TY - JOUR
T1 - Anti-N-Methyl-d-aspartate-receptor encephalitis
T2 - Cognitive profile in two children
AU - Iadisernia, Enzo
AU - Battaglia, Francesca Maria
AU - Vanadia, Elena
AU - Trapolino, Emanuele
AU - Vincent, Angela
AU - Biancheri, Roberta
PY - 2012/1
Y1 - 2012/1
N2 - Background: Anti-N-Methyl d-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder associated with antibodies against NMDAR resulting in a characteristic neuropsychiatric syndrome characterized by seizures, dyskinesias, and cognitive impairment. The extent and specific tasks associated with cognitive dysfunction in anti-NMDAR encephalitis have not been fully investigated. Aims: To describe cognitive and neuropsychological profile in two children with anti-NMDAR encephalitis. Methods: Clinical, laboratory, cognitive and neuropsychological assessments have been performed. Cognitive functions have been evaluated one year after the disease onset, at age 4 years and 10 months in one patient and at age 5 years and 5 months in the other subject. The first patient has been re-assessed one year after the first evaluation. Results: Both children, who were reported to be normal before disease onset, showed a severe neurological impairment during the acute phase of disease with progressive substantial recovery following treatment. Selective and prolonged attention, activation and integration of semantic information and verbal fluency were particularly impaired. Significant improvements were observed at neuropsychological re-assessment. Conclusions: Executive dysfunction seems to be the "core" of the neuropsychological profile of children with anti-NMDAR encephalitis. Cognitive abilities may be, at least to some extent, recovered providing that immunomodulatory treatment and specific psychomotor and pedagogical therapy are started soon after disease onset.
AB - Background: Anti-N-Methyl d-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder associated with antibodies against NMDAR resulting in a characteristic neuropsychiatric syndrome characterized by seizures, dyskinesias, and cognitive impairment. The extent and specific tasks associated with cognitive dysfunction in anti-NMDAR encephalitis have not been fully investigated. Aims: To describe cognitive and neuropsychological profile in two children with anti-NMDAR encephalitis. Methods: Clinical, laboratory, cognitive and neuropsychological assessments have been performed. Cognitive functions have been evaluated one year after the disease onset, at age 4 years and 10 months in one patient and at age 5 years and 5 months in the other subject. The first patient has been re-assessed one year after the first evaluation. Results: Both children, who were reported to be normal before disease onset, showed a severe neurological impairment during the acute phase of disease with progressive substantial recovery following treatment. Selective and prolonged attention, activation and integration of semantic information and verbal fluency were particularly impaired. Significant improvements were observed at neuropsychological re-assessment. Conclusions: Executive dysfunction seems to be the "core" of the neuropsychological profile of children with anti-NMDAR encephalitis. Cognitive abilities may be, at least to some extent, recovered providing that immunomodulatory treatment and specific psychomotor and pedagogical therapy are started soon after disease onset.
KW - Cognitive
KW - Encephalitis
KW - Neuropsychology
KW - NMDA receptor
KW - Pediatric disease
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U2 - 10.1016/j.ejpn.2011.09.004
DO - 10.1016/j.ejpn.2011.09.004
M3 - Article
C2 - 21982543
AN - SCOPUS:84655163225
VL - 16
SP - 79
EP - 82
JO - European Journal of Paediatric Neurology
JF - European Journal of Paediatric Neurology
SN - 1090-3798
IS - 1
ER -