Affiliations: Department of Pediatrics, Assiut University Hospital, Assiut, Egypt | Department of Neurology and Psychiatry, Assiut University Hospital, Assiut, Egypt
Note:  Corresponding author: Sherifa A. Hamed, Consultant Neurologist, Department of Neurology and Psychiatry, Assiut University Hospital, Assiut, Egypt, P.O. Box 71516. Tel.: +20 2 088 2374904; Fax: +20 2 088 2333327; E-mail: [email protected]
Abstract: Cognitive delay is a frequent neurobehavioral manifestation in children with tuberous sclerosis complex (TSC). This study aimed to determine the relationships between cognitive delay with respect to seizure characteristics, interictal electroencephalography (EEG) changes, behavioral abnormalities and brain tubers’ count and location. This study included 24 children with TSC with a mean age of 6.2 ± 2.55 yr. Children underwent clinical, psychometric, interictal EEG and magnetic resonance imaging or magnetic resonance 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 odds for cognitive delay among TSC patients. The knowledge of the clinical features of TSC can help in early diagnosis and management of patients with a multidisciplinary consultation.