scholarly journals A critical reflection on our first patient presenting with Anti-Nmethyl- D-aspartate receptor encephalitis

2019 ◽  
Vol 11 (3) ◽  
Author(s):  
Susanne Buechner ◽  
Igor Florio ◽  
Gabriele J. Sixt ◽  
Francesco Teatini
Keyword(s):  
Treatment Guidelines ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Intravenous Immunoglobulins ◽  
High Dose ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
One Year

One of the best characterized autoimmune encephalitis is the Anti-Nmethyl-D-aspartate receptor (NMDAR) encephalitis, which may occur in the presence of cancer. First- and second-line immunotherapy and oncological investigations are suggested. We present here a case of an 18-year-old female who was our first patient suffering from Anti-NMDAR encephalitis more than 9 years ago. She was satisfactorily treated with intravenous immunoglobulins and high dose steroid therapy. After more than one year the patient had a relapse. First-line immunotherapy was repeated; however, a complete recovery was achieved only after plasmapheresis. Afterwards, she continued maintenance immunotherapy with steroids for two years and with Azathioprine for about five years associated to regular oncological assessment. In the last years our therapeutical approach of Anti-NMDARencephalitis has significantly changed. Nevertheless, established treatment guidelines are still missing and the role of long-term maintenance immunotherapy is largely unexplored. In addition, oncological revaluation might be indicated in selected patients.

Autoimmune Encephalitis in Children: A Case Series at a Tertiary Care Center

2020 ◽  
Vol 35 (9) ◽  
pp. 591-599 ◽  
Author(s):  
Alice Rutatangwa ◽  
Nikita Mittal ◽  
Carla Francisco ◽  
Kendall Nash ◽  
Emmanuelle Waubant
Keyword(s):  
San Francisco ◽  
Care Center ◽  
Tertiary Care ◽  
Case Series ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Response To Treatment ◽  
High Dose ◽  
Presenting Symptoms ◽  
Nmdar Encephalitis

Autoimmune encephalitis is the third most common cause of encephalitis in children. We provide a detailed account of presenting symptoms, diagnosis, and response to treatment in pediatric autoimmune encephalitis patients evaluated at University of California San Francisco within a 2.5-year period. Eleven were identified: anti– N-methyl-d-aspartate receptor (NMDAR) encephalitis (n = 4), antibody-negative autoimmune encephalitis (n = 4), steroid-responsive encephalopathy associated with thyroiditis (SREAT) (n = 2), and glial fibrillary acidic protein (GFAP)–associated encephalitis (n = 1). Most common presenting symptoms included seizures and behavior changes (54%). More than 90% of patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin, and/or plasma exchange). A total of 64% received second-line treatment with rituximab, cyclophosphamide, or mycophenolate mofetil. One patient with NMDAR encephalitis died despite escalating immunotherapy. None of the patients showed complete recovery after median follow-up of 9 months (range 0.5-66). Children with autoimmune encephalitis have a diverse clinical presentation and may lack an identifiable autoantibody. Majority of patients show a good response to immunotherapy; however, recovery can be delayed.

scholarly journals Rituximab for Autoimmune Encephalitis with Epilepsy

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Mohankumar Kurukumbi ◽  
Rahul H. Dave ◽  
Jose Castillo ◽  
Tulsi Shah ◽  
Joanne Lau
Keyword(s):  
Intractable Epilepsy ◽  
Autoimmune Encephalitis ◽  
Aminobutyric Acid ◽  
High Dose ◽  
Gamma Aminobutyric Acid ◽  
Acid Receptor ◽  
Voltage Gated ◽  
Aspartate Receptor ◽  
A Cell

Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and N-methyl-D-aspartate receptor (NMDAR). Diagnosis requires a syndrome consistent with an antibody identified in serum or cerebrospinal fluid (CSF) using methods that minimize risk of false-positives. Although there is no officially approved therapy for these disorders, typical approaches involve chronic high-dose steroids, intravenous immunoglobulin (IVIG), or plasma exchange. Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.

scholarly journals Anti-NMDAR encephalitis for psychiatrists: the essentials

2021 ◽  
pp. 1-7
Author(s):  
Matthew Beattie ◽  
John Goodfellow ◽  
Maria Oto ◽  
Rajeev Krishnadas
Keyword(s):  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Female Adolescents ◽  
Resonance Imaging ◽  
Long Term Outcomes ◽  
Optimal Care ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Uncommon Condition

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis predominantly presents with psychiatric symptoms. Psychiatrists need to be alert to this diagnostic possibility, especially in female adolescents and young adults, as exemplified by the real (de-identified) case outlined below. Earlier diagnosis and immunotherapy improves long-term outcomes. Collaboration with neurology colleagues is essential for optimal care. ‘Red flags’ for autoimmune encephalitis and ‘diagnostic clues’ act as helpful aide memoires for this uncommon condition. The gold standard for testing is the detection of NMDAR antibodies in cerebrospinal fluid, but serum can be tested as a more accessible (but less reliable) preliminary step. The results of routine blood tests, magnetic resonance imaging of the head and electroencephalograms can be normal or show non-specific changes. Diagnostic criteria exist to define probable and definite cases. Immunotherapy for anti-NMDAR encephalitis is effective for many patients, but recovery is prolonged and relapses can occur.

scholarly journals Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Decrease in Blood Flow in Cerebellum

2021 ◽  
pp. 17-23
Author(s):  
Koji Obara ◽  
Tomoko Ono ◽  
Itaru Toyoshima
Keyword(s):  
Working Memory ◽  
Blood Flow ◽  
Ovarian Teratoma ◽  
Emission Computed Tomography ◽  
High Dose ◽  
Promising Alternative ◽  
Flow Monitoring ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Consciousness Disturbance

In anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, progressive cerebellar atrophy potentially leads to severe sequelae. We encountered a patient with anti-NMDAR antibody encephalitis who showed a decrease of blood flow in the cerebellum. A 15-year-old girl presented with consciousness disturbance. Influenza encephalopathy was suspected, and she was treated with glucocorticoid pulse therapy, high-dose intravenous immunoglobulins, and plasma exchange sequentially. She subsequently underwent left oophorectomy due to the presence of anti-NMDAR antibodies and a left ovarian teratoma. In spite of the surgery, her neuropsychiatric symptoms persisted, and she recovered slowly after the introduction of oral methotrexate (MTX). Sequential cerebral blood flow monitoring with single-photon emission computed tomography showed marked cerebellar hypoperfusion. Although mild impairments including working memory and verbal fluency persisted, she eventually returned to high school 3 years after onset. Profound cerebellar hypoperfusion including lobules VI and VII may be the reason for her working memory impairment and speaking problems. Oral MTX may be a promising alternative treatment for some refractory cases of anti-NMDAR encephalitis.

scholarly journals Role of methyleprednisolone succinate in the management of acute spinal cord injury

1970 ◽  
Vol 9 (3) ◽  
pp. 168-172
Author(s):  
NK Karn ◽  
BP Shrestha ◽  
GP Khanal ◽  
R Rijal ◽  
P Chaudhary ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Neurological Status ◽  
Acute Spinal Cord Injury ◽  
Cord Injury ◽  
And Gender ◽  
One Year

Objective: To see the role of methyleprednisolone succinate in the management of acute spinal cord injury. Methods: A randomized control trial was done including the patients with acute spinal cord injury. They were divided into age and gender matched two groups. Patients with presence of active infection, associated open fracture, those on long term steroid and those who did not give consent to participate in the trial were excluded. One group received methyleprednisolone succinate within 8 hours of injury and another group did not receive the drug. Both the groups were managed nonoperatively. The neurological status of the patients was assessed at presentation, once spinal shock was over, at 6th week and 6th month and after one year according to ASIA scoring. Frankel grading was also assessed in every follow up. Conclusion: Methylprednisolone succinct prevents secondary cord injury to a great extent and hence its administration within 8 hours of injury results in a better functional (motor and sensory) outcome. Keywords: acute spinal cord injury; methyleprednisolone succinate DOI: http://dx.doi.org/10.3126/hren.v9i3.5585   HR 2011; 9(3): 168-172

scholarly journals Laporan Kasus: Manifestasi Oral Penderita Hipertensi berupa Ginggival Enlargement

2020 ◽  
Vol 17 (2) ◽  
pp. 54
Author(s):  
Anindita L. ◽  
Aris Aji K. ◽  
Arcadia Sulistijo J.
Keyword(s):  
Case Reports ◽  
Gingival Tissue ◽  
High Dose ◽  
Gingival Inflammation ◽  
Gingival Enlargement ◽  
History Of ◽  
Root Planning ◽  
Pro Inflammatory Cytokines

Hypertension presents an increase in blood pressure following the oral manifestations, such as gingival enlargement. A 42-year-old woman came to the General Sudirman University Dental and Oral Hospital complaining of enlarged front gums seven years ago. The patient had a history of hypertension and regularly consumed drugs, amlodipine 5 mg. Extraoral examination revealed no lymphadenopathy and no swelling of the head and neck area. Intraoral examination revealed a gingival enlargement involving the papilla to the gingival margin present on the entire upper and lower labial gingival surface. The patient's diagnosis was gingival enlargement caused by gingival enlargement due to the use of amlodipine. Gingival enlargement has been noted with long-term or high-dose amlodipine use. The mechanism of amlodipine in causing gingival enlargement is through the role of fibroblasts with abnormal susceptibility to the drug, resulting in increased levels of protein synthesis, especially collagen. The role of pro-inflammatory cytokines occurs through an increase in interleukin-1β (IL-1β) and IL-6 in the inflamed gingival tissue due to the gingival fibrogenic response to drugs. Therapies were DHE and scaling and root planning as phase I in periodontal treatment. Plaque elimination is vital to reduce gingival inflammation that may occur. Substitution of the drug amlodipine may be needed if there is no improvement. Based on case reports, hypertension patients who took amlodipine could have gingival enlargement. The therapy given was plaque elimination in the form of DHE and Scaling and regular check-ups with the dentist.

Anti-N-methyl-D-aspartate receptor encephalitis and ovarian teratomas: is there any association?

2020 ◽  
Vol 19 (5) ◽  
pp. 124-131
Author(s):  
N.M. Podzolkova ◽  
◽  
V.V. Korennaya ◽  
O.S. Levin ◽  
E.E. Vasenina ◽  
...  
Keyword(s):  
Pathological Condition ◽  
Rare Complication ◽  
Autoimmune Encephalitis ◽  
Neurological Deficits ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Life Threatening ◽  
Causal Treatment ◽  
Ovarian Teratomas

This article aims to explore the problem of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and also describes our own experience in treating anti-NMDAR encephalitis in patients with ovarian teratomas. This pathological condition is a rare complication of cancer. Practicing gynecologists are often unfamiliar with it, although its consequences can be life-threatening. Early diagnosis and causal treatment, particularly teratoma removal, are critical for the outcome and can prevent death or the development of neurological deficits in patients. Key words: anti-NMDAR, autoimmune encephalitis, ovarian cysts, complications, ovarian teratoma, encephalitis, encephalitis in women

scholarly journals Effects of Fire and Large Herbivores on Canopy Nitrogen in a Tallgrass Prairie

2019 ◽  
Vol 11 (11) ◽  
pp. 1364 ◽  
Author(s):  
Bohua Ling ◽  
Edward J. Raynor ◽  
Douglas G. Goodin ◽  
Anthony Joern
Keyword(s):  
Spatial Variability ◽  
Nitrogen Content ◽  
Tallgrass Prairie ◽  
High Nitrogen Content ◽  
High Nitrogen ◽  
Grazing History ◽  
One Year ◽  
Canopy Nitrogen

This study analyzed the spatial heterogeneity of grassland canopy nitrogen in a tallgrass prairie with different treatments of fire and ungulate grazing (long-term bison grazing vs. recent cattle grazing). Variogram analysis was applied to continuous remotely sensed canopy nitrogen images to examine the spatial variability in grassland canopies. Heterogeneity metrics (e.g., the interspersion/juxtaposition index) were calculated from the categorical canopy nitrogen maps and compared among fire and grazing treatments. Results showed that watersheds burned within one year had higher canopy nitrogen content and lower interspersions of high-nitrogen content patches than watersheds with longer fire intervals, suggesting an immediate and transient fire effect on grassland vegetation. In watersheds burned within one year, high-intensity grazing reduced vegetation density, but promoted grassland heterogeneity, as indicated by lower canopy nitrogen concentrations and greater interspersions of high-nitrogen content patches at the grazed sites than at the ungrazed sites. Variogram analyses across watersheds with different grazing histories showed that long-term bison grazing created greater spatial variability of canopy nitrogen than recent grazing by cattle. This comparison between bison and cattle is novel, as few field experiments have evaluated the role of grazing history in driving grassland heterogeneity. Our analyses extend previous research of effects from pyric herbivory on grassland heterogeneity by highlighting the role of grazing history in modulating the spatial and temporal distribution of aboveground nitrogen content in tallgrass prairie vegetation using a remote sensing approach. The comparison of canopy nitrogen properties and the variogram analysis of canopy nitrogen distribution provided by our study are useful for further mapping grassland canopy features and modeling grassland dynamics involving interplays among fire, large grazers, and vegetation communities.

scholarly journals Neuroprotective role of lactate in rat neonatal hypoxia-ischemia

2020 ◽  
pp. 0271678X2090835 ◽  
Author(s):  
Hélène Roumes ◽  
Ursule Dumont ◽  
Stéphane Sanchez ◽  
Leslie Mazuel ◽  
Jordy Blanc ◽  
...  
Keyword(s):  
Neuroprotective Effect ◽  
Waste Product ◽  
Brain Lesion ◽  
Complete Recovery ◽  
Long Term Memory ◽  
Lesion Volume ◽  
Apparent Diffusion Coefficients ◽  
Hypoxia Ischemia

Hypoxic-ischemic (HI) encephalopathy remains a major cause of perinatal mortality and chronic disability in newborns worldwide (1–6 for 1000 births). The only current clinical treatment is hypothermia, which is efficient for less than 60% of babies. Mainly considered as a waste product in the past, lactate, in addition to glucose, is increasingly admitted as a supplementary fuel for neurons and, more recently, as a signaling molecule in the brain. Our aim was to investigate the neuroprotective effect of lactate in a neonatal (seven day old) rat model of hypoxia-ischemia. Pups received intra-peritoneal injection(s) of lactate (40 μmol). Size and apparent diffusion coefficients of brain lesions were assessed by magnetic resonance diffusion-weighted imaging. Oxiblot analyses and long-term behavioral studies were also conducted. A single lactate injection induced a 30% reduction in brain lesion volume, indicating a rapid and efficient neuroprotective effect. When oxamate, a lactate dehydrogenase inhibitor, was co-injected with lactate, the neuroprotection was completely abolished, highlighting the role of lactate metabolism in this protection. After three lactate injections (one per day), pups presented the smallest brain lesion volume and a complete recovery of neurological reflexes, sensorimotor capacities and long-term memory, demonstrating that lactate administration is a promising therapy for neonatal HI insult.

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