Case of a Two-Year-Old Boy With Recurrent Seizures, Abnormal Movements, and Central Hypoventilation

We present a case of a 2 year-old boy with anti-NMDAR encephalitis to illustrate and discuss the differences in neurological presentation, frequency of symptoms, and association with underlying tumor between children and adults.
Source: Seminars in Pediatric Neurology - Category: Neurology Authors: Source Type: research

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Anti-N-methyl-d-aspartate receptor (NMDA) encephalitis is characterized by a wide spectrum of neuropsychiatric symptoms. Approximately one quarter of the patients with anti-NMDA encephalitis have been reported to have an underlying neoplasm, such as ovarian teratomas, small cell lung carcinoma, uterine adenocarcinoma, prostate adenocarcinoma, Hodgkin lymphoma, neuroblastoma, and pancreatic neuroendocrine tumor [1]. To our knowledge, however, NMDA encephalitis associated with pheochromocytoma has never been reported.
Source: Parkinsonism and Related Disorders - Category: Neurology Authors: Tags: Correspondence Source Type: research
AbstractThe spectrum of ovarian tumors in the pediatric population differs significantly from that in adults. Germ cell tumors are the predominant class of ovarian tumors in children, whereas epithelial tumors are the most common in adults. Ultrasonography is the modality of choice for the initial evaluation of pediatric ovarian tumors. Determining the diagnosis based on imaging may prove difficult, and combining the imaging findings with the clinical scenario is very helpful in reaching a differential diagnosis during clinical practice. We will discuss the spectrum of ovarian neoplasms in the pediatric population and desc...
Source: Abdominal Imaging - Category: Radiology Source Type: research
Conclusions KLHL11-abs associate with a spectrum of syndromes and tumors wider than those previously reported; 44% of patients have concurrent neuronal antibodies, some of them (anti-NMDAR) pathogenically relevant. Brain immunostaining is not useful for routine screening of KLHL11-abs.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, All Clinical Neurology, Paraneoplastic syndrome Article Source Type: research
ConclusionsSymptomatology and auxiliary findings indicate that ovarian teratoma may be the immunologic trigger for anti-NMDAR encephalitis patients, while viral infection is likely to play a major part in pathogenesis for those without any detectable tumor. Overall, anti-NMDAR encephalitis patients with teratomas present with milder neurological symptoms and have better long-term outcomes after tumor removal.
Source: Seizure - Category: Neurology Source Type: research
The objective of this study was to determine the incidence of anti-NMDAR encephalitis in patients in whom a teratoma was removed. As far as we know, there has been no report on the incidence of anti-NMDAR encephalitis in patients in whom a teratoma was removed.
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Source Type: research
This study showed that dysplastic neuroglial tissue resembling central nervous system tumors, which might promote autoimmunity, distinguished the case with NMDAR encephalitis from the controls. Additionally, abnormal expression of NR1 occurs in non-neural tissues and could be triggered by inflammation and participate in autoimmunity. PMID: 31756658 [PubMed - as supplied by publisher]
Source: Clinical Immunology - Category: Allergy & Immunology Authors: Tags: Clin Immunol Source Type: research
Clinical features that suggest that a first episode of psychosis is caused by autoimmune encephalitis include the following: (a) lack of long ‐term (cognitive and negative) psychiatric prodromes; (b) the presence of an atypical psychotic clinical profile; and (c) hypersensitivity to the side effect of antipsychotic medications. AbstractMost patients with anti ‐NMDA receptor (NMDAR) encephalitis present with acute psychosis which is difficult to differentiate from psychotic episodes related to a primarily psychiatric disease. A precise description of the psychiatric phenotype of this disease would greatly facilitate the...
Source: Clinical Case Reports - Category: General Medicine Authors: Tags: CASE REPORT Source Type: research
Conclusion: The patient's psychiatric symptoms were most likely caused by anti-NMDA receptor encephalitis. Her polymorphic psychotic symptoms first occurred after she had received a Tdap-IPV booster vaccination. Although the vaccination cannot have caused the initial antibody formation since IgG serum antibodies were detected only 3 days after administration of the vaccine, the vaccine may have exerted immunomodulatory effects. MRI, EEG, and CSF findings were non-specific; however, FDG-PET identified brain involvement consistent with anti-NMDA receptor encephalitis. This case shows the importance of implementing a multimod...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
This study included 45 patients treated between July 2015 and November 2016. Laparoscopic oophorocystectomy was performed in 11 female patients with teratomas. Eleven patients required tracheal intubation or tracheotomy and ventilation. The patients were hospitalized for an average of 25.2 days. The mental and neurological symptoms were significantly relieved 23.6 ± 4.8 days after surgery or immunotherapy. Near-normal function was restored in 11 patients, while 34 patients had varying degrees of dysfunction at discharge. After follow-up of 1 to 18 months, 24 patients were found to have permanent impairments. Appro...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research
Rationale: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder that is most frequently induced by ovarian teratoma in young females. The condition can be controlled and reversed via ovarian tumor resection and immunotherapy. However, anti-NMDAR encephalitis induced by bilateral ovarian teratomas with distinct histopathologic types is rarely reported in the literature. Patient concerns: A 23-year-old woman presented with seizures. Diagnoses: The diagnosis was anti-NMDAR encephalitis associated with ovarian teratomas based on positive anti-NMDAR antibody tests in both the cerebrospinal f...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
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