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Published on: November 3, 2016
Cerebellar injury in the extremely premature infant: newly recognized but relatively common outcome
John B Bodensteiner1, Stanley D Johnsen
1Pediatric Neurology Division, St. Joseph's Children's Health Center and the Barrow Neurological Institute, Phoenix, AZ 85013, USA. jbodens@chw.edu
Insights
Extreme prematurity can cause severe cerebellar injury, leading to a distinct form of cerebral palsy in extremely low birthweight infants. This condition presents with significant motor, cognitive, and language delays.
Area of Science:
- Neuroscience
- Pediatrics
- Developmental Biology
Background:
- Cerebral palsy (CP) is a common neurodevelopmental disorder.
- Extreme prematurity and extremely low birthweight (ELBW) are known risk factors for various neonatal complications.
- Recent literature described cerebellar injury as a complication of ELBW, presenting as a distinct clinical diagnosis of CP.
Purpose of the Study:
- To report additional cases of cerebellar injury in ELBW infants.
- To further characterize the clinical and neuroimaging features of this syndrome.
- To emphasize the significance of this condition as a potential outcome of ELBW.
Main Methods:
- Case series reporting on 10 additional infants.
- Clinical assessment of motor impairment, ataxia, athetosis, dystonia, and developmental delays.
- Neuroimaging studies (MRI/CT) to evaluate cerebellar and cerebral structures.
Main Results:
- The 10 reported cases exhibited striking motor impairment, ataxia, and athetosis/dystonia, consistent with a distinct CP subtype.
- All infants were microcephalic, with significant cognitive, language, and motor delays.
- Neuroimaging revealed absence of major cerebellar portions (inferior vermis, hemispheres) and often associated cerebral injury.
Conclusions:
- Cerebellar injury is a significant and not uncommon complication in ELBW infants, leading to a specific type of cerebral palsy.
- The syndrome is characterized by severe motor deficits, microcephaly, and developmental delays.
- Further research is needed to determine the frequency and etiological factors of this condition.
Abstract:
Severe injury to the cerebellum as a complication of extreme prematurity with extremely low birthweight was recently described in 13 children with the clinical diagnosis of cerebral palsy. We report another 10 cases of this syndrome. The clinical features include striking motor impairment and variable degrees of ataxia and athetosis or dystonia, which represent a distinct clinical type of cerebral palsy. Most are severely damaged, with cognitive, language, and motor delays. All are microcephalic, except one with hydrocephalus. Neuroimaging studies demonstrate the absence of major portions of the cerebellum involving both the inferior vermis and hemispheres. Most also have injury of a less severe nature in the cerebrum. This report indicates that this is not an uncommon outcome of extremely low birthweight infants, and we hope to encourage further investigations into the relative frequency and likely etiologies of the condition.
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