TY - JOUR
T1 - Prenatal Brainstem Disruptions
T2 - Small Lesions–Big Problems *
AU - Boltshauser, Eugen
AU - Bauder, Florian
AU - Giarrana, Miriam
AU - Hackenberg, Anette
AU - Lebon, Sébastien
AU - Roulet-Perez, Eliane
AU - Schmid, Regula
AU - Schmitt-Mechelke, Thomas
AU - Poretti, Andrea
PY - 2017/4/12
Y1 - 2017/4/12
N2 - Prenatal disruptive injuries within one or both cerebellar hemispheres, which are mostly caused by hemorrhages, are well known. Primary disruptive lesions of the brainstem, however, are exceptional. Here we report on clinical and neuroimaging findings, as well as outcome of four male infants with prenatal brainstem disruptions that have been seen between 2005 and 2015. Two infants with extensive brainstem defects (from the midbrain to the medulla) had respiratory insufficiency and died at the age of 12 weeks and 22 months, respectively. Two patients had smaller, unilateral/asymmetrical lesions in the pontomesencephalic and medullary regions, respectively, and presented with permanent multiple cranial nerve deficits and long-tract signs. Recognition of prenatal brainstem disruptions and distinction from malformations are essential for the management and the estimation of a recurrence risk.
AB - Prenatal disruptive injuries within one or both cerebellar hemispheres, which are mostly caused by hemorrhages, are well known. Primary disruptive lesions of the brainstem, however, are exceptional. Here we report on clinical and neuroimaging findings, as well as outcome of four male infants with prenatal brainstem disruptions that have been seen between 2005 and 2015. Two infants with extensive brainstem defects (from the midbrain to the medulla) had respiratory insufficiency and died at the age of 12 weeks and 22 months, respectively. Two patients had smaller, unilateral/asymmetrical lesions in the pontomesencephalic and medullary regions, respectively, and presented with permanent multiple cranial nerve deficits and long-tract signs. Recognition of prenatal brainstem disruptions and distinction from malformations are essential for the management and the estimation of a recurrence risk.
KW - brainstem
KW - children
KW - disruption
KW - neuroimaging
KW - prenatal
UR - http://www.scopus.com/inward/record.url?scp=85020286055&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85020286055&partnerID=8YFLogxK
U2 - 10.1055/s-0037-1603516
DO - 10.1055/s-0037-1603516
M3 - Article
C2 - 28571098
AN - SCOPUS:85020286055
SN - 0174-304X
JO - Neuropediatrics
JF - Neuropediatrics
ER -