title: Anti-Ma2-encephalitis in a 2 year-old child: A newly diagnosed case and literature review.
abstract: BACKGROUND
  Anti-Ma2-associated encephalitis is a rare paraneoplastic neurological syndrome characterized by isolated or combined limbic, diencephalic, or brainstem dysfunction. It is rarely reported in children.
  
  
  METHODS
  We describe the clinical data of a 2-year-old girl referred to our department for refractory focal seizures associated with fever, followed by behavioural changes, speech disturbances and confusional episodes. Brain magnetic resonance imaging (MRI) showed left temporoparietal brain involvement. Haematological, biochemical and infectious evaluations were unremarkable. Autoimmune encephalitis was suspected. Paraneoplastic antibodies tests showed positive results for anti-Ma2 antibodies. Screening for underlying tumour was negative. Immunomodulatory treatment was administrated. The patient showed improvement of vigilance and behaviour. However, she kept refractory epilepsy.
  
  
  CONCLUSIONS
  Although poor response to immunotherapy, early diagnosis and appropriate treatment of this disorder may prevent irreversible sequelae.
authors.affiliation: Research Unit UR12 SP24 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia.
authors.forename: Saloua
authors.initials: S
authors.lastname: Mrabet
authors.affiliation: Research Unit UR12 SP24 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia. Electronic address: nediaachour@yahoo.fr.
authors.forename: Nedia
authors.initials: N
authors.lastname: Ben Achour
authors.affiliation: Research Unit UR12 SP24 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia.
authors.forename: Ichraf
authors.initials: I
authors.lastname: Kraoua
authors.affiliation: Research Unit UR12 SP24 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia.
authors.forename: Hanène
authors.initials: H
authors.lastname: Benrhouma
authors.affiliation: Research Unit UR12 SP24 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia.
authors.forename: Hedia
authors.initials: H
authors.lastname: Klaa
authors.affiliation: Research Unit UR12 SP24 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia.
authors.forename: Aida
authors.initials: A
authors.lastname: Rouissi
authors.affiliation: Department of Clinical Immunology, Pasteur Institute, Tunis, Tunisia.
authors.forename: Malika
authors.initials: M
authors.lastname: Ben Ahmed
authors.affiliation: Research Unit UR12 SP24 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia.
authors.forename: Ilhem
authors.initials: I
authors.lastname: Ben Youssef Turki
mesh_terms: D001519:Behavior; D003221:Confusion / Q000209:etiology / Q000523:psychology; D003241:Consanguinity; D000069279:Drug Resistant Epilepsy / Q000209:etiology; D004660:Encephalitis / Q000473:pathology* / Q000523:psychology; D005260:Female; D005334:Fever / Q000209:etiology; D050031:Hashimoto Disease / Q000473:pathology* / Q000523:psychology; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D010296:Parietal Lobe / Q000473:pathology; D012640:Seizures / Q000209:etiology; D013064:Speech Disorders / Q000209:etiology; D013702:Temporal Lobe / Q000473:pathology
keywords: Anti Ma2 antibodies; Childhood; Limbic encephalitis; Refractory epilepsy