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High incidence of NMDAR encephalitis among Austronesians: A population-based study in Sabah, Malaysia

Wong CK, Hor JY, Loo YP, Heng HS, Lee S, Perianen PP, et al.

J Neuroimmunol, 2021 07 15;356:577584.
PMID: 33933821 DOI: 10.1016/j.jneuroim.2021.577584

NMDAR encephalitis may be more common among non-Caucasians. A population-based study was conducted to estimate its incidence in Sabah, Malaysia, where the population consists predominantly of Austronesians (84%), and with a Chinese minority. Registries of NMDAR encephalitis at neurology referral centers were reviewed for case ascertainment. The annual incidence was 2.29/million (Austronesians: 2.56/million, Chinese: 1.31/million). Among pediatric population, the incidence was: Austronesians: 3.63/million, Chinese: 2.59/million. Our study demonstrated a higher incidence of NMDAR encephalitis among Austronesians than the predominantly Caucasian populations in Europe (0.5-0.9/million; pediatric: 0.7-1.5/million). Racial and genetic factors may contribute to risks of developing NMDAR encephalitis.

Matched MeSH terms: Anti-N-Methyl-D-Aspartate Receptor Encephalitis/diagnosis*; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/genetics; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/epidemiology*
Important differential in a patient presenting with neuropsychiatric symptoms: Anti-N-Methyl-D-Aspartate receptor encephalitis

Low JM

Med J Malaysia, 2017 10;72(5):306-307.
PMID: 29197887 MyJurnal

Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an immune mediated condition, which remains relatively unknown in Malaysia outside tertiary hospitals with neurology unit. It is often misdiagnosed as a psychiatric illness before definitive treatment is instituted. We report here an 18-year-old man who initially presented to the psychiatry unit before he was subsequently diagnosed as having anti-NMDAR encephalitis. To our knowledge, this is the first reported case of anti-NMDAR encephalitis in the east coast of Peninsular Malaysia.

Matched MeSH terms: Anti-N-Methyl-D-Aspartate Receptor Encephalitis/diagnosis*; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/drug therapy; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/physiopathology*
Fulltext Anti N-Methyl-D-Aspartate receptor encephalitis: An under-recognised cause of encephalitis

Rajahram GS, Nadarajah R, Lim KS, Menon J

Med J Malaysia, 2015 Dec;70(6):363-4.
PMID: 26988212 MyJurnal

Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an immune mediated condition with characteristic clinical presentation. We report the first case from Borneo, Sabah and the use of electroconvulsive therapy (ECT) in treating recalcitrant psychiatrist symptoms associated with this condition.

Matched MeSH terms: Anti-N-Methyl-D-Aspartate Receptor Encephalitis
Ovarian teratoma-associated anti-NMDAR encephalitis in a 12-year-old girl

Lwin S, San Yi M, Mardiana K, Woon SY, Nwe TM

Med J Malaysia, 2020 11;75(6):731-733.
PMID: 33219185

The association of ovarian teratoma and anti-N-Methyl-Daspartate receptor (anti-NMDAR) is one of the most common autoimmune encephalitis syndromes and it is a serious and potentially fatal pathology that occurs in young women. This case report describes of a pediatric patient with anti-NMDAR encephalitis. A-12-year-old girl presented with abnormal behavior for one week came to Emergency Department of Sarawak General Hospital, Malaysia. She had psychotic spectrum symptoms including suicidal tendency. She was diagnosed with anti-NMDAR encephalitis as positive antibody was seen in her cerebrospinal fluid. She was treated with Injection Immunoglobulin. She turned out to have teratoma which was successfully removed later. Her progress was remarkable after the surgery with the Immunoglobulin. A multi-disciplinary team involving a psychiatrist, neurologist and gynaecologist liaised with intensivist to successfully manage the case and achieve the good outcome.

Matched MeSH terms: Anti-N-Methyl-D-Aspartate Receptor Encephalitis
Fulltext Organic disorder with neuropsychiatric symptoms - case report of anti-NMDA receptor encephalitis with neuropsychiatric manifestations

Sivarooban, V., Yogitagavari, Y., Che, C.K., Lee, C.W.

Malaysian Journal of Psychiatry, 2017;26(1):0-0.
MyJurnal

Neuropsychiatric symptoms are symptoms that caused by organic brain
disorders. Multiple neuropsychiatric symptoms can occur concurrently in the
course of brain related organic disorders. Two main components of
neuropsychiatric symptoms are cognitive impairment and disturbance of
consciousness while other neuropsychiatric symptoms, such as hallucinations,
delusions, mood disorders, anxiety, apathy, behavioural and personality
changes also commonly occur. Some of the mild neuropsychiatric symptoms
could possibly be the earliest manifestations of brain related organic
disorders. Clinicians should carefully evaluate organic factors in the
treatment of psychosis, especially in patients of very young age or older age.
They should have enough knowledge and experience in the integrating
neuroscience, that is, neuropsychiatry. The present report illustrates a case of
organic disorder with neuropsychiatric manifestations. It is about a young
male patient who was diagnosed with anti-NMDA receptor encephalitis and
subsequently developed acute delirium secondary to the illness.

Matched MeSH terms: Anti-N-Methyl-D-Aspartate Receptor Encephalitis
Development of the clinical assessment scale in autoimmune encephalitis

Lim JA, Lee ST, Moon J, Jun JS, Kim TJ, Shin YW, et al.

Ann Neurol, 2019 03;85(3):352-358.
PMID: 30675918 DOI: 10.1002/ana.25421

OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale.
METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38).
RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p

Matched MeSH terms: Anti-N-Methyl-D-Aspartate Receptor Encephalitis/complications; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/physiopathology; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/psychology
Fulltext EEG is sensitive in early diagnosis of anti NMDAR encephalitis and useful in monitoring disease progress

Abdullah S, Lim KS, Wong WF, Tan HJ, Tan CT

Neurology Asia, 2015;20(2):167-175.
MyJurnal

Background& Objective: Investigation modalities, such as MRI and CSF examination, are neither sensitive nor specific in the early phase of anti-NMDAR encephalitis. Nuclear imaging may be useful to monitor the response to treatment but limited by the availability.We aimed to determine the role of EEG as a tool for early diagnosis as well as a tool to assess disease progression and response to treatment. Methods: A total of 99 EEGsdone in 16 patients diagnosed with anti-NMDAR encephalitis throughout the course of illness, were reviewed retrospectively. The EEG changes were correlated with the clinical presentations and response to treatment. Sixteen EEGs of patients with schizophrenia and mood disorder, and 10 EEGs of patients with infective encephalitis were included as control. Results: EEGs performed during the psychiatric and cognitive dysfunctionphase in patient with anti-NMDAR encephalitis, showed diffuse background slowing in the delta-theta range in all the patients. Serial EEGs showed that the dominant background frequency improved with improvement in cognitive status. Nine patients had complete recovery with normalisation of the EEG abnormalities. Eight patients had their typical clinical seizure recorded during EEG monitoring, but only 2 (25.0%) with EEG correlation. Ten patients had status epilepticus (62.5%), 5 had EEG recorded during their status epilepticus, of which only one with EEG correlation (20.0%). Eleven patients had asymmetric background (68.8%), but only 1 has correlation with focal changes in the MRI brain (9.1%). Even though the EEGs of patients with infective encephalitis also showed background slowing, their CSF analysis was supportive of an infective cause. EEGs of patients with established psychiatric disorder were within normal limits.
Conclusion: EEG abnormality has a good correlation with the degree of psychiatric and cognitive dysfunction in patient with anti-NMDAR encephalitis, and is useful in early diagnosis, monitoring the progress and the response to treatment. However, it has poor correlation with clinical seizures.

Matched MeSH terms: Anti-N-Methyl-D-Aspartate Receptor Encephalitis