Prenatal Brainstem Disruptions: Small Lesions–Big Problems

Eugen Boltshauser; Florian Bauder; Miriam Giarrana; Anette Hackenberg; Sébastien Lebon; Eliane Roulet-Perez; Regula Schmid; Thomas Schmitt-Mechelke; Andrea Poretti

doi:10.1055/s-0037-1603516

Neuropediatrics, Inhaltsverzeichnis

Neuropediatrics 2017; 48(05): 350-355
DOI: 10.1055/s-0037-1603516

Original Article

Georg Thieme Verlag KG Stuttgart · New York

Prenatal Brainstem Disruptions: Small Lesions–Big Problems^[*]

Eugen Boltshauser

¹Department of Pediatric Neurology, University Children's Hospital, Zürich, Switzerland

,

Florian Bauder

²Division of Pediatric Neurology, Children's Hospital, Lucerne, Switzerland

,

Miriam Giarrana

¹Department of Pediatric Neurology, University Children's Hospital, Zürich, Switzerland

,

Anette Hackenberg

¹Department of Pediatric Neurology, University Children's Hospital, Zürich, Switzerland

,

Sébastien Lebon

³Pediatric Neurology and Neurorehabilitation Unit, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland

,

Eliane Roulet-Perez

³Pediatric Neurology and Neurorehabilitation Unit, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland

,

Regula Schmid

⁴Division of Pediatric Neurology, Children's Hospital, Winterthur, Switzerland

,

Thomas Schmitt-Mechelke

²Division of Pediatric Neurology, Children's Hospital, Lucerne, Switzerland

,

Andrea Poretti

⁵Division of Pediatric Radiology and Pediatric Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States

⁶Department of Neurogenetics, Kennedy Krieger Institute, Baltimore, Maryland, United States

› Institutsangaben

Abstract

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.

Keywords

brainstem - disruption - prenatal - neuroimaging - children

Volltext

Referenzen

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Prenatal Brainstem Disruptions: Small Lesions–Big Problems[*]

Prenatal Brainstem Disruptions: Small Lesions–Big Problems^[*]