Abstract
Recent studies show that the insulin-like growth factor II (IGFII) in contrast to IGF I appears ineffective in stimulating longitudinal growth. Both factors are present in human serum, whereas in CSF only IGF II can be detected. This hormone has been suggested to be produced in significant amounts in brain.-We report a patient with extreme megalencephaly with markedly elevated concentrations of IGF II in CSF and cerebral cortex. Head circumference at birth (gestational age 34 weeks)was 40.5cm (+4.8SD); it increased to 51.5cm (+8.9SD) at the age of 4 1/2 months when the boy died. He had muscular hypotonia and severe epilepsy. Body length (including head) was on the 97th percentile. The cause of megalencephaly could not be identified. IGF I and II in serum were in the normal range for age. IGF II in CSF was 24 ng/ml compared with 10 ng/ml in a pool of CSF from controls of the same age. At autopsy brain weight was 1450 g (normal range 567±81g). IGF II extracted from frontal cortex was 50ng/g compared to 3-11 ng/g in four controls.
We speculate that the megalencephaly of this patient has been caused by an excess of IGF II in brain. This data supports the assumption that IGF II is a major factor in CNS growth.
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Schoenle, E., Haselbacher, G., Humbel, R. et al. Elevated IGF II in cerebrospinal fluid and brain in a newborn with extreme megalencephaly. Pediatr Res 18, 1211 (1984). https://doi.org/10.1203/00006450-198411000-00063
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DOI: https://doi.org/10.1203/00006450-198411000-00063
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