Article

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents

Division of Neurology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Annals of Neurology (Impact Factor: 11.91). 07/2009; 66(1):11-8. DOI: 10.1002/ana.21756
Source: PubMed

ABSTRACT To report the clinical features of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in patients < or = 18 years old.
Information was obtained by the authors or referring physicians. Antibodies were determined by immunocytochemistry and enzyme-linked immunosorbent assay (ELISA) using HEK293 cells ectopically expressing NR1.
Over an 8-month period, 81 patients (12 male) with anti-NMDAR encephalitis were identified. Thirty-two (40%) were < or =18 years old (youngest 23 months, median 14 years); 6 were male. The frequency of ovarian teratomas was 56% in women >18 years old, 31% in girls < or =18 years old (p = 0.05), and 9% in girls < or =14 years old (p = 0.008). None of the male patients had tumors. Of 32 patients < or =18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction. On admission, 53% had severe speech deficits. Eventually, 77% developed seizures, 84% stereotyped movements, 86% autonomic instability, and 23% hypoventilation. Responses to immunotherapy were slow and variable. Overall, 74% had full or substantial recovery after immunotherapy or tumor removal. Neurological relapses occurred in 25%. At the last follow-up, full recovery occurred more frequently in patients who had a teratoma that was removed (5/8) than in those without a teratoma (4/23; p = 0.03).
Anti-NMDAR encephalitis is increasingly recognized in children, comprising 40% of all cases. Younger patients are less likely to have tumors. Behavioral and speech problems, seizures, and abnormal movements are common early symptoms. The phenotype resembles that of the adults, although dysautonomia and hypoventilation are less frequent or severe in children. Ann Neurol 2009;66:11-18.

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    • "L'évolution peut être sévère et conduire à une prise en charge en réanimation. Elles peuvent être associées à un tératome ovarien, mais avant l'âge de 12 ans les formes paranéoplasiques sont rares [3] [4] [5] [6]. "
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    ABSTRACT: Dysimmune encephalitis are frequent and disabling pathologies with efficacious treatments (immunotherapy). Evocative clinical signs are: rapid progressive evolution, seizure, movement disorder, psychiatric troubles, memory defect, dysautonomia and hyponatremia. Classical ancillary test: magnetic resonance imaging (MRI) and standard cerebro-spinal fluid (CSF) analysis can be normal. In this context, the cerebral positron emission tomography (PET) is helpful for the diagnosis. More often reported patterns are: association of hyper- and hypo-metabolism; fronto-temporal hyper-metabolism/parieto-occipital hypo-metabolism gradient (anti-N-methyl-D-aspartate receptor [NMDAR] encephalitis); temporal and basal ganglia hyper-metabolism (anti-leucine-rich glioma inactivated protein 1 [LGI1] encephalitis); temporal hyper-metabolism (other limbic encephalitis); parieto-occipital ± basal ganglia hypo-metabolism (neurolupus); unihemispheric diffuse hypo-metabolism (Rasmussen encephalitis). The diagnosis could require research of specific antibodies. Finally, the cerebral PET is also useful during the follow-up: treatment's efficacy (improvement of metabolism), relapse (aggravation of metabolism's alteration), and evaluation of possible sequelae.
    Medecine Nucleaire 04/2015; DOI:10.1016/j.mednuc.2015.03.002 · 0.16 Impact Factor
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    • "Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an increasingly recognized etiology of previously unexplained encephalopathy and encephalitis, since its original description in 2007 [1] [2]. The disease has initially been described in women with an ovarian teratoma but can also be seen in seen in women without an ovarian teratoma, men [3]. The syndrome usually develops with a sequential presentation of symptoms including headache and fever followed by behavioral changes, psychosis, catatonia, decreased level of consciousness, dyskinesias , and autonomic instability [4]. "
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    ABSTRACT: Since its original description in 2007, anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis associated with an ovarian teratoma is an increasingly recognized etiology of previously unexplained encephalopathy and encephalitis. Extreme delta brush (EDB) is a novel electroencephalogram (EEG) finding seen in many patients with anti-NMDAR encephalitis. The presence of this pattern is associated with a more prolonged illness, although the specificity of this pattern is unclear. Additionally, the frequency and sensitivity of EDB in anti-NMDAR encephalitis and its implications for outcome have yet to be determined. We report a patient with early evidence of extreme delta brush and persistence of this pattern 17.5 weeks later with little clinical improvement.
    12/2014; 2(1). DOI:10.1016/j.ebcr.2014.01.002
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    • "Whereas the return of antibody testing results from clinical laboratories may take weeks, EEG abnormalities that might be specific to the illness, detected at the time of presentation, can possibly improve the speed of discovery of associated teratomas and reduce delays in diagnosis and treatment. Most reports in the past have described non-specific EEG abnormalities (Florance et al., 2009; Dalmau et al., 2011). A number of recent studies, however , have attempted to systematically characterise the pattern of EEG abnormalities in both adults and children during wakefulness and sleep. "
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    ABSTRACT: Variations in clinical presentation can lead to delays in the diagnosis and initiation of treatment of anti-N-methyl-D-aspartate receptor encephalitis. Most patients have an EEG study performed early in the course of their illness. Although not specific, there may be clues in the electroclinical features that should alert clinicians and electroencephalographers to the possibility of this diagnosis. This case is a reminder that anti- anti-N-methyl-D-aspartate receptor encephalitis may present initially with a movement disorder as the sole symptom, without features of an encephalopathy. In addition, it adds to the growing body of evidence that recognition of certain electroclinical clues may shorten the time to diagnosis. [Published with video sequence].
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