scholarly journals RARE-36. BORTEZOMIB WOKE UP A PATIENT WITH ANTI-NMDA RECEPTOR ENCEPHALITIS REFRACTORY TO STANDARD THERAPY AND LONG TERM FOLLOW-UP

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi229-vi229
Author(s):  
Kong Xiao-Tang ◽  
Leonid Groysman ◽  
Cyrus Dastur ◽  
Beverly Fu ◽  
Daniela Bota
Keyword(s):  
Nmda Receptor ◽  
Standard Therapy ◽  
Autoimmune Encephalitis ◽  
Ivig Therapy ◽  
Term Care ◽  
Good Communication ◽  
Nmda Receptor Encephalitis ◽  
Long Term Follow Up ◽  
One Year

Abstract OBJECTIVE To report a case with refractory NMDA encephalitis in comatose for 18 months, who was treated successfully with bortezomib. BACKGROUND Anti-NMDA encephalitis is a rare autoimmune encephalitis. Standard therapy include corticosteroid, IVIG or plasma exchange, cyclophosphamide, rituximab, and tumor removal. Refractory cases are very severe and often stay in ICU on ventilation for several months to years. Bortezomib for the treatment of refractory anti-NMDA receptor encephalitis was reported. We have applied the treatment to our refractory case and successfully woke up the patient. And we have followed up the patient for 3 years. METHODS Case report. RESULTS A 40 yo male was diagnosed as anti-NMDA encephalitis. Standard therapy was applied. After stabilization, the patient was eventually discharged to ICU at a long term care subacute hospital. The patient was brought back for more Rituxan or steroid or IVIG therapy. The condition had not improved at all. Eighteen months in comatose, the patient had worsening NMDA titer in CSF to 1:640. Decision was made to start bortezumib as reported with modification: 1.3 mg/m2 bortezomib were administered on days 1, 8, 11 and 14 and allowed two weeks off therapy. After first cycle, the patent started to talk first word “hurt.” After 6 cycles, the patient sat up and started riding bicycles for physical therapy. The NMDA titer in CSF was reduced to 1:40 at the end of 6 cycles. One year later, the patent stood up and ambulated with a walker. One and half year later, the patient walks without assistance and his speech and cognition have significantly improved with good communication with family members and staff. CONCLUSIONS Proteasome inhibitor bortezomib might be considered to be the third line therapy as early as possible if the first line and second line are ineffective to treat anti-NMDA receptor encephalitis.

scholarly journals Efficacy of tacrolimus as long-term immunotherapy for neuronal surface antibody-mediated autoimmune encephalitis

2022 ◽  
Vol 13 ◽  
pp. 204062232110630
Author(s):  
Chenchen Liu ◽  
Suqiong Ji ◽  
Huajie Gao ◽  
Zhuajin Bi ◽  
Qin Zhang ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Autoimmune Encephalitis ◽  
Efficacy And Safety ◽  
Nmda Receptor Encephalitis ◽  
Significant Difference ◽  
Observational Cohort ◽  
Surface Antibody ◽  
Retrospective Observational Cohort Study

Aims: We aimed to verify the efficacy and safety of tacrolimus as long-term immunotherapy for the treatment of neuronal surface antibody-mediated autoimmune encephalitis (AE) during the first attack. Methods: In this retrospective observational cohort study, patients with neuronal surface antibody-mediated AE who experienced the first attack were enrolled. We compared the outcomes of 17 patients who received tacrolimus with those of 47 patients treated without tacrolimus. Patients were assessed at onset and 3, 6, and 12 months, as well as at the last follow-up, by using the modified Rankin scale (mRS) and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The efficacy of tacrolimus was also compared in a subgroup of patients with anti-NMDA receptor encephalitis. Results: Among all patients with neuronal surface antibody-mediated AE, those receiving tacrolimus had lower median mRS scores [1 (IQR = 0–1) versus 2 (IQR = 1–3) in controls, p = 0.001)], CASE scores [2 (IQR = 1–3) versus 3 (IQR = 2–7), p = 0.006], and more favorable mRS scores (94.1% versus 68.1%, p = 0.03) at the 3-month follow-up. No difference was found at the last follow-up. There was no significant difference in the occurrence of relapse and adverse events between the two groups (11.8% versus 14.9%, p = 0.75). In the subgroup of patients with anti-NMDA receptor encephalitis, patients treated with tacrolimus had a lower median mRS score at the 3-month follow-up [1 (IQR = 0–2) versus 2 (IQR = 1–3), p = 0.03]; however, no difference in the outcome was detected at the last follow-up. Conclusion: Tacrolimus can be used as long-term immunotherapy in patients with neuronal surface antibody-mediated AE during the first attack. Treatment with tacrolimus appears to accelerate the clinical improvement of neuronal surface antibody-mediated AE.

scholarly journals Anti NMDA receptor encephalitis: a potentially treatable encephalitis

2020 ◽  
Vol 7 (7) ◽  
pp. 1624
Author(s):  
Archana K. M. ◽  
Sushma Save ◽  
Koppikar Rahul Gautam
Keyword(s):  
Nmda Receptor ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Ivig Therapy ◽  
Receptor Subunit ◽  
Nmda Receptor Subunit ◽  
Nmda Receptor Encephalitis ◽  
Autonomic Instability ◽  
High Index Of Suspicion ◽  
Age Range

Anti NMDA receptor encephalitis is autoimmune encephalitis where antibodies are directed against NMDA receptor subunit. It represents a new category of immune-mediated disorder that is often paraneoplastic, presenting with neuropsychiatric symptoms, which is treatable and can be diagnosed serologically affecting both children and adults. Patient can have variable clinical presentation ranging from prodromal illness, neuropsychiatric symptoms, seizures, autonomic instability, hyperkinesias, catatonia, hypoventilation and with or without an associated teratoma. A positive serum or CSF sample screening for antibodies to the NMDA receptor subunit is confirmative. Supportive findings include CSF study, EEG and MRI Brain. The first-line therapies includes IVIG, corticosteroids or plasma exchange. Second line immunotherapy is rituximab or cyclophosphamide or both. Given the high mortality rate (up to 25%), the likelihood of presentation across the age range and the potential for treatment, a high index of suspicion is warranted by clinicians. Authors report a case of a 5 year old child with anti NMDA receptor encephalitis who responded well to IVIG therapy.

scholarly journals Anti-NMDA Receptor Encephalitis Presented with Acute Psychotic Symptoms: A Case Report

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Shabnam Asadi ◽  
Fahimeh Hajiakhundi ◽  
Fatemeh Sadat Mirfazeli ◽  
Homa Mohammadsadeghi
Keyword(s):  
Nmda Receptor ◽  
Psychiatric Hospital ◽  
Autoimmune Encephalitis ◽  
Female Adolescent ◽  
Psychotic Symptoms ◽  
Brain Images ◽  
Nmda Receptor Encephalitis ◽  
History Of ◽  
One Year ◽  
Perceptual Changes

Introduction: Anti-N-methyl-d-aspartic acid receptor (NMDAR) autoimmune encephalitis is one of the most challenging disorders with both psychiatric and neurologic presentations. Approximately three-fourth of patients with anti-NMDA receptor encephalitis (anti-NMDARE) first referred to psychiatrists. Case Presentation: A 15-year-old female adolescent was admitted to a psychiatric hospital. She presented a history of behavior changes, including aggression, the symptoms mimicking depression, and suicidal ideation, for one year. Then she showed more severe disinhibited behaviors, stupor, and generalized tonic-colonic seizure, so she was hospitalized in a general hospital. After neurological assessments, she was referred to a psychiatric hospital due to her uncontrollable aggressive behaviors in addition to sleep disturbance, rapid mood swings, restlessness, stereotypic behaviors, fluctuating attention and concentration, misidentification delusion, and perceptual changes. All lab tests and brain images were normal. Electroencephalograms showed generalized sharp and slow waves. The autoimmune panel tests were requested. When anti-glutamate receptor antibody was finally reported in her CSF, anti-NMDARE was finally diagnosed. Her symptoms improved by receiving five corticosteroid pulses. Conclusions: In patients with first and acute psychotic symptoms, especially in young individuals with seizures, the possibility of autoimmune disorders should be kept in mind, and complementary tests must be done for autoantibody detection.

Transcatheter closure of defects within the oval fossa using the Amplatzer® Septal Occluder

2002 ◽  
Vol 12 (3) ◽  
pp. 224-228 ◽  
Author(s):  
Haifa Abdul Latiff ◽  
Mazeni Alwi ◽  
Hasri Samion ◽  
Geetha Kandhavel
Keyword(s):  
Transcatheter Closure ◽  
Standard Procedure ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Term Outcome ◽  
Long Term Follow Up ◽  
One Year ◽  
The Right

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer® Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer® device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 ± 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer® Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.

Abstract 18649: Long-term Outcome and the Mechanisms of Pulmonary Antrum Radial-linear Ablation in Patients With Paroxysmal Atrial Fibrillation

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Xue Zhao ◽  
Jianqiang Hu ◽  
Yan Huang ◽  
Yawei Xu ◽  
Yanzhou Zhang ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Paroxysmal Atrial Fibrillation ◽  
Holter Monitoring ◽  
Pericardial Tamponade ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Long Term Follow Up ◽  
One Year

Objectives: The aim of this study was to determine the mechanisms and effectiveness of pulmonary antrum radial-linear (PAR) ablation in comparison with pulmonary vein isolation (PVI) in patients with paroxysmal atrial fibrillation (AF) after a long-term follow-up. Background: The one-year follow up data suggested that PAR ablation appeared to have a better outcome over the conventional PVI for paroxysmal AF. Methods: The enrollment occurred between March, 2011, and August, 2011, with the last follow-up in May, 2014. A total of 133 patients with documented paroxysmal AF were enrolled from 5 centers and randomized to PAR group or PVI group. Event ECG recorder and Holter monitoring were conductedduring the follow-up for all patients. Results: The average procedure time was 151±23 min in PAR group and 178±43 min in PVI group ( P <0.001). The average fluoroscopy time was 21±7 min in PAR group and 27±11 min in PVI group ( P= 0.002). AF triggering foci were eliminated in 59 patients (89.4%) in PAR group, whereas, only 4 patients (6.0%) in PVI group (P<0.001).At median 36 (37-35) months of follow-up after single ablation procedure, 43 of 66 patients in PAR group (65%) and 28 of 67 patients in PVI group (42%) had no recurrence of AF off antiarrhythmic drug (AAD) (P=0.007); and 47 of 66 patients in PAR group (71%) and 32 of 67 patients in PVI group (48%) had no recurrence of AF with AAD (P=0.006). At the last follow-up, the burden of AF was significantly lower in PAR group than in PVI group (0.9% ± 2.3% vs 4.9% ± 9.9%;90th percentile, 5.5% vs 19.6%; P=0.008). No major adverse event (death, stroke, PV stenosis) was observed in all the patients except one case of pericardial tamponade. Conclusions: PAR ablation is a simple, safe, and effective strategy for the treatment of paroxysmal AF with better long-term outcome than PVI. PAR ablation might exhibit the beneficial effect on AF management through multiple mechanisms. Registration: ChiCTR-TRC-11001191

scholarly journals Meningiomas of pineal region in children

2007 ◽  
Vol 65 (4a) ◽  
pp. 1000-1006 ◽  
Author(s):  
Hamilton Matushita ◽  
Fernando Campos Pinto ◽  
José Píndaro Pereira Plese
Keyword(s):  
Clinical Presentation ◽  
Complete Removal ◽  
Pineal Region ◽  
Total Resection ◽  
Long Term Follow Up ◽  
One Year ◽  
Velum Interpositum ◽  
Transtentorial Approach

Meningiomas are uncommon tumors in children and either more rarely encountered in the pineal region. We report two cases of meningioma of the pineal region in children. One of these cases was a five years-old girl and the other a one year-old boy. No specific clinical presentation or tomographic examinations findings was identified before treatment, suggestive of a diagnosis of menigioma. The clinical and laboratory features were very similar to the most common tumors of the pineal region. Prior to the surgery, the histology of these tumors was not suspected. Both patients underwent direct surgery and complete removal was achieved by a suboccipital transtentorial approach. The tumors originated from velum interpositum in both cases. At the follow up, one case presented with recurrence six years later, and she underwent a reoperation with total resection without morbidity. Long-term follow up presented no other recurrences.

Frequency and temporal sequence of clinical features in adults with anti-NMDA receptor encephalitis presenting with psychiatric symptoms

2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
Author(s):  
Ronald J. Gurrera
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Signs And Symptoms ◽  
Nmda Receptor Encephalitis ◽  
Nmdar Encephalitis ◽  
Unexplained Fever ◽  
Study Inclusion

AbstractBackgroundAnti-NMDA receptor (NMDAr) encephalitis is the most common autoimmune encephalitis in adults. It mimics psychiatric disorders so often that most patients are initially referred to a psychiatrist, and many are misdiagnosed. Without prompt and effective treatment, patients are likely to suffer a protracted course with significant residual disability, or death. This study focuses on the frequency and chronology of salient clinical features in adults with anti-NMDAr encephalitis who are likely to be first evaluated by a psychiatrist because their presentation suggests a primary psychiatric disorder.MethodsA systematic search of PubMed and EMBASE databases identified published reports of anti-NMDAr encephalitis associated with prominent behavioral or psychiatric symptoms. After eliminating redundancies, the frequencies and relative timing of clinical features were tabulated. Signs and symptoms were assigned temporal ranks based on the timing of their first appearance relative to the first appearance of other signs and symptoms in each patient; median ranks were used to compare temporal sequencing of both individual features and major symptom domains.ResultsTwo hundred thirty unique cases (185 female) met study inclusion criteria. The most common features were seizures (60.4%), disorientation/confusion (42.6%), orofacial dyskinesias (39.1%), and mutism/staring (37.4%). Seizures, fever, and cognitive dysfunction were often the earliest features to emerge, but psychiatric features predominated and sequencing varied greatly between individuals.ConclusionsClinicians should consider anti-NMDAr encephalitis when new psychiatric symptoms are accompanied by a recent viral prodrome, seizures or unexplained fever, or when the quality of the psychiatric symptoms is unusual (e.g. non-verbal auditory hallucinations).

scholarly journals Improved Biochemical Parameters in Patients Who Undergo Early Resection in Isolated Ileocaecal Crohn’s Disease

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Mirzafaraz Saeed ◽  
Hari Hullur ◽  
Amro Salem ◽  
Abbas Ali ◽  
Yousif Sahib ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Surgical Resection ◽  
Complete Blood Count ◽  
Long Term Results ◽  
Number Of Patients ◽  
Long Term Follow Up ◽  
One Year

Introduction. The aim of this study is to evaluate the outcome of introduction of early surgery in the course of isolated ileocecal Crohn’s disease, where there is no absolute indication of surgery. Methods. Observational study involving patients with isolated ileocecal Crohn’s disease who underwent early surgical resection (within one year of the presentation of the hospital). A complete blood count, ESR, and CRP were done and compared between the preoperative value, 1st postoperative visit (3-4 weeks), and last follow-up visit. Statistical analysis was done using Analysis of Variance (ANOVA) to compare the different figures. Results. There was a statistically significant increase in the hemoglobin levels between preoperative, postoperative, and long-term follow-up and a significant decrease in leukocyte count between the pre- and postoperative values (F=19.8, p<0.001 and F=8.9, p=0.002, resp.). Similarly, the ESR and CRP values were decreased significantly at long-term follow-up (F=8.5, p=0.019 and F=8.3, p=0.013, resp.). Conclusion. Early surgical resection in isolated ileocaecal Crohn’s disease achieved significant biochemical improvements. These successful results in this small number of patients indicate that early surgical intervention may provide better outcomes. These initial results encourage larger and comparative studies of long-term results versus long-term use of biological agents.

scholarly journals A Conservative Approach to a Peripheral Ameloblastoma

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Rocco Borrello ◽  
Elia Bettio ◽  
Christian Bacci ◽  
Marialuisa Valente ◽  
Stefano Sivolella ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Soft Tissues ◽  
Complete Removal ◽  
The Other ◽  
Conservative Approach ◽  
Choice Of Treatment ◽  
Long Term Follow Up ◽  
One Year

Peripheral Ameloblastoma (PA) is the rarest variant of ameloblastoma. It differs from the other subtypes of ameloblastoma in its localization: it arises in the soft tissues of the oral cavity coating the tooth bearing bones. Generally, it manifests nonaggressive behavior and it can be treated with complete removal by local conservative excision. In this study we report a case of PA of the maxilla in a 78-year-old female patient and we describe the four different histopathological patterns revealed by histological examination. After local excision and diagnosis, we planned a long term follow-up: in one year no recurrence had been reported. The choice of treatment is illustrated in Discussion.

Custody of the Myelodysplastic Child: Implications for Selection for Early Treatment

PEDIATRICS ◽  
1974 ◽  
Vol 53 (2) ◽  
pp. 253-256
Author(s):  
Patricia W. Hayden ◽  
David B. Shurtleff ◽  
Arline B. Broy
Keyword(s):  
University Hospital ◽  
Contributory Factor ◽  
Foster Home ◽  
Term Care ◽  
Long Term Follow Up ◽  
Selection For ◽  
Foster Home Care ◽  
Highly Correlated ◽  
High Level

Of 173 patients with myelodysplasia followed in the Birth Defects Center at University Hospital between 1968 and 1972, 30 (17%) have been placed outside their natural families for temporary or long-term care. Only one has been adopted and five have been institutionalized; the remainder have been in foster home care. High level paralysis, mental retardation, and lower socioeconomic status correlate positively with placement. In this series, gender was not a contributory factor. An initial "hopeless" prognosis and/or selection for "no treatment" were decisions often made prior to referral to this center but were highly correlated to placement. Considering the multiple medical, emotional, and economic problems facing these families, relinquishment of custody should be anticipated in a significant percentage of cases. To date, placement outside the natural family has been viewed primarily as abandonment or as an emergency solution to a crisis. Long-term follow-up study of this group of children may indicate that transfer of custody can be a positive therapeutic alternative for the child and his family.

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