Divyani Garg1, Shekeeb S Mohammad2, Suvasini Sharma3. 1. Department of Neurology, Lady Hardinge Medical College, New Delhi, India. 2. Movement Disorder Clinic, Department of Neurology and USyd Clinical School, The Children's Hospital at Westmead, Sydney, Australia. 3. Neurology Division, Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India. Correspondence to: Dr Suvasini Sharma, Associate Professor, Department of Pediatrics, Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, Bangla Sahib Road, New Delhi 110 001, India. sharma.suvasini@gmail.com.
Abstract
CONTEXT: Autoimmune encephalitis has acquired immense significance as a treatable cause of encephalopathy, epilepsy and movement disorders in children. In this review, we discuss the various clinical syndromes, diagnosis, treatment and prognosis in children. EVIDENCE ACQUISITION: A MEDLINE search strategy using the following terms (1998-2019) was adopted for this review. Limits of 'Human' and 'English' were applied. Search terms included: "autoimmune encephalitis", "autoimmune encephalitis AND epidemiology", "pathophysiology", "diagnosis" and "treatment" for studies in children. Review articles, practice parameters, guidelines, systematic reviews, meta-analyses, randomized controlled trials, cohort studies, case series and case reports were included. CONCLUSIONS: Autoimmune encephalitis is being increasingly recognized in children. Anti-NMDAR encephalitis is the most common form. Children present with a polysymptomatic presentation including behavioral changes, psychosis, sleep disturbances, mutism, seizures, movement disorders, memory impairment as well as other neurocognitive deficits. Diagnosis is based on suggestive history and ancillary investigations including magnetic resonance imaging, cerebrospinal fluid analysis, and serology for autoantibodies. Treatment is based on immunomodulation of the acute episode followed by maintenance therapy, with earlier initiation being associated with better outcomes. Prognosis depends on the type of clinical syndrome.
CONTEXT: Autoimmune encephalitis has acquired immense significance as a treatable cause of encephalopathy, epilepsy and movement disorders in children. In this review, we discuss the various clinical syndromes, diagnosis, treatment and prognosis in children. EVIDENCE ACQUISITION: A MEDLINE search strategy using the following terms (1998-2019) was adopted for this review. Limits of 'Human' and 'English' were applied. Search terms included: "autoimmune encephalitis", "autoimmune encephalitis AND epidemiology", "pathophysiology", "diagnosis" and "treatment" for studies in children. Review articles, practice parameters, guidelines, systematic reviews, meta-analyses, randomized controlled trials, cohort studies, case series and case reports were included. CONCLUSIONS:Autoimmune encephalitis is being increasingly recognized in children. Anti-NMDAR encephalitis is the most common form. Children present with a polysymptomatic presentation including behavioral changes, psychosis, sleep disturbances, mutism, seizures, movement disorders, memory impairment as well as other neurocognitive deficits. Diagnosis is based on suggestive history and ancillary investigations including magnetic resonance imaging, cerebrospinal fluid analysis, and serology for autoantibodies. Treatment is based on immunomodulation of the acute episode followed by maintenance therapy, with earlier initiation being associated with better outcomes. Prognosis depends on the type of clinical syndrome.