Neuropathology of a 4-month-old infant born to a woman with phenylketonuria

Developmental Medicine and Child Neurology
Richard KochFloyd H Gilles

Abstract

We evaluated the brain of a 4-month-old male infant whose mother had inadequately controlled maternal phenylketonuria (MPKU). At autopsy his brain was normally developed but underweight. We found ventriculomegaly, hypoplastic cerebral white matter, and delay of myelination in late myelinating tracts without white matter astrocytosis and without chronic lesions in any gray matter structure. We compared the development of the infant's white matter tracts with published data on infant myelination. Congenital heart disease complicated the case. Abnormalities in developmental white matter may account for neurological abnormalities in infants with MPKU.

References

Jan 1, 1978·Journal of Inherited Metabolic Disease·G E JoshuaK V Mathai
May 1, 1992·Journal of Neurology, Neurosurgery, and Psychiatry·P A McCombeM P Pender
Jan 1, 1990·Developmental Neuroscience·R BurriN Herschkowitz
May 1, 1988·Journal of Neuropathology and Experimental Neurology·H C KinneyF H Gilles
May 1, 1987·Journal of Neuropathology and Experimental Neurology·B A BrodyF H Gilles
Dec 15, 1966·The New England Journal of Medicine·C C MabryJ G Coldwell
Nov 24, 1983·The New England Journal of Medicine·H L Levy, S E Waisbren
Aug 1, 1995·The Journal of Pediatrics·M A ClearyJ P Jenkins
Dec 1, 1994·Acta Paediatrica. Supplement·K UllrichH G Koch
Oct 1, 1994·Neuropediatrics·K UllrichH Hahn-Ullrich
Jul 9, 1994·Lancet·M A ClearyD Whittle
Aug 1, 1993·Brain : a Journal of Neurology·A J ThompsonD P Brenton
Aug 1, 1993·Genetics·A ShedlovskyW F Dove
Jun 1, 1996·The Journal of Pediatrics·H L LevyT Y Poussaint
Oct 24, 2000·European Journal of Pediatrics·P R Huttenlocher
Aug 24, 1963·British Medical Journal·J D ALLANJ T IRELAND
Dec 26, 1963·The New England Journal of Medicine·C C MABRYC D SON
Jan 25, 2005·Archives of Disease in Childhood·P J LeeF Cockburn

❮ Previous
Next ❯

Citations

Aug 5, 2009·Neuroradiology·Renzo ManaraAlberto B Burlina
Mar 7, 2020·Journal of Neuroscience Research·Bruna Klippel FerreiraPatricia Fernanda Schuck

❮ Previous
Next ❯

Related Concepts

Related Feeds

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.

Brain developing: Influences & Outcomes

This feed focuses on influences that affect the developing brain including genetics, fetal development, prenatal care, and gene-environment interactions. Here is the latest research in this field.