlusion (rMCAo) model has been shown to be safe and effective. As per STAIR recommendations, we performed a dose-escalation (DE) study of IA-MSCs in a large animal stroke model. Methods: An endovascular canine rMCAo model using retractable platinum coil for 60-120 min was established. At 48 hr post-rMCAo, allogeniec canine MSCs were delivered using a 0.0165” microcatheter in the ipsilateral upper cervical internal carotid artery in escalating doses (based on proportion of rodent to canine total cerebral blood volume). Serial MRIs and neurological deficit scoring (NDS) were performed over 30 days. Animals were euthanized at 15-30 d post-rMCAo and brains were harvested. Results: Female canines (n=13), age 12-36 months, weighing 22-26 kg received IA MSCs ranging from 5-80 x 10 6 (M). At doses of 5-40 M IA-MSCs no neurological worsening was observed. Serial NDS and stroke volume on MRI showed no increase post-IA-MSCs and actually showed progressive reduction. A higher numerical reduction was seen in the 10-40 M groups compared to 5 M. However, in the one canine receiving 80 M IA-MSCs, there was significant worsening of the MCA-area infarction and NDS due to microembolization at this higher dose. Gross examinations and histopathology of brain tissue were consistent with ischemia. The brain of a canine receiving 80 M cells showed differentially aged areas of necrosis supporting two ischemic events. Neuroblasts, doublecortin-positive cells, and neovascularization were observed in canine brains suggesting regenerative mechanisms. Conclusions: These data suggest that IA-MSCs are safe in a large animal model up to 40 M IA-MSCs and is the maximum tolerated dose in this DE study. Furthermore, our data suggests that up to 40 M IA-MSCs may be promising in exploring efficacy in AIS.

Comparison of Clinical and Sociodemographic Characteristics of Patients with Schizophrenia Treated Stationary and at Day Hospital

Schizophrenia is a disorder that can disrupt practically every aspects of human functioning. It is a chronic disorder which can cause serious difficulties even at the level of elementary functioning for the affected individual and his/her family. Until twenty years ago, main goal of schizophrenia treatment was practically entirely directed on reducing positive symptoms, with significant residual deficits, such as negative symptoms, cognitive impairment, coping difficulties in everyday life situations, impaired social and working aspects of everyday life. Today the aim of treatment is deinstitutionalisation, fewer number of relapses, reduced number of hospitalizations, as well as improved personal, family, social and work functionality, which can all be achieved by treating patients suffering from schizophrenia in the day hospital setting, in other words – with partial hospitalization. The goal of this research was to investigate and compare differences and specificities of sociodemographic and clinical features in two groups of patients suffering from schizophrenia, those treated in a stationary setting and those who attended a day-hospital program. The study also attempted to identify and compare the differences between these two groups of patients, as well as compare the efficacy of treatment of the two treatment modalities while using a questionnaire designed specifically for the purpose of this research. The study included 60 patients suffering from schizophrenia, who formed two groups of 30 patients and was entirely performed at the Department of Psychiatry of KBC Sestre milosrdnice. The hypothesis of this research was that patients suffering from schizophrenia treated in a day hospital setting have fewer hospitalizations than patients treated in an acute psychiatric ward. Results confirmed this hypothesis, as there was a statistically significant difference in the number of hospitalizations between the two groups of patients. By comparing these two groups, it can be concluded that patients treated in the day hospital have a higher level of education, are less work impaired and and are treated at an early stage of their illness (up to 5 years of treatment) compared to patients treated at the acute psychiatric ward. Compared to patients treated in the day hospital setting, patients treated acutely in general have a lower level of education, increased hereditary loading and aggressiveness, as well as longer treatment duration (about 10 years) and discontinued (irregular) course of outpatient treatment. By comparing data obtained from these two groups of patients with schizophrenia we can shape guidelines that will help us in everyday nursing practice.

Postoperative radiation for primary melanoma of the cervical spinal cord in a pregnant patient: A case report &amp; review of the literature

Abstract Only 1% of melanomas present with isolated involvement of the central nervous system. Primary melanoma of the spinal cord is especially rare. There are only a handful of cases reported and only one reported during pregnancy. The patient was a 27-year-old female who presented with 16 weeks of neck pain and numbness in the distal upper extremities when she was 31 weeks pregnant. MRI revealed a mass in the cervical spine. The plan was for delivery at term followed by surgical resection. However, due to progression of symptoms, she underwent emergent Cesarean section. The procedure was performed without complications, but the patient demonstrated quadriplegia post-operatively. She then underwent surgical resection. Final post-operative imaging showed no gross disease. Pathology revealed malignant melanoma. She slowly regained neurologic function with no significant residual deficits. She received post-operative radiotherapy (37.5 Gy in 15 fractions using 3D-CRT) with minimal toxicity. Due to the rarity of primary melanoma of the spinal cord, there are no standardized guidelines for treatment. Presentation during pregnancy adds more complexity. Based on prior reports, primary melanoma of the spinal cord appears to have a better overall prognosis compared to primary cutaneous melanoma with spinal cord metastasis. Surgical resection is the mainstay of treatment. The role for radiotherapy is not clear. As a result, the optimal treatment for these patients should be determined on a case-by-case basis.

Disorders of the Cauda Equina.

Conditions that affect the cauda equina are a diverse group of disorders that require timely recognition and management. This article reviews cauda equina anatomy, the diagnostic approach to disorders of the cauda equina, features of cauda equina syndrome, and diskogenic and nondiskogenic disorders of the cauda equina. Establishing clinical criteria for cauda equina syndrome has been a focus of a number of reviews, although the clinician must maintain a low threshold for emergent imaging in cases of suspected cauda equina syndrome because of the suboptimal reliability of various signs and symptoms in identifying this condition clinically. The timing of surgical intervention for compressive causes of cauda equina dysfunction remains a point of contention, although urgent decompression remains standard practice. A recent review that focused on outcomes in patients with cauda equina compression who underwent surgical decompression identified significant residual deficits in patients despite appropriate and timely intervention. Autoimmune conditions targeting the cauda equina have been increasingly recognized, including chronic immune sensory polyradiculopathy and chronic immune sensorimotor polyradiculopathy. Disorders that affect the cauda equina require thoughtful and timely clinical examination and diagnostic testing to establish a definitive cause and an appropriate treatment approach.

Decompressive Hemicraniectomy Versus Medical Management in Cerebral Venous Thrombosis with Signs of Raised Intracranial Pressure

Background: Mass effect is the major cause of death in cortical venous sinus thrombosis and there is no clear cut the role of decompressive hemicraniectomy. Aims: To study the outcome of patients with large venous infarct and acutely increased intracranial pressure subjected to either conservative treatment or decompressive surgery. Materials & Methods: 20 consecutive patients admitted with moderate to large venous infarct were examined for features of raised intracranial pressure (ICP) . Cranial CT and or MRI and CT venogram or MR venogram and or cerebral DSA were examined for site of Sino venous occlusion. Results: There were 20 patients with 10 each in conservatively and surgically treated group. Cranial CT/MRI head revealed large venous infarct with midline shift and mass effect in all patients. Overall 15 of 16 survivors had good outcome at 3 months without any significant residual deficit irrespective of mode of treatment used. There is no statistical difference between medical and surgical groups in mRS 1 month, 3 months and death with p value 0.651, 0.185 and 0.474 respectively. Conclusion: Patient with large venous infarct with signs of raised intracranial pressure carries good overall outcome with timely care irrespective of conservative or surgical management.

An outbreak of Japanese encephalitis in a non-endemic region of north-east India.

Background There are few comprehensive reports of epidemic Japanese encephalitis in a previously unaffected region. We report our experience of a first-ever outbreak of it in Sonitpur District, Assam, India, with 45 laboratorycon confirmed cases at a single hospital. Method Between 2 July and 11 August 2008, patients meeting the WHO definition of acute encephalitis syndrome were assessed in a single hospital and had tests including blood and cerebrospinal fluid for Japanese encephalitis antibody titres. Results Ninety-six cases meeting the definition of acute encephalitis syndrome were identified with 45 cases of Japanese encephalitis confirmed by cerebrospinal fluid or blood results. For Japanese encephalitis positive patients, mean age was 36 (range 4-80). Of the 45, 11 (24%) died and a further 21 (47%) had significant residual deficits. Focal neurological signs (40%) and seizures (25%) were common. Conclusion An effective Japanese encephalitis vaccine is the key intervention for limiting the population impact of this disease. Identification of these cases led to a district-wide mass vaccination campaign.

Temporal trajectories of novel object recognition performance in mice exposed to intermittent hypoxia.

Intermittent hypoxia is one of the major perturbations of sleep-disordered breathing and has been causally implicated in neurocognitive deficits. However, the reversibility of such deficits is unclear.Male C57BL/6J mice were exposed to either intermittent hypoxia or room air for 3-240 days, and then half were randomly selected and allowed to recover in normoxic conditions for the same duration of the previous exposure. A novel object recognition (NOR) test was performed.NOR performance was stable over time in room air. Intermittent hypoxia induced significant reductions in recognition index that progressed over the first 45 days and stabilised thereafter. Normoxic recovery of recognition index was essentially complete and indistinguishable from room air in mice exposed to shorter intermittent hypoxia times (<90 days). However, significant residual deficits emerged after normoxic recovery following prolonged intermittent hypoxia exposures (p<0.01). In addition, gradual attenuation of the magnitude of recovery in recognition index occurred with increasingly longer intermittent hypoxia exposures (MANOVA p<0.0001).Intermittent hypoxia during the resting period reduces NOR performance in a time-dependent fashion. Reversal of NOR performance deficits is unlikely after prolonged intermittent hypoxia duration. These findings suggest that early recognition of sleep apnoea and effective treatment are critical for restoration of the adverse cognitive effects of the disease.

Cerebral Venous Sinus Thrombosis: Two Case presentations with different clinical manifestations and multifactorial etiology

Cerebral Venous Sinus Thrombosis (CVST) is a rare form of venous thrombosis which commonly affects young adults. CVST has variable clinical presentation such as headache, seizure, hemiparesis and even coma, thus mimicking stroke and other neurological disorders. Due to such non-specific wide spectrum of clinical presentations and variable etiology in young adults, CVST can be misdiagnosed easily, especially in initial stages of presentation which consequently affects the outcome and prognosis of the patient. Thus when a previously healthy young adults acutely presented with such variable but primarily neurological clinical presentation, it is vital for medical practitioner to have fine knowledge and skills to suspect CVST as a differential diagnosis. We present two cases: first case is a 21 year old female with puerperal CVST who had caesarean section under spinal anaesthesia three weeks before, who presented with headache and recurrent seizures, followed by hemiparesis; the second case is a 25 year old female with CVST, on oral contraceptives, who presented with right monoparesis, aphasia and facial asymmetry. In both cases though CVST was not the initial diagnosis, it was subsequently diagnosed early and confirmed by MRI brain and MRI angiogram and anticoagulation was commenced effectively, despite associated haemorrhagic infarct in the first case. Both patient made remarkable recovery without any significant residual neurological deficit.

Epileptogenesis in neurocutaneous disorders with focus in Sturge Weber syndrome.

Epilepsy is a major morbidity in Sturge Weber syndrome, a segmental vascular neurocutaneous disorder classically associated with facial angiomas, glaucoma, and leptomeningeal capillary-venous type vascular malformations. The extent of the latter correlates with neurological outcome. Post-zygotic mosaicism for the activating mutation p.R183Q of the GNAQ gene has been identified as the major cause. GNAQ encodes for an alpha subunit of a heterotrimeric G protein critical to blood vessel development. The earlier the timing of the mutation in development, the more severe the involvement, e.g. from isolated port-wine stains to the full syndrome. The strongest predictors of adverse outcomes are MRI and the presence of angiomas involving any part of the forehead, delineated inferiorly from the outer canthus of the eye to the top of the ear, and including the upper eyelid. The neurological course may be progressive and the typical constellation of symptoms is focal onset seizures, hemiparesis, headache, stroke-like episodes, behavior problems, intellectual disability, and visual field deficits. Antiseizure medications are effective in about half of patients. The presence of localized seizures, focal neurological deficits, and drug resistant epilepsy indicate epilepsy surgical evaluation. Earlier seizure onset, i.e. before six months of age, is associated with a more severe course with significant residual deficits. Factors contributing to epileptogenesis include decreased brain tissue perfusion due to abnormal venous drainage, anoxic injury contributing to cerebral calcification, breakdown of the blood-brain barrier, and the presence of developmental cortical malformations. Pre-symptomatic prophylactic treatment may be a future option to modify the course of the disease including the associated epileptogenesis.

Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm

A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral...