Abstract
The longitudinal development of head growth was investigated in girls with Rett syndrome
(RS). Growth retardation was expressed in standard deviation (SD) scores. In classic
types, the mean head circumference fell successively to 2 SD scores below the norm
at the age of 4 years. After the age of 8 it stabilized close to −3 SD scores. In
forme fruste variants, the mean head circumference was within normal limits; however,
it was significantly below the norm, −0.8 SD scores. In girls with classic RS, head
growth had decelerated by less than 1 SD score in 20% of the girls at the age of 6
years and in 10% at the age of 12 years. In forme fruste variants only a small decline
in head growth occurred. Head growth decline may thus be very small in classic RS
and is usually not present at all in forme fruste variants. In the future, it should,
therefore, neither be regarded as a necessary diagnostic criterion for classic RS,
nor as a valid one for forme fruste variants.
Keywords
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References
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Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.