Signs Of Meningeal Irritation Research Articles

Clinical features of COVID-19-related encephalitis: comparison with the features of herpes virus encephalitis and autoimmune encephalitis.

Identifying coronavirus disease 2019 (COVID-19)-related encephalitis without clear etiological evidence is clinically challenging. The distinctions between this condition and other prevalent encephalitis types remain unknown. Therefore, we aimed to explore the similarities and differences in the clinical characteristics of COVID-19-related encephalitis and other encephalitis types. Adult patients with encephalitis admitted to the neurology department at Xuanwu Hospital were enrolled and categorized into the following six groups based on the results of metagenomic next-generation sequencing and autoimmune antibody detection in cerebrospinal fluid (CSF): COVID-19-related encephalitis (n = 36), herpes simplex virus type 1 encephalitis (HSV-1 encephalitis; n = 28), human herpesvirus 3 encephalitis (HHV-3 encephalitis; n = 10), NMDAR-antibody encephalitis (n = 18), LGI1-antibody encephalitis (n = 12), and GABAB-antibody encephalitis (n = 8). The predominant characteristics of COVID-19-related encephalitis include a low incidence of seizures (38.9%), cognitive defects (30.6%), and meningeal irritation signs (8.3%). Compared with HSV-1 and HHV-3 encephalitis, COVID-19-related encephalitis exhibited lower white blood cell count (2.5 count/mm3), protein (32.2mg/dL), and immunoglobulin M, G, and A levels (0.09, 3.2, and 0.46mg/dL, respectively) in the CSF tests. Abnormal imaging findings were present in only 36.1% of COVID-19-related encephalitis cases, mostly showing diffuse inflammation scattered in various parts, which differed from HSV-1 encephalitis. Additionally, COVID-19-related encephalitis exhibited significant differences in clinical symptoms and CSF white blood cell counts compared with NMDAR-antibody encephalitis; however, it showed limited differences compared with LGI1-antibody and GABAB-antibody encephalitis. COVID-19-related encephalitis and herpes virus or autoimmune encephalitis differ clinically. Symptoms and auxiliary examinations can be used as distinguishing tools.

Computed tomography and magnetic resonance imaging findings in central nervous system listeriosis.

To describe the imaging findings and determine the incidence of a characteristic worm-like pattern along the white matter tracts in neurolisteriosis on CT/MRI. An IRB-approved retrospective study in 21 consecutive neurolisteriosis cases during January 2002-July 2020. At least one of the following is required: (1) Positive Listeria monocytogenes (LM) in blood with clinical signs of meningeal irritation and/or abnormal CSF profile, (2) positive LM in blood with signs of encephalitis, (3) positive LM in CSF, (4) positive LM from brain biopsy/aspiration. Six cases were excluded due to the lack of contrast-enhanced images, leaving a total of 15 cases for analysis (mean age 53.5years ± 18.8 SD). The imaging studies were independently reviewed by two blinded readers. Demographic data, imaging findings, and incidence of the worm-like pattern were reported. The Cohen's kappa was used to calculate interrater reproducibility. Of the 12 patients with relevant imaging findings, nine cases (75%) had parenchymal lesions (eight cases in supratentorial compartment and one case in infratentorial compartment), four cases (33.3%) had leptomeningeal enhancement and two cases (16.7%) had hydrocephalus. Brain abscesses were found in eight cases and nodules evocative of abscess in one case. Restricted diffusion in the central area and hemosiderin deposition were observed in all cases. The involvement of white matter tract in a worm-like pattern was demonstrated in eight of nine patients with parenchymal lesions (88.9%). Abnormal findings in brain CT/MRI images are common in neurolisteriosis. The incidence of worm-like spread along the white matter tracts is high and may help diagnose suspicious patients.

Neuroimaging Changes and Treatment Outcome of Tubercular Meningitis- Experiences 22 Cases in a Tertiary Care Center

Background: Tuberculous meningitis (TBM) is caused by Mycobacterium tuberculosis (M. tuberculosis) and is the most common form of central nervous system (CNS) tuberculosis (TB).The prevalence of TB meningitis remains largely underestimated because clinical manifestations are nonspecific in early stages of the disease. Prompt diagnosis is critical for initiating appropriate therapy, facilitating measures to prevent dissemination of this highly contagious disease and to combat fatal complication in children Aims: To evaluate the role of neuroimaging changes and treatment outcome of tubercular meningitis. Methods: This prospective hospital-based cohort study was conducted in Department of Paediatric Neurology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, during January 2022 to December 2022. Children age belonged to 1 month to 10 year diagnosed as TBM during study period were enrolled in this study. Detailed history, clinical examination, CSF analysis and other relevant investigations were done. Data were recorded in standard questionnaire. Statistical analyses of the results were obtained by using window-based computer software devised with Statistical Packages for Social Sciences (SPSS-22). Results: Total 22 diagnosed case of TBM was enrolled and evaluated. Among them two third (63.63%) children were in 5-10 year age group. Male (59%) were outnumbered than female (36.36 %).Male female ratio was 1.6:1. More than half (59%) of patients came from urban area. All of the patients were vaccinated. Cent percent (100%) patients were presented with fever followed by headache (77.27%), sign of meningeal irritation (54.54%), vomiting (54.54%) and seizures (50%). Most common complication was hemiparesis (45.45%) followed by cranial nerve palsy (40.90%), visual problem (9.09%) and hydrocephalus (4.54%). Abnormal neuroimaging changes were found in 100% cases. Common findings were hydrocephalus (40.90%), tubercloma (36.36%), basilar enhancement (18.18%), Benign enlargement of subdural space & cerebellar hyperintensity (9%) and thalamic infract was present in 4.54% cases. Anti TB and steroid treatment were given in 100% cases and among them18.18% cases were required shunt procedure. More than one-third cases (40.90%) were completely normal without any no sequelae. Among abnormal sequelae found in two third (59%) cases. Speech impairment (36.36%) was most common followed by GDD (18.18%), hemiparesis (13.63%), quadriparesis (9.09%) and epilepsy in 9.09% cases. Conclusion: In our study all children of TBM were presented with fever followed by other predominant clinical features headache, vomiting, seizures and sign of meningeal irritation. Abnormal neuroimaging changes were found in all cases. Common findings were basilar enhancement, hydrocephalus, tubercloma, benign enlargement of subdural space & cerebellar hyper intensity and thalamic infract. After treatment more than one-third cases were completely normal without any no sequelae. J Bangladesh Coll Phys Surg 2024; 42: 214-218

Enterococcal Central Nervous System Infections in Children: A 22 Years' Experience in a Tertiary Center and Review of the Literature.

Enterococcal meningitis in children is rare, and its clinical presentation, laboratory characteristics and outcomes are not well defined. We conducted a retrospective analysis of Enterococcal meningitis cases during 2002-2023 at our tertiary center. We identified 10 cases in children aged 2 weeks to 15 years (median age: 8 months). Seven children were males and 9 had comorbidities, including a ventriculoperitoneal shunt in 5 children. All children with shunt infections presented with nonspecific signs and symptoms. While 8 children presented with fever, only 3 had signs of meningeal irritation and altered consciousness. Cerebrospinal fluid pleocytosis was evident in almost all children with a median of 173 cells/mL. Nine cases were due to Enterococcus faecalis, and 1 case was due to E. faecium. All 5 children with ventriculoperitoneal shunt underwent shunt removal and replacement. All children recovered without documented sequelae. Enterococcal meningitis is rare, especially in healthy neonates. It typically occurs following neurosurgical interventions and may only present with fever and shunt malfunction, without overt meningeal signs and with mild inflammation. The prognosis is favorable.

Outbreak analysis of meningococcal meningitis: A case series from a tertiary care hospital in Eastern India

In a span of 4 months, there were 7 cases of meningococcal meningitis who presented with diagnostic dilemma and also a few atypical manifestations. A retrospective data analysis was carried out in children diagnosed with meningococcal meningitis who were admitted from October 2022 to January 2023. There were 7 confirmed cases of Neisseria meningitidis infections during the study period. The median age of presentation was 6 years (range: 4.5 months–9 years) with four males and three females. All the children presented with fever, but none had signs of meningeal irritation as such. Routine blood and cerebrospinal fluid (CSF) analysis were also near normal. Reverse transcriptase polymerase chain reaction (RT-PCR) for N. meningitidis was positive in the CSF samples of all the seven cases. All of the patients recovered completely, except that there were residues of neurodeficit in one patient. In endemic areas, pediatricians should have a low threshold of suspicion for N. meningitidis in children presenting with signs and symptoms involving the central nervous system and consider an RT-PCR of the CSF sample even if routine blood and CSF studies are near normal.

Correlation between clinical-laboratory profile and outcome of AES cases in children: A hospital-based study

Objective: This study aims to determine the correlation between clinical as well as laboratory profile changes with the outcome of acute encephalitis syndrome (AES) cases. Materials and Methods: This prospective observational study was conducted in the Department of Pediatrics at Jorhat Medical College and Hospital, Assam over a period of 1 year where 49 diagnosed cases of AES in children aged between 1 month and 12 years were enrolled. Results: The study found that fever (100%) and altered sensorium (100%) were the most common clinical presentations in AES cases, followed by seizure (63%), headache (34.7%), vomiting (26.5%), diarrhea (22.4%), etc. Japanese encephalitis (JE) was the most common cause of AES (28.6%), followed by herpes simplex virus (4.1%), pyogenic meningitis (4.1%), and tubercular meningitis (2%), etc. However, the majority of cases (61.2%) were found to have an unknown etiology. Among the 49 cases, 27 (55.1%) recovered without neurological sequelae, 8 (16.8%) had neurological sequelae, and 14 (28.6%) died during the course of treatment. The most common neurological sequelae observed were motor deficit (37.5%), behavioral disorders, aphasia (25% each), and cranial nerve palsy. Glasgow coma scale (GCS) at the time of admission and during the time of hospital stay was found to have a significant (p<0.05) correlation with outcome in AES patients. Conclusion: The study concludes that AES cases commonly present with fever, altered sensorium, seizure, headache, vomiting, and signs of meningeal irritation. JE remains a major known cause of AES in children in this region of India. GCS at the time of admission and during the time of hospital stay were found to have a significant correlation with outcome. The neurological sequelae highlight the need for global attention to combat them save the lives of children.

A rare case of anti-DPPX encephalitis combined with neuroleptospirosis

BackgroundNeuroleptospirosis and anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis are both very rare and have only been reported in the form of respective case reports. There are no reports of anti-DPPX encephalitis combined with neuroleptospirosis in the literature. We reported the first case of neuroleptospirosis combined with elevated DPPX antibodies in serum and cerebrospinal fluid (CSF).Case presentationA previously healthy 53-year-old Chinese male farmer with a history of drinking raw stream water and flood sewage exposure was brought to the hospital due to an acute onset of neuropsychiatric symptoms. No fever or meningeal irritation signs were detected on physical examination. Routine laboratory investigations, including infection indicators, leukocyte and protein in CSF, electroencephalogram and gadolinium-enhanced magnetic resonance imaging of the brain, all revealed normal. While metagenomic next-generation sequencing (mNGS) identified the DNA genome of Leptospira interrogans in the CSF. Anti-DPPX antibody was detected both in blood and in CSF. A diagnosis of neuroleptospirosis combined with autoimmune encephalitis associated with DPPX-Ab was eventually made. He resolved completely after adequate amount of penicillin combined with immunotherapy.ConclusionWe highlight that in patients with acute or subacute behavioral changes, even in the absence of fever, if the most recent freshwater exposure is clear, physicians should pay attention to leptospirosis. Due to the low sensitivity of routine microscopy, culture, polymerase chain reaction and antibody testing, mNGS may have more advantages in diagnosing neuroleptospirosis. As autoimmune encephalitis can be triggered by various infections, neuroleptospirosis may be one of the causes of autoimmune encephalitis. Since neuronal antibody measurements themselves are not that common in neuroleptospirosis, future studies are needed to determine whether the detection of anti-DPPX antibodies is a rare event in leptospirosis. Early identification of autoimmune encephalitis and timely administration of immunotherapy may lead to a better outcome.

Acute psychosis as the presenting manifestation of lupus: case reports

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disorder that affects multiple organs with a wide range of clinical manifestations. Neuropsychiatric manifestation (NPSLE) is present among 28–40% of the SLE patients. Psychosis is rarely the only initial symptom of lupus, as neuropsychiatric lupus is often associated with systemic activity. We present two cases of young ladies presenting with acute psychosis as the predominant symptom and further diagnosed with SLE disease. Case reports: Case 1 A 20-year-old female, unmarried, presented to the Psychology Department with acute behavioral abnormality associated with staring, insomnia and, aggressive behavior, hearing voices for the last 3 days. No other psychiatric or neurological symptoms; she was taking 20 mg oral prednisolone prescribed according to a history of fatigue, low-grade fever, loss of appetite, pain in almost all joints, and myalgia for the last 1 month. There was no sign of focal neurological deficit or meningeal irritation. Examination of other systems did not find any clinical abnormality except for the presence of photosensitivity. A psychiatric consultation was ordered. Laboratory tests: white blood cells 3200 cell/mm3 (range=4000–11 000) with normal differentiation, hemoglobin 9.2 mg/dl (range=12.5–16), platelet 90 000/dl (range=150 000–400 000), C-reactive protein (CRP) 65 mg/l (n≤6), and erythrocyte sedimentation rate (ESR) 100 mm/h(range=0–20). anti-nuclear antibody (ANA) profile which was positive in 1:320 dilution, homogenous type, along with anti-ds-DNA positive (+), decreased C3 and C4. The diagnosis of SLE was done according to the European League Against Rheumatism/American College of Rheumatology Classification Criteria (EULAR/ACR Criteria). Case2 A 37-year-old married female with 3 children presented to the Psychology Department with disorientation, staring, hearing voices, seeing images of people who want to hurt her, refusing to respond to people, and insomnia for the last 10 days. She had complained of fatigue and low-grade fever for last month. A psychiatric consultation was ordered, and the patient was managed with an injection of haloperidol 5 mg BD. Laboratory tests: white blood cells 7300 cells/mm3 (range=4000–11 000) with normal differentiation, hemoglobin 8.7 mg/dl (range=12.5–16), platelet 90 000/dl (range=150 000–400 000), reticulocyte:1.4, CRP 7 mg/l (n≤6), and ESR 50mm/h (range=0–20). The ANA profile was positive in 1:320 dilution, homogenous type, along with the anti-ds-DNA negative (−), decreased C3, and normal C4. Brain magnetic resonance imaging (MRI) showed a high white matter signal around the lateral ventricles and in the semi-oval centers. Mild dilation of the lateral ventricles and slight elevation of the left insular and medial temporal lobe signal. Conclusion: Our case reports showed that organic disorders should be considered while evaluating acute psychosis, especially autoimmune disorders. The presence of laboratory features like anemia, elevated ESR, and elevated CRP should be taken with utmost importance.

Evaluation of Neurosurgical Emergencies in the Pediatric Emergency Department: Clinical Warning Signs

BackgroundWe aimed to evaluate the patients who underwent neuroimaging with suspicion of neurosurgery pathology and identify the clinical warning signs for the early recognition of neurosurgical emergencies. MethodsPatients aged one month to 18 years who underwent neuroimaging with a preliminary diagnosis of intracranial pathology requiring emergency surgery and symptom duration less than one month were included in the study. Patients were divided into three groups according to their definitive diagnosis as neurosurgical emergencies, neurological emergencies, and nonurgents. ResultsA total of 140 patients were included in the study (the median age was 8 [interquartile range IQR 3 to 13] years and 52.8% were male). Neurosurgery emergency group and neurological emergency group were significantly younger than the nonurgent group (P < 0.001). Vomiting, meningeal irritation findings, and papilledema (grade 2 and above) were more common in the neurosurgical emergency group (P 0.029, 0.023, and < 0.001, respectively). For neurosurgical emergencies, in the presence of papilledema (grade 2 and above) and focal neurological deficit, the specificity was 99.2%, positive predictive value (PPV) 83.3%, negative predictive value (NPV) 88.1%, and odds ratio (OR) 36.8 (P < 0.001, confidence interval [CI] 4.04 to 336.0); in the presence of altered consciousness and focal neurological deficit, the specificity was 97.5%, PPV 50%, NPV 86.6%, and OR 6.4 (P = 0.014, CI 1.20 to 34.4). ConclusionsYounger age, presence of vomiting, signs of meningeal irritation, papilledema grade 2 and above, and altered consciousness are the crucial “warning signs” of a potential neurosurgical emergency.

The clinical utility of metagenomic next-generation sequencing for the diagnosis of central nervous system infectious diseases.

To evaluate the clinical utility of metagenomic next-generation sequencing (mNGS) for the diagnosis of central nervous system infections (CNSI). Cerebrospinal fluid (CSF) from 54 patients who were high-level clinical suspicion of CNSI was collected and sent for mNGS and conventional tests from January 2019 to March 2022. Twenty out of 54 patients were diagnosed with CNSI and 34 non-CNSI. Among the 34 non-CNSI, one was false positive by mNGS. Among the 20 CNSI, 11 had presumed viral encephalitis and/or meningitis, 5 had presumed bacterial meningitis, 2 had presumed TMB, 1 had Crytococcus meningitis and 1 had neurosyphilis. The sensitivity of viral encephalitis and/or meningitis was 0.73 (8/11); 10 virus were detected; 9/10 was dsDNA; 1/10 was ssRNA. SSRN ranged from 1 to 13. The accuracy rate was 0.4, the accuracy rate was positively correlated with SSRN (r = 0.738, P = 0.015), SSRN ≥ 1, the accuracy rate was 0.4; SSRN ≥ 3, the accuracy rate was 0.66; SSRN ≥ 4, the accuracy rate was 0.75; SSRN ≥ 6, the accuracy rate was 1. The sensitivity of bacterial meningitis was 1. Seven kinds of bacteria were detected, among which 3/7 were gram positive, 3/7 were gram negative, and 1/7 was infected NTM (nontuberculous mycobacteria). The accuracy rate was 0.43 (3/7). The sensitivity of TBM was 0.66 (2/3), the accuracy rate was 1. The sensitivity of Crytococcus meningitis was 1, the accuracy rate was 0.5. PPV (positive predictive value) of mNGS was 0.94, NPV (negative predictive value) of mNGS was 0.89, specificity was 0.97 and sensitivity was 0.8. The AUG for CSF mNGS diagnosis of CNSI was 0.89 (95% CI = 0.78-0.99) Headache, meningeal irritation sign and image of meninges abnormal were correlated with the sensitivity of mNGS (r = 0.451, 0.313, 0.446; p = 0.001, 0.021, 0.001); CSF Glucose and CSF Chloride were negatively correlated with sensitivity of mNGS (r = -0.395, -0.462; p = 0.003, ＜ 0.001). mNGS is a detection means with high sensitivity, wide coverage and strong timeliness, which can help clinicians to identify the pathogen diagnosis quickly, conduct targeted anti-infection treatment early and reduce antibiotic abuse. The pathogen which causing low CSF Glucose, low CSF Chloride or meninges infections was more likely to be detected by mNGS. It may be related to growth and structural characteristics of the pathogen and blood-brain barrier damage.

Multiple Cranial Nerve Palsy &amp; Choroid Tuberculoma in a Patient with Disseminated TB: A Clinic Biochemical Study

Disseminated Tuberculosis is a potentially lethal disease if not diagnosed and treated early. A high index of clinical suspicion and early diagnosis and timely institution of anti TB treatmen is life saving.Patient’s sociodemographic, clinical, labarotory investigations and treatment outcome were recorded in a case record form Here we report a case of 42 year old male, known case of diabetes mellitus, presented with the complaints of fever and headache for 1 month, dropping of both eyelids, diplopia and painful red eyes for 7 days. There was no sign of meningeal irritation. Neurological examination revealed proptosis and 3rd, 4th, 6th cranial nerve palsy as evident by bilateral ptosis, diplopia, restricted ocular movement in all direction. Fundoscopy showed choroid tubercle on left eye. CSF study suggestive of tubercular meningitis. CXR showed pleural effusion and pleural fluid study suggestive of tuberculous pleural effusion. We diagnosed him as disseminated tuberculosis (tubercular meningitis, ocular TB, pleural TB). After starting anti tubercular regimen and steroid, patient’s condition got improved. Disseminated tuberculosis usually presents with constitutional symptoms rather than respiratory features. Tubercular meningitis not always present with features of meningism. Choroid tubercle is pathognomic for tubercular meningitis. Early diagnosis and treatment can reduce the mortality, morbidity and complications of disseminated TB. J Medicine 2023; Vol. 34, No. 2(1) Supplement: 224

A case series of pediatric neurobrucellosis: a rare complication to a common disease.

The first case is from a rural area and presented with a prolonged fever associated with profuse sweating, which was complicated later by headache and sudden left-sided weakness associated with urine incontinence without signs of meningeal irritation. Laboratory and radiological tests confirmed the diagnosis of NB after other cerebral infections were ruled out, and the patient received the full brucella regimen and recovered well. The second patient presented with a gradual onset of fever that did not respond to conventional treatment. Days later, his condition was complicated by a convulsion that was not preceded by an aura and was not accompanied by symptoms of weakness, increased intracranial pressure, or sphincteric disturbances. He has a history of drinking raw milk, and tests for brucella were positive, ruling out other intracranial infections and masses. He received full brucella treatment and showed good recovery. A patient from an endemic area who has a prolonged fever and neurological symptoms should be considered positive for NB until it is ruled out.

Akut sinüzit ve başarısız antimikrobiyal tedavi sonrası gelişen toplum kökenli pnömokokal menenjit olgusu

Rapid diagnosis and treatment are critical for patient recovery in bacterial meningitis. Delayed initiation of antibiotic therapy is associated with increased in-hospital mortality and an unfavorable outcome at discharge. We report a 31-year-old female patient presenting with a history of acute sinusitis and cefuroxime medication for 7 days. On the 7th day, the patient was admitted to the emergency department with nausea, vomiting, confusion, headache, tendency to sleep. On physical examination, the patient presented with unresponsiveness and weak neck stiffness as a sign of meningeal irritation. Cerebrospinal fluid analysis revealed 13,446 leukocytes per mm3 with 95% neutrophils and 5% lymphocytes. CSF biochemical analysis indicated low CSF glucose (&amp;lt;1 mg/dL glucose), elevated CSF protein (476 mg/dL total protein) and &amp;gt;2000,00 mg/L albumin. CSF RT-qPCR was positive for Streptococcus pneumoniae. The patient was immediately administered ceftriaxone and vancomycin, and recovered without any neurological sequelae. This study highlights the importance of patient follow-up, accurate prognosis and rapid laboratory testing in patients with failed sinusitis treatment.

Bacterial Meningitis, Streptococcus Suis Isolate: A Case Series (P1-10.007)

<h3>Objective:</h3> NA <h3>Background:</h3> Streptococcus Suis is an avoidable infection in humans with exposure/contact of contaminated pigs/pig products causing meningitis and septicemia; loss of hearing is a more frequent complication (Gill, C.O. et al). We present here 2 cases of bacterial meningitis from streptococcus suis infection. A 57-year old male with fever, biparieto-occipital headache, non-rotatory dizziness, vomiting, left ear hearing loss, and decreasing sensorium. He recently ate at a local Samgyupsal place. He was febrile, drowsy, disoriented, had left ear sensorineural hearing loss, and signs of meningeal irritation. On brain MRI, splenium restricted diffusion with bilateral frontal flair hyperintensity without enhancement seen. Blood culture showed streptococcus suis. Cerebrospinal fluid (CSF) was turbid, leukocyte 60 cells/uL, elevated protein and glucose, other CSF studies were unremarkable. He was started on Penicilin G and steroid therapy. A 51-year old female with fever, bi-parietal headache, non-rotatory dizziness, vomiting, hearing loss, and signs of meningeal irritation. She recently ate at a local Samgyupsal place. She was febrile, awake, had right ear sensorineural hearing loss, and signs of meningeal irritation. On brain MRI, there was bilateral cerebral sulci and cerebellar interfoliar spaces diffuse flair hyperintensities with ventral brainstem and leptomeningeal enhancement. CSF was clear with leukocyte 16 cells/uL, elevated protein, and low glucose. Blood and CSF culture showed streptococcus suis; other CSF studies were unremarkable. The patient was started on Penicilin G and steroid therapy. <h3>Design/Methods:</h3> NA <h3>Results:</h3> NA <h3>Conclusions:</h3> Streptococcus suis in humans is acquired from occupational or household exposure to contaminated pigs/pig meat (Huang, Y.T. et al). It is recognized that suboptimal pork preparation/consumption may increase the risk of acquiring this disease, as our cases above. The treatment principles in bacterial meningitis are mostly the same regardless of the isolate. The treatment with steroids is still controversial however there are studies that show improvement in the patient’s hearing and dizziness. <b>Disclosure:</b> Ms. Abbot has nothing to disclose. Dr. Ng has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Unilab. Dr. Ng has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Menarini. Dr. Ng has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Torrent Pharma. Samantha Anne Gutierrez has nothing to disclose.

Periodic Fever in Children: A Report of Three Unusual Cases

Introduction: Periodic fever syndrome (PFS) is a rare monogenic autoinflammatory disease group. The innate immune system abnormalities have a characteristic onset and spontaneous inflammation without any infectious or autoimmune trigger. It differs from autoimmune disorders (e.g., systemic lupus erythematosus (SLE)) occurring due to a defect in the adaptive immune system with auto-antibodies. Case Presentation: The clinical features of three patients presented with a periodic pattern of fever and a different constellation of symptoms were investigated. The final diagnosis of Periodic fever syndrome was reached based on standard diagnostic criteria and genetic testing. All three cases were observed to present with recurrent fever episodes at an interval of 6 - 12 weeks, 3 - 4 weeks, and one month, respectively. The first patient, presenting with a diffuse erythematous plaque-like lesion along the calf with severe calf pain and tenderness with signs of meningeal irritation, was diagnosed with a tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS) like phenotype. The genetic panel was negative in this case. The second patient presenting with recurrent pharyngitis, cervical adenitis, and tonsillitis unresponsive to antibiotics was diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. The last patient presenting with recurrent monoarthritis, hepatosplenomegaly, and a positive Mediterranean fever (MEFV) gene mutation was diagnosed with familial Mediterranean fever (FMF). All three patients had normal growth and development. Conclusions: Although periodic fever syndrome was an uncommon entity, it was recommended that this syndrome should be considered when a patient presented with recurring fever episodes with a characteristic constellation of symptoms.

Subaraknoid Kanamaya Bağlı Parkinson Hastalığının Karanlık Bir Nedeni Olarak Subtalamik Çekirdek Dejenerasyonu: Deneysel Bir Ön Çalışma

Background: Although the subtahalamic nucleus degeneration has been accused of Parkinson’s disease, the obscure roles of subtalamic nucleus degeneration induced by subarachnoid hemorrhages has not been adequately studied. The aim of the study is to examine the histopathological changes in the subthalamic nucleus after subarachnoid hemorrhage.&#x0D; Materials and Methods: Twenty-one wild male healthy rabbits were included in this study. The test subjects were divided as: control (GI, n=5); SHAM 1.2 cc of saline injected (GII, n=6) and 1.2 cc of autologous blood injection into cisterna magna (GIII, n=10). They followed up for three weeks and sacrificed under general anesthesia. Vasospasm index (VSI) was estimated by the circle surface estimation method, degenerated neuron densities of the subthalamic nucleus were estimated by Stereological methods and analyzed by Mann Witney U test.&#x0D; Results: Two rabbits dead in the study group were represented by meningeal irritation signs and unconsciousness. Prolonged QT intervals, ST depressions, and low voltage QRSs were noticed in GIII animals. Numerical documents of heart-respiratory rates (n/min), VSI values, and degenerated neuron densities of the subthalamic nucleus (n/mm3) as follows: 1.05±0.03/ 219±324/21±4/8±3 in GI; 1.75±0.23/209±14/15±4/16±4 in GII; and 2.03±0.14/175±19/19±5/123±21 GIII. P values between the VSI values and degenerated neuron densities of the subthalamic nucleus were nearly eqund: p

A West Nile Virus infection expressed as unilateral limb paralysis and complicated by Parsonage–Turner syndrome: a case report

BackgroundWest Nile Virus is a single-stranded Ribonucleic Acid arbovirus of the Flaviviridae family that is transmitted to humans by Culex species mosquitoes. West Nile Virus infection is asymptomatic in the majority of affected people. Of those who develop symptoms, the usual manifestation is a febrile syndrome, while only 1% develop neurological symptoms due to a neuroinvasive form of infection, including encephalitis, meningitis, asymmetrical flaccid paralysis, or a combination of all these features. Parsonage–Turner syndrome is a rare disorder characterized by sudden painful symptoms and subsequent paralysis, involving a shoulder or one of the upper limbs due to post-infective brachial plexopathy. The etiology is unknown, although it can be considered a multifactorial process: a predisposing factor, such as viral infection or strenuous upper-extremity exercise, can trigger an immune-mediated process localized in the brachial plexus.Clinical presentationIn late summer, a 79-year-old male Italian patient was admitted to the emergency department for acute right upper limb weakness and high fever, without any mental status impairment, pain, sensory alterations, or signs of meningeal irritation. Laboratory tests confirmed acute West Nile Virus infection, expressed as a unilateral upper limb flaccid paralysis. After a few days, the patient reported an acute pain in the right upper limb scarcely responsive to nonsteroidal anti-inflammatory drug therapy and a subsequent wider distribution of flaccid paralysis. After multiple examinations, Parsonage–Turner syndrome could be suspected. Patient was treated with steroids and reported an improvement of clinical condition after 2 months, with complete pain remission but partial strength recovery in the affected limb.ConclusionsWest Nile Virus disease has a broad spectrum of neurological manifestations, among which the most common are signs of meningeal irritation or cognitive impairment. We report an unusual presentation of neuroinvasive West Nile Virus infection with arm weakness as expression of unilateral viral neuritis, followed by a post-infective brachial plexopathy consistent with Parsonage–Turner syndrome diagnosis. We diagnosed Parsonage–Turner syndrome after excluding the most common causes of atraumatic acute upper limb pain, through a challenging differential diagnosis in a patient with several comorbidities.

Delayed Appearance of Brain Magnetic Resonance Imaging Abnormalities in a Patient with Glial Fibrillary Acidic Protein Astrocytopathy.

Recent studies have reported that autoantibodies against glial fibrillary acidic protein (GFAP), a major cytoskeletal protein expressed in astrocytes, can lead to GFAP astrocytopathy, an autoimmune central nervous system inflammatory disease. We herein report the unique case of a 59-year-old Japanese woman with GFAP astrocytopathy who presented with characteristic symptoms, including signs of meningeal irritation, cerebellar ataxia, and bladder/rectal dysfunction, in the absence of specific findings on initial brain magnetic resonance imaging (MRI). The patient exhibited new abnormal changes mainly in the brainstem on follow-up MRI, illustrating the need to recognize that MRI abnormalities may appear later in GFAP astrocytopathy.

A case of neurosyphilis presenting as sudden onset of limbic encephalitis

A 52-year-old male was carried to hospital by ambulance, because of an abrupt abnormal behavior and impaired consciousness. Soon after the arrival, the patient started a generalized seizure. Although the seizure was stopped by Midazolam, amnesia were observed. With meningeal irritation signs, in addition to the clinical course, the patient was thought to develop limbic encephalitis. The cause of the encephalitis was diagnosed as neurosyphilis because of the positive serum and CSF syphilis reactions, and the patient was treated with penicillin G from the first admission day on. Steroid pulse therapy was also conducted, followed by acyclovir since herpes encephalitis could not be ruled out; the brain MRI showed left-side dominant T2/FLAIR high intensity lesions in the bilateral temporal lobes and left hippocampus. With the treatment progression, the amnestic syndrome improved and the patient returned to work. Although neurosyphilis is a rare cause of acute onset limbic encephalitis, it is important to keep the possibility of this disease in mind in making a treatment plan.

Anti-NMDA Receptor Encephalitis: A Rare but Important Auto-Immune Cause of Encephalitis

Abstract: A previously healthy 30-year old lady presented with 1-day history of high grade fever and drowsiness. Five days prior to presentation, she developed insomnia and visual hallucinations of seeing unknown faces. Three days prior to presentation, she suffered from 5 episodes of generalized tonic colonic fits. On admission, she had a temperature of 102oF and GCS of 10/15 with no signs of meningeal irritation, no focal neurological deficit, normal deep tendon reflexes and down-going plantar reflex bilaterally. Her brain MRI scan showed mild hyper-intense signals in right cerebellum. Her CSF was tested for Anti-NMDA receptor antibodies which were positive. She was diagnosed as having Anti-NMDA Receptor Encephalitis and started on immediate Plasma Exchange with drastic improvement.