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J Child Neurol. 2014 Nov;29(11):1453-9. doi: 10.1177/0883073813494474. Epub 2013 Oct 4.

Pediatric anti-N-methyl-D-aspartate (NMDA) receptor encephalitis: experience of a tertiary care teaching center from north India.

Author information

1
All India Institute of Medical Sciences, New Delhi, India.
2
All India Institute of Medical Sciences, New Delhi, India sheffaligulati@gmail.com.

Abstract

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is characterized by acute- or subacute-onset encephalopathy with extrapyramidal, psychiatric, and epileptic manifestations. Diagnosis is confirmed by positive antibodies to NMDA receptor in cerebrospinal fluid and serum. Eleven pediatric cases presented over a 2-year period at a tertiary care teaching hospital in North India. The average age at presentation was 9 years (range: 2.5 to 18 years, median: 10 years) with a slight female predominance (1.2:1). The common modes of presentation were progressive extrapyramidal syndrome with global neuroregression in 45% (5 of 11), epileptiform encephalopathy in 27% (3 of 11), and an overlap between the 2 in 27% (3 of 11). Fifty-eight percent showed significant response to steroids and intravenous immunoglobulin. This entity should be considered in an acute- or subacute-onset encephalopathy if common infectious etiologies are ruled out and there are specific clinical pointers. Early diagnosis and treatment significantly improves the outcome.

KEYWORDS:

NMDA receptor; encephalitis; immunotherapy

PMID:
24097850
DOI:
10.1177/0883073813494474
[Indexed for MEDLINE]

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