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.