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Published on: July 4, 2007
Autoimmune Encephalitis in Children: An Update
Divyani Garg1, Shekeeb S Mohammad2, Suvasini Sharma3
1Department of Neurology, Lady Hardinge Medical College, New Delhi, India.
Insights
Autoimmune encephalitis is a treatable cause of neurological disorders in children. Early diagnosis and immunomodulatory treatment improve outcomes for pediatric autoimmune encephalitis, particularly anti-NMDAR encephalitis.
Area of Science:
- Pediatric Neurology
- Immunology
- Neuroscience
Background:
- Autoimmune encephalitis is a significant, treatable cause of encephalopathy, epilepsy, and movement disorders in children.
- Increasing recognition of autoimmune encephalitis in pediatric populations.
- Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most prevalent form.
Purpose of the Study:
- To review clinical syndromes, diagnosis, treatment, and prognosis of autoimmune encephalitis in children.
- To provide an overview of current understanding and management strategies for pediatric autoimmune encephalitis.
Main Methods:
- MEDLINE search from 1998-2019 using terms related to autoimmune encephalitis in children.
- Inclusion of various study types: reviews, guidelines, RCTs, cohort studies, case series, and reports.
- Application of 'Human' and 'English' limits.
Main Results:
- Pediatric autoimmune encephalitis presents with diverse symptoms including behavioral changes, psychosis, seizures, and neurocognitive deficits.
- Diagnosis relies on clinical history, MRI, CSF analysis, and autoantibody serology.
- Early immunomodulatory treatment and maintenance therapy are crucial for better outcomes.
Conclusions:
- Autoimmune encephalitis is an increasingly diagnosed condition in children, with anti-NMDAR encephalitis being the most common.
- Prompt diagnosis and timely immunomodulatory treatment are associated with improved prognosis.
- Prognosis is variable and depends on the specific 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.
