Cognitive delay in children with tuberous sclerosis in a developing country: Clinical correlations

Abstract

Cognitive delayisa frequent neurobehavioral manifestation inchildrenwith tuberoussclerosiscomplex (TSC). This study aimed todeterminethe relationshipsbetweencognitive delaywithrespecttoseizurecharacteristics,interictal electroencephalography (EEG)changes, behavioralabnormalities andbrain tubers'count and location. Thisstudy included24childrenwithTSC witha mean age of 6.2 ± 2.55 yr. Children underwent clinical, psychometric, interictal EEG and magnetic resonance imaging or magnetic reso- nance imaging of the brain. Nearly 58.33% of children had cognitive delay and 45.83% had autism. Compared to children without cognitive delay, children with cognitive delay were younger (P < 0.01), had higher frequencies of seizures (particularly infantile spasms with onset predominantly below 6 mo of age) (P < 0.01), severe epileptogenic EEG changes, high tuber burden (particularly in the left hemisphere with frontal and temporal predominance) (P < 0.01) and frequent behavioral abnormalities as autism (P < 0.001) and attention deficit hyperactivity disorders (P < 0.05). Cognitive scores were correlated with age of seizures' onset (r = 0.432, P = 0.01), presence of infantile spasms (r = −0.675, P = 0.001), autism (r = −0.350, P = 0.05), and number of brain tubers (r = −0.865, P = 0.0001) but non correlated to age nor gender. In multivariate analysis, only the age of seizure onset (odds ratio 1.90; 95% confidence interval 1.02 to 3.55, P = 0.044) and the number of brain tubers (odds ratio 2.36; 95% confidence interval 0.95 to 5.81; P= 0.06) increased the oddsforcognitive delay among TSC patients. The knowledgeofthe clinical features of TSC can help in early diagnosis and management of patients with a multidisciplinary consultation.