scholarly journals Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Bissene Douma ◽  
Thouraya Ben Younes ◽  
Hanene Benrhouma ◽  
Zouhour Miladi ◽  
Imen Zamali ◽  
...  
Keyword(s):  
Brain Mri ◽  
Autoimmune Encephalitis ◽  
Therapeutic Interventions ◽  
High Dose ◽  
First Line ◽  
Nmda Receptor Encephalitis ◽  
Progressive Encephalopathy ◽  
Laboratory Features ◽  
Fluid Attenuated Inversion Recovery ◽  
The Central Nervous System

Background. Autoimmune encephalitis (AE) is a rapidly progressive encephalopathy caused by antibodies targeting neurons in the central nervous system generating specific immune responses. It is increasingly recognized in children. Objective. To describe clinical, neuroimaging, and laboratory features, treatment, and outcome in a cohort of Tunisian children with AE. Methods. We conducted a retrospective review of the medical records of all children attending the Department of Child and Adolescent Neurology (Tunis) with autoimmune encephalitis between 2004 and 2020. Clinical, neuroimaging, laboratory features, therapeutic data, and outcome were analyzed. Results. Nineteen children were included in the study (12 girls and 7 boys). The median age at diagnosis was 7.68 years (range: 10 months-13 years). The most frequent manifestations were seizures and behavioral disorders. Eleven cases were diagnosed with anti-NMDA receptor encephalitis, 4 cases with anti-Ma2 encephalitis, 3 cases with anti-GAD encephalitis, and 1 case with anti-SOX1 encephalitis. Brain MRI showed increased T2 and fluid-attenuated inversion recovery (FLAIR) signal of the temporal lobe in 5 patients. Eighteen patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin). One patient with anti-GAD encephalitis died despite escalating immunotherapy. Conclusion. Diagnosis of autoimmune encephalitis is challenging in children, because of misleading presentations. An early and accurate diagnosis is important to enable proper therapeutic interventions.

scholarly journals Anti-Alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic Acid Receptor Encephalitis: A Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Tian-Yi Zhang ◽  
Meng-Ting Cai ◽  
Yang Zheng ◽  
Qi-Lun Lai ◽  
Chun-Hong Shen ◽  
...  
Keyword(s):  
Medial Temporal Lobe ◽  
Short Term Memory ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Abnormal Behavior ◽  
High Dose ◽  
First Line ◽  
Acid Receptor ◽  
Cancer Breast ◽  
Fluid Attenuated Inversion Recovery

Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis, a rare subtype of autoimmune encephalitis, was first reported by Lai et al. The AMPAR antibodies target against extracellular epitopes of the GluA1 or GluA2 subunits of the receptor. AMPARs are expressed throughout the central nervous system, especially in the hippocampus and other limbic regions. Anti-AMPAR encephalitis was more common in middle-aged women and most patients had an acute or subacute onset. Limbic encephalitis, a classic syndrome of anti-AMPAR encephalitis, was clinically characterized by a subacute disturbance of short-term memory loss, confusion, abnormal behavior and seizure. Magnetic resonance imaging often showed T2/fluid-attenuated inversion-recovery hyperintensities in the bilateral medial temporal lobe. For suspected patients, paired serum and cerebrospinal fluid (CSF) testing with cell-based assay were recommended. CSF specimen was preferred given its higher sensitivity. Most patients with anti-AMPAR encephalitis were complicated with tumors, such as thymoma, small cell lung cancer, breast cancer, and ovarian cancer. First-line treatments included high-dose steroids, intravenous immunoglobulin and plasma exchange. Second-line treatments, including rituximab and cyclophosphamide, can be initiated in patients who were non-reactive to first-line treatment. Most patients with anti-AMPAR encephalitis showed a partial neurologic response to immunotherapy.

scholarly journals Peculiarities of penetration of anti-tuberculosis drugs into the foci of infection in patients with tuberculosis of the central nervous system and the choice of pharmacotherapy

2020 ◽  
pp. 252-253
Author(s):  
R. Ruslami
Keyword(s):  
Central Nervous System ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Side Effects ◽  
Residence Time ◽  
Meta Analysis ◽  
Lower Mortality ◽  
High Dose ◽  
First Line ◽  
The Central Nervous System

Background. Tuberculosis (TB) of the central nervous system (CNS) is the most severe and life-threatening form of TB. Diagnosis of TB of CNS is difficult, and treatment is suboptimal. At present, the treatment of tuberculous meningitis (TBM) involves the same drugs and doses as for pulmonary TB, however, the problem is that not all the drugs cross the blood-brain barrier. Objective. To describe the penetration of anti-TB drugs (ATBD) into the foci of infection in patients with TB of CNS and the choice of pharmacotherapy. Materials and methods. Analysis of literature sources on this issue. Results and discussion. Options for optimizing the TBM treatment include a non-pharmacological approach, treatment prolongation, and increasing the residence time of ATBD at the infection site. A meta-analysis of 17 observational studies found no significant benefits of 9-month treatment over 6-month regimens. To increase the residence time of the drug in CNS, you can increase the dose of drugs that poorly penetrate the CNS, add drugs with better brain penetration characteristics, modify drug delivery systems and physical and chemical properties of drugs. The optimal dose provides the maximum effectiveness of the active substance on the background of the minimum number of side effects, so increase of the dose without taking into account the risks of side effects is not advisable. One of the main ATBD rifampicin is characterized by poor penetration into the cerebrospinal fluid. The killer activity of rifampicin depends on its concentration. In our own study, it was found that the administration of a high dose of rifampicin (600 mg) intravenously for 14 days was characterized by lower mortality in patients with TBM than treatment with oral rifampicin (standardized risk ratio was 0.42). Intravenous high-dose treatment was safe and well tolerated by patients. The disadvantages of this treatment include its high cost, invasiveness and poor availability. A meta-analysis of Indonesian patient data confirmed that high doses of rifampicin were associated with lower mortality (Svensson E. et al., 2019). Other drugs that need research in TBM include a new drug bedaquiline, fluoroquinolones (levofloxacin), linezolid. Isoniazid, pyrazinamide, cycloserine, ethionamide, prothionamide are also characterized by the good permeability to cerebrospinal fluid. Therefore, in a strategy to optimize the TBM treatment high-dose rifampicin, high-dose isoniazid and pyrazinamide (?) are the first line, and cycloserine, ethionamide, linezolid, delamanide, pretomanide – the second line. Conclusions. 1. Diagnosis of TB of CNS is difficult, and treatment is suboptimal. 2. Not all the drugs cross the blood-brain barrier. 3. Options for optimization of the TBM treatment include a non-pharmacological approach, prolongation of therapy and increasing the residence time of ATBD in the infection focus. 4. Administration of high-dose rifampicin (600 mg) intravenously for 14 days was characterized by lower mortality in patients with TBM than treatment with oral rifampicin. 5. High-dose rifampicin, high-dose isoniazid and pyrazinamide (?) are the first line of TBM treatment.

scholarly journals Use of Cyclophosphamide in a Child With Fulminant Acute Disseminated Encephalomyelitis

2018 ◽  
Vol 5 ◽  
pp. 2329048X1875463
Author(s):  
Hana Ayed ◽  
Mohammed W. Chaudhary ◽  
Raidah AlBaradie ◽  
Ali Mir
Keyword(s):  
Intravenous Immunoglobulin ◽  
Immunosuppressive Agents ◽  
Acute Disseminated Encephalomyelitis ◽  
High Dose ◽  
Intravenous Methylprednisolone ◽  
First Line ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
Minimal Improvement ◽  
The Central Nervous System

Acute disseminated encephalomyelitis is an immune-mediated inflammatory demyelinating disorder of the central nervous system. The first-line treatment is usually high-dose intravenous methylprednisolone. Intravenous immunoglobulin and plasmapheresis have also shown to be beneficial. Immunosuppressive agents like cyclophosphamide have been used in adults with fulminant acute disseminated encephalomyelitis. We report a case of a 3-year-old boy with fulminant acute disseminated encephalomyelitis. Minimal improvement was seen with high-dose intravenous methylprednisolone, intravenous immunoglobulin, and plasmapheresis. Based on the reports of cyclophosphamide being used successfully to treat adult patients with fulminant acute disseminated encephalomyelitis, we used it in our patient who then showed dramatic and quick improvement. We suggest that if conventional treatment fails, cyclophosphamide could be tried in pediatric patients with fulminant acute disseminated encephalomyelitis.

scholarly journals Catatonia in Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis Misdiagnosed as Schizophrenia

2020 ◽  
Vol 33 (3) ◽  
pp. 208
Author(s):  
André Ponte ◽  
Ana Brito ◽  
Camila Nóbrega ◽  
Sofia Pinheiro ◽  
João Gama Marques
Keyword(s):  
Psychotic Disorders ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Poor Response ◽  
Diagnostic Investigation ◽  
Nmda Receptor Encephalitis ◽  
Aspartate Receptor ◽  
Previous Diagnosis ◽  
The Central Nervous System ◽  
Prompt Diagnosis

Anti-N-Mmethyl-D-aspartate receptor encephalitis is an autoimmune disease of the central nervous system with prominent neurologic and psychiatric features. Symptoms appear progressively and sometimes with an exclusively psychiatric initial presentation. The patient’s evaluation should be meticulous, and we should use all the diagnostic tests required for the exclusion of entities that can mimic this disease. We report the diagnostic investigation of a case of anti-N-methyl-D-aspartate receptor encephalitis in a patient with a previous diagnosis of schizophrenia with poor response to antipsychotics. The aim of this case report is to highlight the importance of close surveillance for neuropsychiatric symptoms, especially catatonia, and to recognize autoimmune encephalitis in the differential diagnosis of psychotic disorders with neurological symptoms and resistance or intolerance to antipsychotics. A prompt diagnosis will contribute to a faster onset of therapy and an overall improvement in prognosis.

scholarly journals Mycoplasma Pneumoniae and Antibodies against Galactocerebroside in a 9-Year-Old Boy with Encephalitis

2018 ◽  
Vol 50 (01) ◽  
pp. 054-056
Author(s):  
Joost Smolders ◽  
Bart Jacobs ◽  
Anne Tio-Gillen ◽  
Frouke Nijhuis ◽  
Aad Verrips
Keyword(s):  
Cerebrospinal Fluid ◽  
Mycoplasma Pneumoniae ◽  
Brain Mri ◽  
Chain Reaction ◽  
Serologic Response ◽  
Acute Encephalitis ◽  
Epileptic Discharges ◽  
Fluid Attenuated Inversion Recovery ◽  
The Central Nervous System ◽  
Polymerase Chain

AbstractWe report the case of a 9 year-old boy, presenting with an acute encephalitis with cerebrospinal fluid pleiocytosis. MRI showed T2/FLAIR (fluid attenuated inversion recovery) hyperintense signals of basal ganglia and cortex, EEG (electro encephalogram) showed diffuse slowing with epileptic discharges. A repetitively elevated IgM/IgG serologic response against Mycoplasma pneumoniae was observed with polymerase chain reaction in serum and cerebrospinal fluid remaining negative. No other pathogen or antigen could be identified. High IgG and IgM levels against the glycolipid galactocerebroside were detected in serum but not in CSF. After treatment with corticosteroids, the patient fully recovered. Brain MRI and EEG investigation returned completely normal. Besides a primary infection of the central nervous system, M. pneumoniae is associated with a parainfectious encephalitis in children which may be mediated by antibodies to galactocerebroside.

scholarly journals Cryptogenic NORSE

2017 ◽  
Vol 4 (6) ◽  
pp. e396 ◽  
Author(s):  
Takahiro Iizuka ◽  
Naomi Kanazawa ◽  
Juntaro Kaneko ◽  
Naomi Tominaga ◽  
Yutaka Nonoda ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Clinical Features ◽  
Brain Mri ◽  
Ventilatory Support ◽  
Autoimmune Encephalitis ◽  
Refractory Status Epilepticus ◽  
Oligoclonal Bands ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Nmda Receptor Encephalitis

Objective:To report the distinctive clinical features of cryptogenic new-onset refractory status epilepticus (C-NORSE) and the C-NORSE score based on initial clinical assessments.Methods:A retrospective study was conducted for 136 patients with clinically suspected autoimmune encephalitis who underwent testing for autoantibodies to neuronal surface antigens between January 1, 2007, and August 31, 2016. Eleven patients with C-NORSE were identified. Their clinical features were compared with those of 32 patients with anti-NMDA receptor encephalitis (NMDARE).Results:The clinical outcome of 11 patients (median age, 27 years; 7 [64%] women) with C-NORSE was evaluated after a median follow-up of 11 months (range, 6–111 months). Status epilepticus was frequently preceded by fever (10/11 [91%]). Brain MRIs showed symmetric T2/fluid-attenuated inversion recovery hyperintensities (8/11 [73%]) and brain atrophy (9/11 [82%]). Only 2 of the 10 treated patients responded to the first-line immunotherapy, and 4 of the 5 patients treated with IV cyclophosphamide responded to the therapy. The long-term outcome was poor in 8 patients (73%). Compared with 32 patients with NMDARE (median age, 27 years; 24 [75%] women), those with C-NORSE had more frequent prodromal fever, status epilepticus, ventilatory support, and symmetric brain MRI abnormalities, had less frequent involuntary movements, absent psychobehavioral symptoms, CSF oligoclonal bands, or tumor association, and had a worse outcome. The C-NORSE score was higher in patients with C-NORSE than those with NMDARE.Conclusions:Patients with C-NORSE have a spectrum of clinical-immunological features different from those with NMDARE. The C-NORSE score may be useful for discrimination between them. Some patients could respond to immunotherapy.

scholarly journals Clinical case of encephalitis with antibodies to NMDA receptors

2019 ◽  
Vol 18 (4) ◽  
pp. 67-69
Author(s):  
L. U. Ulukhanova ◽  
N. S. Karnayeva ◽  
M. M. Yaraliev ◽  
A. G. Gadzhimirzaevа ◽  
S. G. Agaevа
Keyword(s):  
Nmda Receptor ◽  
Clinical Observation ◽  
Neuropsychiatric Symptoms ◽  
Fatal Outcome ◽  
Autoimmune Encephalitis ◽  
Final Diagnosis ◽  
Nmda Receptor Encephalitis ◽  
General Weakness ◽  
The Central Nervous System ◽  
Nr2 Subunits

Anti-NMDA receptor encephalitis is an autoimmune disease of the central nervous system caused by autoantibodies to the NR1 and NR2 subunits of the glutamate NMDA receptor, with the possibility of both death and rapid remission. The binding of these antibodies blocks receptors and causes slowly developing psychiatric disorders, motor disorders and seizures.Presented clinical observation in a 9-year-old patient. The disease debuted with prodromal flu-like symptoms, fever, headache and general weakness, after which neuropsychiatric symptoms, impaired memory and speech developed, further progression of the disease, convulsive status, and coma with a fatal outcome were noted. The final diagnosis of autoimmune encephalitis was made after identification of antibodies to the N-methyl-D-aspartate (NMDA) receptor in the blood.

scholarly journals Solitary juxtacortical lesion associated with anti-N-methyl-D-aspartate receptor encephalitis: a case report

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Rupan Gao ◽  
Xiang Zhang ◽  
Abhijeet Kumar Bhekharee ◽  
Yue Zhang
Keyword(s):  
Nmda Receptor ◽  
Frontal Lobe ◽  
Brain Mri ◽  
Brain Lesion ◽  
Autoimmune Encephalitis ◽  
Left Frontal Lobe ◽  
Solitary Lesion ◽  
Nmda Receptor Encephalitis ◽  
Epileptiform Discharges ◽  
Receptor Antibodies

Abstract Background Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe autoimmune encephalitis mediated by anti-NMDA receptor antibodies. Brain MRI manifestations vary and are non-specific. If there are any lesions, they tend to be diffusely or multifocally distributed. Solitary lesion is relatively rare. Case presentation We report a 16-year-old girl who initially presented with focal seizures but developed severe psychiatric and extrapyramidal symptoms later on. Brain MRI revealed a solitary juxtacortical demyelinating lesion in the left frontal lobe. No enhancement was noted. Electroencephalogram captured epileptiform discharges in the same region. NMDAR IgGs were tested positive in the serum and cerebrospinal fluid. Corticosteroid and intravenous IgG were administered and the patient completely recovered. Brain MRI revealed a fainter lesion in the left frontal lobe. Conclusion In very rare instances, anti-NMDA receptor encephalitis can present with a solitary brain lesion. A full panel of antibodies for autoimmune encephalitis is the key leading to the diagnosis.

scholarly journals Molecular mimicry of NMDA receptors may contribute to neuropsychiatric symptoms in severe COVID-19 cases

2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Veronika Vasilevska ◽  
Paul C. Guest ◽  
Hans-Gert Bernstein ◽  
Matthias L. Schroeter ◽  
Christian Geis ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Molecular Mimicry ◽  
Psychiatric Symptoms ◽  
Auditory Hallucination ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Altered Mental Status ◽  
Warning Signs ◽  
High Dose ◽  
Nmda Receptor Encephalitis

AbstractApproximately 30% of individuals with severe SARS-CoV-2 infections also develop neurological and psychiatric complaints. In rare cases, the occurrence of autoimmune encephalitis has been reported after SARS-CoV-2 infection. In this systematic review, we have identified eight SARS-CoV-2-associated cases of anti-NMDA receptor encephalitis. All had cerebrospinal fluid antibodies against the NMDA receptor and a recent onset of working memory deficits, altered mental status, or psychiatric symptoms, such as confusion, agitation, auditory hallucination, catatonia and speech dysfunction. All patients received high-dose steroid and immunoglobulin therapeutics and conditions improved in each case. These findings suggest that clinical attention should be paid to warning signs of autoimmune encephalitis in severe COVID-19 cases. If characteristic features of autoimmune encephalitis are present, autoantibody diagnostics should be performed and confirmed cases should be treated with immunotherapy to minimize neurological impairments.

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