Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong

Ho, Alvin Chi Chung; Chan, Sophelia Hoi-shan; Chan, Eric; Wong, Samuel Po‐Shing; Fung, Sharon; Cherk, Sharon W.W.; Fung, Eva; Ma, Kam-hung; Tsui, Kwing-wan; Yau, Eric; Wong, Virginia

doi:10.1016/j.braindev.2018.02.005

Cited by 36 publications

(30 citation statements)

References 25 publications

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“…And symptoms presentation varied between children and adult. In our pediatric study, the proportion of seizures (39.2%, 42/107) among initial symptoms was similar to others' studies, whereas the movement disorder (29.9%, 32/107) was more common than other studies and psychiatric symptoms (18.7%, 20/107) was less than other studies [17,3,10,20,16,14,15] (See Additional le 1: Table S1). The distribution of symptoms during whole course was similar to other studies [17,3,10,20,16,14,15].…”

Section: Discussionsupporting

confidence: 88%

“…According to previous studies, pediatric anti-NMDAR encephalitis in China and other countries was more common in children aged around 8-12 years [3,[12][13][14][15][16]. While in our study, the mean age at onset was 6.3 ± 3.1 years old which was slightly younger (See Additional le 1: Table S1).…”

Section: Discussionsupporting

confidence: 40%

“…The initial symptoms were various in the different age. Previous studies found that in adult and children older than 12 years old, psychiatric symptoms were the most common initial presentation [17,10,15,14].…”

Section: Discussionmentioning

confidence: 97%

“…While in studies from western country female was moderately predominate (higher than 70%) [10]. And the gender ratio was also various in different age, with the age increase, proportion of female patient increase [14,20,15]. One reason for the predominance of female is teratomas which are more common in female more than 14 years old [10].…”

Section: Discussionmentioning

confidence: 98%

“…However, in children younger than 12 years old, the proportion of psychiatric symptoms at initial symptoms was nearly equal to that of seizures, but more common than that of movement disorder [10,15,21]. It remained unclear why there existed age differences on the initial symptoms of anti-NMDAR encephalitis.…”

Section: Discussionmentioning

confidence: 99%

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Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

Hou

et al. 2020

Preprint

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Background: Research on pediatric anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is limited. We studied clinical features of children with anti-NMDAR encephalitis in southern China.Methods: Pediatric anti-NMDAR encephalitis between 2014 and 2019 from one tertiary medical center was analyzed. The Modified Rankin Scale (mRS) score was adopted to evaluate outcomes.Results: 107 children (M/F=50/57; mean onset age=6.3 y) with anti-NMDAR encephalitis were involved. Boys significantly showed earlier onset age, higher ratio of fever, longer hospital day, more courses of steroid treatment, higher mRS score after treatment. 48.6% of patients had prodromal events with infectious events most common. The most common symptom at onset and during whole course was seizures and psychiatric symptoms respectively. Initial median white cell count of cerebral spinal fluid (CSF) was 22.0x106/L. 12.1% of patients had positive herpes simplex virus DNA of CSF. All patients had CSF positive NMDAR antibodies, although 86.9% of patients had serologically positive. 9.3% of patients had overlapped other neuronal antibodies. Electroencephalograph showed abnormalities with slow wave (95.3%) and epileptic activity (41.5%). Delta brush was revealed in 3.8% of patients. 36.4% of patients had lesions in brain MRI, with local lesions most common. No tumor was found in all. 84.9% of patients responded well to the first line therapy (steroid plus immunoglobulin), while 12 patients accepted second-line therapy (Rituximab) with 91.7% of effective rate. 12.1% of patients relapsed. 2 male patients died. The median length of hospital stay was 28 days. The mRS score was significantly improved after treatment. 51.0% of patients had a full recovery. 76.6% of patients had mild neurological disability (mRS≤2). Male, speech disorder, initial score of mRS and administration of second-line therapy were independent risk factors associated with the outcome.Conclusions: Of pediatric anti-NMDAR encephalitis in southern China: the mean onset age was about 6-year-old, earlier in boys; boys remained slower to recover and with a worse prognosis; the most common respective symptom at onset and during whole course was seizures and psychiatric symptoms; combination with tumor was rare; most patients respond well to the immunotherapy; relapse and fatality rate were relatively low; most patients had a good prognosis.

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