In this study, we assessed cognitive function and neurophysiological develo
pment in congenitally hypothyroid (CH) children. We performed a cross-secti
onal study at the outpatient Pediatric Clinic and Department of Neurophysio
logy at San Raffaele Hospital, Milan. The study enrolled 25 CH patients (6.
00-10.83 years of age) detected by neonatal screening, and 34 healthy contr
ol children (4-11 years of age). Patients and controls had comparable score
s at neuropsychological tests (WISC-R), and at auditory P300 tests. In cont
rast, we found significantly longer LLSEP latencies in CH patients (p < 0.0
3). CH patients treated 30 days after birth showed lower scores at neuropsy
chological tests, but not at neurophysiological tests, compared to patients
who started the replacement therapy earlier. Patients with more severe fet
al hypothyroidism (T4 levels at diagnosis <less than or equal to> 2 mug/dl)
had lower neuropsychological scores, and similar neurophysiological result
s, compared with patients with moderate fetal hypothyroidism. The severity
of fetal hypothyroidism and early treatment influence the mental outcome of
CH patients. Neurophysiological results show that central nervous system d
amage occurs in some patients despite early treatment.