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.
Keywords
Tuberous sclerosis - brain tubers - cognitive delay - infantile spasms - autism -
attention deficit hyperactivity disorder
