Developmental Cerebellar Cognitive Affective Syndrome in Ex-preterm Survivors Following Cerebellar Injury

Abstract

Cerebellar injury is increasingly recognized as an important complication of very preterm birth. However, the neurodevelopmental consequences of early life cerebellar injury in prematurely born infants have not been well elucidated. We performed a literature search of studies published between 1997 and 2014 describing neurodevelopmental outcomes of preterm infants following direct cerebellar injury or indirect cerebellar injury/underdevelopment. Available data suggests that both direct and indirect mechanisms of cerebellar injury appear to stunt cerebellar growth and adversely affect neurodevelopment. This review also provides important insights into the highly integrated cerebral-cerebellar structural and functional correlates. Finally, this review highlights that early life impairment of cerebellar growth extends far beyond motor impairments and plays a critical, previously underrecognized role in the long-term cognitive, behavioral, and social deficits associated with brain injury among premature infants. These data point to a developmental form of the cerebellar cognitive affective syndrome previously described in adults. Longitudinal prospective studies using serial advanced magnetic resonance imaging techniques are needed to better delineate the full extent of the role of prematurity-related cerebellar injury and topography in the genesis of cognitive, social-behavioral dysfunction.

Neurodegeneration and the Cerebellum

“…Children with underdeveloped right lateral cerebellar hemispheres exhibit impaired cognition (typically in areas controlled by the right cerebral cortex, including expressive, verbal, and literary language deficits), auditory sequential memory, fine and gross motor skills, expressive language, and language processing [29,30,31,32,33]. Additionally, the right cerebellum regulates key frontal associative areas of the left cerebral hemisphere (an area dominant for language functions) via the thalamus [31,34].…”

Brain Injury in Preterm Infants: Pathogenesis and Nursing Implications

“…6,15,23,24 The cerebellum acts as a node in distribution of neural networks with interconnections with thalamus and parietal and prefrontal cortex, integrates sensory information and is important in socialization skills. 1,18,25 The cerebellum is one of the later areas of the brain to mature, During late second trimester and third trimester, when preterm infants are in the neonatal intensive care unit, the cerebellum undergoes rapid growth and complex, critical developmental processes.…”

“…25 Three mechanisms have been proposed for the alterations seen in the cerebellum in VLBW infants: (1) direct injury (usually due to cerebral hemorrhage) leading to atrophy and growth failure; (2) indirect cerebellar injury or underdevelopment associated with cerebral injury; and (3) underdevelopment without any evidence of direct or indirect injury (e.g., prematurity itself may be associated with impaired cerebellar development due to unknown causes). 23,27 Indirect injury involves cerebellar injury in areas contralateral to the cerebral injury that leads to decreased blood flow and metabolism in the contralateral cerebellum resulting in decreased growth and cerebellar hypoplasia. 23 The indirect injury occurs via a processes called -crossed cerebellar diachisis‖.…”

“…23,27 Indirect injury involves cerebellar injury in areas contralateral to the cerebral injury that leads to decreased blood flow and metabolism in the contralateral cerebellum resulting in decreased growth and cerebellar hypoplasia. 23 The indirect injury occurs via a processes called -crossed cerebellar diachisis‖. 15,16 This means that disruption of activity in one area of the brain area can affect the organizational and function of other remote areas to which it is connected via fiber tracts.…”

“…30 CH accompanied by supratentorial injury has a high mortality. 23,25,28 Recent recognition of a silent presentation confined to the cerebellum ranging from mild unilateral focal injury to extensive hemorrhage that involves both cerebellar hemispheres as well as the vermis (area between the hemispheres) that may or may not be associated with…”