scholarly journals Cerebrospinal Fluid IL-17A Could Predict Acute Disease Severity in Non-NMDA-Receptor Autoimmune Encephalitis

2021 ◽  
Vol 12 ◽  
Author(s):  
Michael Levraut ◽  
Véronique Bourg ◽  
Nicolas Capet ◽  
Adrien Delourme ◽  
Jérôme Honnorat ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Disease Severity ◽  
Disease Onset ◽  
Autoimmune Encephalitis ◽  
Demyelinating Diseases ◽  
Acute Disease ◽  
Aspartate Receptor ◽  
Initial Severity ◽  
Csf Concentration

IntroductionMost of our knowledge into autoimmune encephalitis (AE) comes from N-Methyl-D-Aspartate Receptor (NMDAR) encephalitis. The concentrations of cytokines in cerebrospinal fluid (CSF) including IL-17A have been found to be increased and associated with poor outcome. However, data on the cytokine concentration in CSF and its correlation with outcome is lacking for other types of AE.ObjectiveTo report the concentrations of CSF sIL-2R, IL-6, IL-8, IL-10 and IL-17A and to correlate it with acute disease severity and the 1-year outcome in non-NMDAR AE.MethodsWe measured the CSF concentration of each cytokine in 20 AE patients, and compared IL-6 and IL-17A concentrations with 13 patients with CNS demyelinating diseases and 20 non-inflammatory controls. Patients were > 18yr and had at least 1-year clinical follow-up. Intracellular and NMDAR antibody (Ab) -mediated encephalitis were excluded. A mRS ≤ 2 was retained as a 1-year good outcome.ResultsThe IL-17A concentration in CSF was higher in AE patients than in both control groups (p<0.01). No difference was observed in CSF concentration of IL-6 between groups. At disease onset, a high CSF IL-17A concentration correlated with a high modified Rankin Scale (p<0.05), a high Clinical Assessment Scale for Autoimmune Encephalitis score (p<0.001) and ICU admission (p<0.01). There was no correlation between the concentration of all CSF cytokines and the 1-year clinical outcome.ConclusionOur results show that CSF IL-17A could be interesting to assess initial severity in non-NMDAR AE. Thus, CSF IL-17A could be an interesting therapeutic target and be useful to assess early selective immunosuppressive therapy.

Rapidly Progressive Memory Loss, Mood Change, Mutism, and Abnormal Movements

2021 ◽  
pp. 83-85
Author(s):  
Shailee S. Shah ◽  
Marie F. Grill
Keyword(s):  
Cerebrospinal Fluid ◽  
Short Term Memory ◽  
White Blood Cells ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Aspartate Receptor ◽  
Normal Glucose ◽  
Disorganized Speech ◽  
Patient’S History

A 24-year-old woman sought care for 2 weeks of disorientation and short-term memory difficulties, as well as diffuse tremor of all extremities. She returned with further decline in memory and new severe headaches. She had intermittent agitation and emotional outbursts of crying or laughing, insomnia, spells consisting of disorganized speech and episodes of intermittent right gaze deviation with facial twitching and lip smacking. She was nearly mute. Her appetite had decreased and she had not had a bowel movement in several days. She was noted to have significant tachycardia and was intermittently febrile. Within several days she became unresponsive to all external stimuli, with nonpurposeful eye movements and frequent dyskinesias observed, and ultimately required ventilator support. Testing of the cerebrospinal fluid showed 236 white blood cells/µL, mildly increased protein concentration of 50 mg/dL, and normal glucose values. Electroencephalography initially demonstrated generalized slowing and generalized periodic epileptiform discharges and was also notable for an extreme delta brush pattern. Bilateral ovarian masses were identified on pelvic ultrasonography, and subsequent computed tomography of the abdomen and pelvis showed bilateral teratomas. An autoimmune encephalitis autoantibody panel was positive for antibodies targeting the N-methyl-d-aspartate receptor in the serum and cerebrospinal fluid, by both cell-based and immunofluorescence assays. The patient was diagnosed with anti- N-methyl-d-aspartate receptor encephalitis. The patient initially received intravenous methylprednisolone, followed by intravenous immunoglobulin. Benzodiazepines and propranolol were used to manage agitation and dysautonomia. Antiepileptic drugs were initiated for seizures. She required mechanical ventilation and parenteral nutrition given her persistent profound encephalopathic state. She underwent left ovarian cystectomy and right salpingo-oophorectomy. This patient’s history highlights the progressive clinical features characteristic of anti- N-methyl-d-aspartate receptor encephalitis and the long but often complete or near-complete recovery.

scholarly journals Sclerosis multiplex a közép-magyarországi régióban: a helyi adatbázisfejlesztés tapasztalatai és jövőbeli lehetőségei

2019 ◽  
Vol 160 (4) ◽  
pp. 131-137
Author(s):  
Anna Iljicsov ◽  
Magdolna Simó ◽  
Nárcisz Tegze ◽  
Miklós Szócska ◽  
Péter Mátyus ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Economic Status ◽  
Disease Onset ◽  
Data Entry ◽  
Automatic Data ◽  
Healthcare Data ◽  
Transfer Of Information ◽  
User Friendly ◽  
Pdf Format

Abstract: Introduction: Data during routine patient care are created in multiple digital and paper-based hardcopy systems, therefore their retrieval is cumbersome in the follow-up of patients. Multiple sclerosis is the most prevalent neurological disorder in the young age, with major consequences on health and socio-economic status. Aim: We set forth to create a user-friendly, detailed local database where it is easy to access, register and analyze data. Based on our experiences during building this registry, we develop the model of a modern type of database. Method: First we established a local registry in Excel, then data were transferred to the worldwide used iMed system. Separate pages were used to register basic data, follow-up visits, relapses, accompanying diseases, results of neuroimaging, cerebrospinal fluid, evoked response and other tests, pharmacological and non-pharmacological treatments. Results: The database currently contains data of 316 patients. MRI was performed in 96%, cerebrospinal fluid examination in 45% of the patients. The rate of primary progressive disease at disease onset is 9%. Disease modifying treatments were applied in 82% of the patients. Conclusion: The traditional manual data entry and data export in PDF format is obsolete and time-consuming. The development of local disease-specific databases appropriate for clinical and research purposes requires continuous and mostly automatic data entry. In future local registries the establishment of uniform documentational language and structure, and automatic transfer of information among different digital systems are required. We present the model of such a registry, which is based on a healthcare data lake. Orv Hetil. 2019; 160(4): 131–137.

scholarly journals Cerebrospinal Fluid IL-21 Levels in Neuromyelitis Optica and Multiple Sclerosis

2012 ◽  
Vol 39 (6) ◽  
pp. 813-820 ◽  
Author(s):  
Aimin Wu ◽  
Xiaonan Zhong ◽  
Honghao Wang ◽  
Wen Xu ◽  
Chen Cheng ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Cerebrospinal Fluid ◽  
Neuromyelitis Optica ◽  
Demyelinating Diseases ◽  
Enzyme Linked Immunosorbent Assay ◽  
Human Central Nervous System ◽  
Immune Activity ◽  
Humoral Immune ◽  
Csf Concentration

Background:Neuromyelitis optica (NMO) and multiple sclerosis (MS) are inflammatory demyelinating diseases of human central nervous system (CNS) with complex pathogenesis. IL-21/IL-21R regulates activation, proliferation and survival of both T cells and B cells, which are involved in the pathogenesis of NMO and MS. High levels of serum IL-21 were observed in NMO patients. However, concentration of cerebrospinal fluid (CSF) IL-21 in MS and NMO patients still remain unknown.Object:To detect the CSF concentration of IL-21 in NMO and MS patients and to evaluate its relationship with disease activity, particularly concerned about its impact on humoral immunity.Methods:CSF IL-21 was detected by an enzyme-linked immunosorbent assay (ELISA) in NMO patients (n=21), MS patients (n=20) and controls (n=16).Results:CSF concentration of the IL-21 was noticeably elevated in NMO (p=0.012) and borderline significantly increased in MS (p=0.115). In addition, this occurrence was associated with humoral immune activity as shown by a correlation between IL-21 and complement in NMO cohort (p=0.023) and high IL-21 levels in autoantibody-positive subgroup (p=0.027).Conclusions:The concentration of CSF IL-21 was noticeably elevated and might have a positive correlation with humoral immune activity in NMO.

scholarly journals Clinical Relevance of Cerebrospinal Fluid Antibody Titers in Anti-N-Methyl-d-Aspartate Receptor Encephalitis

2021 ◽  
Vol 12 (1) ◽  
pp. 4
Author(s):  
Meng-Ting Cai ◽  
Yang Zheng ◽  
Sa Wang ◽  
Qi-Lun Lai ◽  
Gao-Li Fang ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Memory Deficits ◽  
Clinical Severity ◽  
Weak Correlation ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Significant Difference ◽  
The Relationship

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is the most common autoimmune encephalitis. To date, there has been no study on the relationship between antibody (Ab) titers and clinical phenotype. This study aims to clarify the relationship between cerebrospinal fluid Ab titers and clinical manifestations of anti-NMDAR encephalitis at onset. Seventy-six consecutive patients with a definite diagnosis were enrolled. The relationship between Ab titers and different onset symptoms including psychiatric symptoms, seizures, and memory deficits were analyzed. We further investigated the correlation between Ab titers and clinical severity as assessed by the modified Rankin scale (mRS) and the clinical assessment scale for autoimmune encephalitis (CASE), respectively. The Ab titers had a median value of 1:10 (range 1:1–1:100). There was no significant difference in titers among various clinical factors including gender and combination of tumor and other diseases (each p > 0.05). Patients presenting with psychiatric symptoms at onset had higher titers than those with seizures (p = 0.008) and memory deficits (p = 0.003). The mRS scores revealed a significant but weak correlation with Ab titers (r = 0.243, p = 0.034), while CASE scores did not correlate with the titers (p = 0.125). Our findings indicated that the Ab titers were associated with the type of onset symptoms, with a higher level of patients with psychiatric symptoms. Regarding the clinical severity, the titers showed a weak correlation with the mRS, but no correlation with the CASE.

scholarly journals Systemic Inflammation Index Values Are Associated With Worsened Disease Severity and Poor Response to Autoimmune Encephalitis Treatment

2021 ◽  
Vol 12 ◽  
Author(s):  
Yanliang Mei ◽  
Jing Yang ◽  
Yanpeng Yuan ◽  
Yutao Liu ◽  
Xiaojing Liu ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Treatment Response ◽  
Disease Severity ◽  
Predictive Value ◽  
Blood Lymphocyte ◽  
Treatment Initiation ◽  
Fluid Pressure ◽  
Autoimmune Encephalitis ◽  
Poor Response ◽  
Peripheral Biomarker

Both specific and innate immune responses play important roles in autoimmune encephalitis (AE). We aimed to explore the predictive value of the systemic inflammation index (SII) at admission as a peripheral biomarker of treatment response of AE. A total of 146 patients diagnosed with AE in the First Affiliated Hospital of Zhengzhou University from January 1, 2018 to September 22, 2020 were retrospectively and consecutively analyzed as per the inclusion criteria and divided into two groups according to their response to immunotherapy after 30 days. The predictive value of the SII as a peripheral biomarker for AE treatment response was calculated using the receiver operating characteristic curve analysis, which showed that the best SII cut-off value for predicting poor response to AE treatment was 863.3; the area under the curve was 0.75, with 83.0% sensitivity and 72.0% specificity. The risk factors for poor response to AE treatment were analyzed; univariable analysis showed that the rate of decreased level of consciousness, rate of cognitive or mental behavior abnormality, cerebrospinal fluid pressure, blood neutrophils, platelets, time until treatment initiation, neutrophil to lymphocyte ratio, platelet to lymphocyte ratio, and SII were significantly higher in patients with poor response to AE immunotherapy after 30 days than in patients with good response. Meanwhile, the blood lymphocyte counts and Glasgow Coma Scale (GCS) scores in patients with poor response were significantly lower than those in patients with good response (all p < 0.05), and multivariable binary logistic regression with backward stepwise method showed that decreased levels of consciousness, time until treatment initiation and SII were associated with poor response to immunotherapy. Moreover, the SII ≤ 863.3 group had lower rates of decreased consciousness levels, admission to the intensive care unit, and mechanical ventilation; lower cerebrospinal fluid pressure, blood neutrophil count, and platelet count; and higher blood lymphocyte count and GCS scores. The SII was associated with worsened disease severity and poor response to treatment after 30 days of the initially diagnosed AE, and patients with an SII > 863.3 were more likely to have poor response to immunotherapy.

scholarly journals Pediatric autoimmune encephalitis

2020 ◽  
Vol 7 (3) ◽  
pp. e682 ◽  
Author(s):  
Marienke A.A.M. de Bruijn ◽  
Arlette L. Bruijstens ◽  
Anna E.M. Bastiaansen ◽  
Agnes van Sonderen ◽  
Marco W.J. Schreurs ◽  
...  
Keyword(s):  
Autoimmune Encephalitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Incidence Rates ◽  
Study Cohort ◽  
Current Guideline ◽  
Clinical Criteria ◽  
Aspartate Receptor ◽  
Hashimoto Encephalopathy ◽  
Neurologic Disorders

ObjectiveThe aims of this study were (1) to describe the incidence of autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) in children, (2) to validate the currently used clinical criteria to diagnose AIE, and (3) to describe pitfalls in the diagnosis of pediatric autoimmune (AI) and inflammatory neurologic disorders.MethodsThis study cohort consists of 3 patient categories: (1) children with antibody-mediated AIE (n = 21), (2) children with ADEM (n = 32), and (3) children with suspicion of an AI etiology of their neurologic symptoms (n = 60). Baseline and follow-up clinical data were used to validate the current guideline to diagnose AIE. In addition, patient files and final diagnoses were reviewed.ResultsOne-hundred three of the 113 included patients fulfilled the criteria of possible AIE. Twenty-one children had antibody-mediated AIE, of whom 19 had anti-N-methyl-D-aspartate receptor (NMDAR), 1 had anti–α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, and 1 had anti–leucine-rich glioma-inactivated protein 1 encephalitis. Finally, 34 children had ADEM, and 2 children had Hashimoto encephalopathy. Mean incidence rates were 1.54 children/million (95% CI 0.95–2.35) for antibody-mediated AIE and 2.49 children/million (95% CI 1.73–3.48) for ADEM. Of the other 48 children, treating physicians' diagnoses were reviewed. In 22% (n = 6) of children initially diagnosed as having an AI/inflammatory etiology (n = 27), no support for AI/inflammation was found.ConclusionBesides anti-NMDAR encephalitis and ADEM, other AIEs are rare in children. The current guideline to diagnose AIE is also useful in children. However, in children with nonspecific symptoms, it is important to review data critically, to perform complete workup, and to consult specialized neuroinflammatory centers.

scholarly journals Case Report: Daratumumab in a Patient With Severe Refractory Anti-NMDA Receptor Encephalitis

2020 ◽  
Vol 11 ◽  
Author(s):  
Dominica Ratuszny ◽  
Thomas Skripuletz ◽  
Florian Wegner ◽  
Matthias Groß ◽  
Christine Falk ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Neuropsychiatric Symptoms ◽  
Clinical Status ◽  
Disease Onset ◽  
Autoimmune Encephalitis ◽  
Intravenous Immunoglobulins ◽  
Nmda Receptor Encephalitis ◽  
Life Threatening ◽  
Therapy Strategies

Anti-NMDA receptor encephalitis is the most common type of antibody mediated autoimmune encephalitis (AIE). Patients often develop neuropsychiatric symptoms and seizures, women are affected about four times more than men, and in about 50% the disease is associated with a neoplasia, especially teratomas of the ovary. We describe the case of a 20-year-old woman suffering from a severe therapy refractory course of anti-NMDA receptor encephalitis. Treatment included glucocorticoids, plasma exchange, intravenous immunoglobulins, rituximab, and bortezomib without clinical improvement. Due to a therapy refractive course 28 weeks after disease onset, the patient received 10 cycles of daratumumab. Therapy escalation was performed with the anti-CD38 monoclonal antibody daratumumab as off label treatment, based on the therapy of refractory myeloma and led to an improvement of her clinical status. She spent about 200 days on the intensive care unit, followed by several weeks on the intermediate care unit with close follow ups every 4–6 weeks afterward. During follow-up, the patient was able to resume everyday and self-care activities, reflected by the modified Rankin scale (mRS) and Barthel index. Because this disease is potentially life threatening and can lead to irreversible brain atrophy, development of further therapy strategies are of great importance. Our case describes a successful treatment for therapy refractory anti-NMDA receptor encephalitis using the anti-CD38 antibody daratumumab.

Extremely Elevated Cerebrospinal Fluid Protein and Glucose Level in a Neonate with Hypernatremic Dehydration

2020 ◽  
Author(s):  
Arti Maria ◽  
Tapas Bandyopadhyay
Keyword(s):  
Cerebrospinal Fluid ◽  
Neurodevelopmental Outcome ◽  
Neurological Sequelae ◽  
Glucose Levels ◽  
Csf Analysis ◽  
First Case ◽  
Time Of Admission ◽  
Cerebrospinal Fluid Protein ◽  
Hypernatremic Dehydration

AbstractWe describe the case of a term newborn who presented with hypernatremic dehydration on day 19 of life. The baby was otherwise hemodynamically stable with no evidence of focal or asymmetric neurological signs. The laboratory tests at the time of admission were negative except for hypernatremia and the extremely elevated levels of cerebrospinal fluid (CSF) protein (717 mg/dL) and glucose levels (97 mg/dL). The hypernatremic dehydration was corrected as per the unit protocol over 48 hours. Repeat CSF analysis done after 5 days showed normalization of the protein and glucose levels. Serial follow-up and neuroimaging showed no evidence of neurological sequelae. Unique feature of our case is this is the first case reporting such an extreme elevation of CSF protein and glucose levels that have had no bearing on neurodevelopmental outcome at 1 month and 3 months of follow-up.

scholarly journals Increased cerebrospinal fluid adenosine 5'-triphosphate in patients with amyotrophic lateral sclerosis

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Takamasa Nukui ◽  
Atsushi Matsui ◽  
Hideki Niimi ◽  
Tomoyuki Sugimoto ◽  
Tomohiro Hayashi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cerebrospinal Fluid ◽  
Serum Creatinine ◽  
Disease Severity ◽  
Assay System ◽  
Atp Levels ◽  
Sum Score ◽  
Highly Sensitive ◽  
Severe Group ◽  
Lateral Sclerosis

Abstract Background Extracellular adenosine 5'-triphosphate (ATP) has been suggested to cause neuroinflammation and motor neuron degeneration by activating microglia and astrocytes in amyotrophic lateral sclerosis (ALS). Since we have developed a highly sensitive ATP assay system, we examined cerebrospinal fluid (CSF) ATP levels in patients with ALS whether it can be a useful biomarker in ALS. Methods Forty-eight CSF samples from 44 patients with ALS were assayed for ATP with a newly established, highly sensitive assay system using luciferase luminous reaction. CSF samples from patients with idiopathic normal pressure hydrocephalus (iNPH) were assayed as a control. Patients were divided into two groups depending on their disease severity, as evaluated using the Medical Research Council (MRC) sum score. Correlations between the CSF ATP levels and other factors, including clinical data and serum creatinine levels, were evaluated. Results CSF ATP levels were significantly higher in patients with ALS than in the iNPH (716 ± 411 vs. 3635 ± 5465 pmol/L, p < 0.01). CSF ATP levels were significantly higher in the more severe group than in the iNPH group (6860 ± 8312 vs. 716 ± 411 pmol/L, p < 0.05) and mild group (6860 ± 8312 vs. 2676 ± 3959 pmol/L, p < 0.05) respectively. ALS functional rating scale-revised (ALSFRS-R) (37.9 ± 5.7 vs. 42.4 ± 2.8, p < 0.01) and serum creatinine levels (0.51 ± 0.13 vs. 0.68 ± 0.23 mg/dL, p < 0.05) were significantly lower in the severe group than in the mild group respectively. A negative correlation of CSF ATP levels with MRC sum score was demonstrated in the correlation analysis adjusted for age and sex (r = -0.3, p = 0.08). Conclusions Extracellular ATP is particularly increased in the CSF of patients with advanced ALS. CSF ATP levels may be a useful biomarker for evaluating disease severity in patients with ALS.

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