China
Africa
Explore chapters and articles related to this topic
Post-viral syndromes
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Anusha K. Yeshokumar, Eliza Gordon-Lipkin, Brenda Banwell
Traditional neurologic evaluation has only moderate sensitivity and specificity in the evaluation of anti-NMDA receptor encephalitis. A study of 100 patients [86] found increased FLAIR/T2 signal on MRI of the brain in 55 (55%), most commonly in the medial temporal lobes, and faint or transient contrast enhancement of the cortex, meninges, or basal ganglia in 14 (14%). Follow-up imaging of 42 of these patients showed resolution of abnormalities in 26 (62%), while the remainder had developed cortical atrophy or had stable subcortical, non-enhancing increased FLAIR/T2 signal.
Case 18: First Fit
Published in Layne Kerry, Janice Rymer, 100 Diagnostic Dilemmas in Clinical Medicine, 2017
NMDA receptors are a class of glutamate receptors that are responsible for processes involved in memory function, synaptic plasticity and generation of rhythms involved in breathing and locomotion. Anti-NMDA receptor encephalitis is a form of autoimmune encephalitis where patients develop antibodies to the NR-1 subunit of the NMDA receptor.
Oncological effects on the central nervous system
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Anti-NMDA-receptor encephalitis is a diffuse encephalitis which predominates in women (81%) and young patients (37% <18 years, 95% <45 years). A prodrome of fever, headache, nausea, and/or vomiting is followed by psychiatric symptoms and behavioural abnormalities (>95%) which often dominate other symptoms, such as memory deficits (60%–80%) (7). Seizures and status epilepticus are common (70%), as are abnormal movements. Cranial imaging is often normal; however, in around 50% of cases T2 or FLAIR signal hyperintensity may be seen in a variety of brain regions: hippocampi, cerebellar or cerebral cortex, insular regions, basal ganglia, brain stem, and, infrequently, the spinal cord (Figure 37.2). These findings are usually mild or transient (7). CSF and electroencephalogram (EEG) are usually abnormal (7). Ovarian teratomas are by far the commonest associated tumour, 94% in a large series (4). The presence of a tumour is more common in females and in patients between 12 and 45 years (4). Tumours other than teratomas are rare, but found preferentially in patients older than 45 years. Only 6% of male patients have underlying tumours; lymphoma has been reported occasionally (7). Screening for tumours should be performed according to the European guidelines, summarized in Table 37.4 (8). In many cases of PNS, the neurological symptom onset precedes the tumour diagnosis and the tumours are often initially too small to detect; however, most tumours will manifest themselves within a year. If no tumour is found with available methods, close oncological follow-up every 3–6 months for at least 5 years is advised by the current guidelines for syndromes associated with onconeural antibodies (8).
The clinical features, treatment and outcomes of 33 children from Northwestern China with Anti-N-methyl-D-aspartate receptor encephalitis
Published in Neurological Research, 2022
Xiangjun Dou, Dongjing Li, Fang Wu, Zhijing Wang, Mengmeng Niu, You Wu, Ting Deng, Dong Wang, Xia Li
Anti-NMDA receptor encephalitis is an antibody-mediated disease that was originally reported by Dalmau and colleagues in 2007 [1]. Anti-NMDA receptor encephalitis was first described in adult women with ovarian teratoma, yet it is increasingly recognized in children and men, particularly in the pediatric population [1,2]. The clinical presentation of anti-NMDA receptor encephalitis is characterized by seizures, psychiatric symptoms, language and memory deficits, abnormal involuntary movements, disturbances of consciousness, autonomic nervous dysfunction and breath instability [3]. Common treatments include tumor resection and first-line immunotherapy consisting of corticosteroids, IVIg or plasma exchange. Patients that do not have tumors may not respond to the first-line immunosuppressive therapies, and second-line immunotherapy including cyclophosphamide or rituximab, or in combination should be considered [2,3].
Synergistic effect of rituximab with anti-epileptic drugs in treating sero-negative limbic encephalitis: a case report
Published in International Journal of Neuroscience, 2020
The treatment strategies for AE include first line and second line immunotherapies [4]. About 10% of anti-NMDA receptor encephalitis patients are refractory to first line and second line therapies and further treatments are required [8]. The LE associated with antibodies against intracellular neuronal antigens (onconeural antibodies) is predominantly mediated by cytotoxic T-cell mechanisms and has limited response to treatment; whereas LE associated with antibodies against neuronal surface/synaptic antigens has less frequently brain inflammatory infiltrates, less association with cancer and has much better response to immunotherapy. The treatment strategies for this kind of LE are similar to that of AE. The treatment algorithm for sero-negative LE included IVMP, IVIg, plasmapheresis and active search for occult malignancy for at least 5 years [2]. The immunologic mechanism of sero-negative LE is not clear; some antibodies against neuronal surface/synaptic antigens might be discovered in the future. So, we think that second line immunotherapies such as rituximab could also be tried if first line therapies resulted in limited effect in sero-negative LE. It is reported that delayed treatment with rituximab even 400 days after the onset of AE could improve the cognitive function of the patient [9]. We also found that treatment with rituximab more than 3 months after the onset of the LE could reduce the seizures significantly. It is also reported that rituximab is safe [10].
Anti-NMDA receptor encephalitis: two case reports associated with ovarian teratoma and a literature review
Published in Journal of Obstetrics and Gynaecology, 2019
Javier Sancho-Saúco, Virginia Corraliza-Galán, Jesus Lázaro-Carrasco de la Fuente, Concepcion Sánchez-Martínez, Irene Pelayo-Delgado, Maria Jesus De Pablos-Antona, Elena Cabezas-López, Juan Carlos García-Pérez
Patient two was 33-year-old woman, who had a vaginal delivery five months ago. This patient went to the Emergency Department with the following symptoms: a high fever, generalised headache and behavioural changes for three days. In the Emergency Department, she showed a decreased level of consciousness and sleepiness. Suddenly, she suffered a generalised tonic seizure that lasted for five minutes and had an important postictal period. She needed to be admitted to the ICU to perform cranial CT scan (irrelevant findings) and a lumbar puncture. The patient was diagnosed with meningoencephalitis of an unknown aetiology. Six days later, the results showed that anti-NMDA antibodies were positive and the serum tumour makers were negative. The patient was diagnosed with autoimmune anti-NMDA receptor encephalitis. A pelvic MRI revealed a 15 mm and parietal calcification in right ovary. Transvaginal ultrasound reported an image in the right ovary, with similar characteristics to the teratoma. Laparoscopic right salpingo-oophorectomy was performed and pathologist confirmed the existence of a mature cystic teratoma without immature components inside. One month later after the surgery, anti-NMDA determination was performed in serum and cerebrospinal fluid and they remained positive. No evidence of injury in the left ovary was shown in an ultrasound or pelvic MRI. However, because of a long and slow evolution, intensivists proposed a left salpingo-ooforectomy but the Department of Gynecology refused it. Currently, the patient remains hospitalised in ICU.