Ophelia Syndrome: Hodgkin Lymphoma with Limbic Encephalitis.

Background: Limbic encephalitis, an immune-mediated encephalitis, results from inflammation in the medial temporal lobes. The paraneoplastic form is rare in pediatric population, and frequently precedes tumor diagnosis. Case characteristics: A 9-year-old boy receiving chemotherapy for Hodgkin lymphoma, developed headache, temporal lobe seizures, anxiety, hallucinations, short-term memory loss and autonomic disturbances. Magnetic resonance imaging of brain showed features suggestive of limbic encephalitis. Electro-encephalography showed diffuse slowing with no epileptiform discharges. Outcome: We diagnosed paraneoplastic form of limbic encephalitis. Treatment with steroids and intravenous immunoglobulin failed, and the child died 4 weeks after onset of symptoms. Message: Limbic encephalitis should be kept as differential diagnosis in a child with sub-acutely evolving neuropsychiatric symptoms.

Intellectual Disability in Indian Children: Experience with a Stratified Approach for Etiological Diagnosis.

Objective: To study the clinico-etiological profile of children with intellectual disability using an algorithmic approach. Design: Cross-sectional study. Setting: Tertiary care centre in Northern India. Participants: Consecutive children aged 3 months to 12 years, presenting with intellectual disability, confirmed by Developmental Assessment Scale for Indian Infants, Binet Kulshreshtha Test and Vineland Social Maturity Scale. Method: All children were assessed on an internally validated structured proforma. A targeted approach included thyroid function tests, Brainstem evoked response audiometry, electroencephalogram, neuroimaging and metabolic screen done as a pre-decided schema. Genetic tests included karyotyping, molecular studies for Fragile X, Multiplex Ligation Dependent Probe Amplification and Array Comparative Genomic Hybridisation. Results: Data of 101 children (median age 22 months) was analyzed. The etiological yield was 82.1% with genetic causes being the most common (61.4%) followed by perinatal acquired (20.4%), CNS malformations (12%), external prenatal (3.6%), and postnatal acquired (2.4%). Mild delay was seen in 11.7%, moderate in 21.7%, severe in 30.6% and profound in 35.6%. Conclusion: It is possible to ascertain the diagnosis in most of the cases of intellectual disability using a judicious and sequential battery of tests.