title: Cognitive and neuropsychological evolution in children with anti-NMDAR encephalitis.
abstract: We describe neurological and cognitive/neuropsychological changes from symptom onset in 13 consecutive children (8 females and 5 males; median age 11 years, range 3-17) with anti-NMDAR-encephalitis. We assessed neurological status using the modified Rankin Scale for children and cognitive/neuropsychological status using a standardized battery that was administered serially in 10 prospective patients, and at latest follow-up in three retrospective patients diagnosed before study initiation. Symptom onset was marked by neurological or psychiatric/behavioural manifestations, which became severe but regressed at variable rates after starting immunotherapy. The 10 prospective patients were able to undergo first standardized cognitive/neuropsychological assessment a median of 3 months (range 1-12) after symptom onset: they had extensive deficits, although severity varied. Subsequent assessment showed marked improvements although the timescale varied. At latest evaluation (median 31 months, range 3-112, after symptom onset), seven patients had no neurological disability, five had improved substantially, and one had persistent behavioural problems. Latest cognitive/neuropsychological assessment in 11 patients with at least a year of follow-up showed normal general intellectual abilities, but over half had residual deficits indicating frontal lobe dysfunction. All patients had resumed normal activities. Our findings suggest that early installation of immunotherapy results in good long-term recovery in most paediatric patients with anti-NMDAR-encephalitis, however, recovery is incomplete and the disease leaves subtle lasting defects that impact quality of life, social relationships, and academic achievement.
authors.affiliation: Department of Pediatric Neuroscience, Foundation IRCCS Neurological Institute "C. Besta", Via Celoria 11, 20133, Milan, Italy.|Department of Pediatrics, University of Chieti, Chieti, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Matricardi
authors.affiliation: Department of Pediatric Neuroscience, Foundation IRCCS Neurological Institute "C. Besta", Via Celoria 11, 20133, Milan, Italy.
authors.forename: Mara
authors.initials: M
authors.lastname: Patrini
authors.affiliation: Department of Pediatric Neuroscience, Foundation IRCCS Neurological Institute "C. Besta", Via Celoria 11, 20133, Milan, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Freri
authors.affiliation: Department of Pediatric Neuroscience, Foundation IRCCS Neurological Institute "C. Besta", Via Celoria 11, 20133, Milan, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Ragona
authors.affiliation: Department of Pediatric Neuroscience, Foundation IRCCS Neurological Institute "C. Besta", Via Celoria 11, 20133, Milan, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Zibordi
authors.affiliation: Neuromuscular Diseases and Neuroimmunology Unit, Foundation IRCCS Neurological Institute "C. Besta", Milan, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Andreetta
authors.affiliation: Department of Pediatric Neuroscience, Foundation IRCCS Neurological Institute "C. Besta", Via Celoria 11, 20133, Milan, Italy.
authors.forename: Nardo
authors.initials: N
authors.lastname: Nardocci
authors.affiliation: Department of Pediatric Neuroscience, Foundation IRCCS Neurological Institute "C. Besta", Via Celoria 11, 20133, Milan, Italy. granata@istituto-besta.it.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Granata
mesh_terms: D000293:Adolescent; D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000523:psychology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology*; D005260:Female; D006801:Humans; D007167:Immunotherapy / Q000379:methods; D008297:Male; D009483:Neuropsychological Tests
keywords: Anti-NMDAR encephalitis; Cognitive functioning; Long-term outcomes; Paediatric patients