Related Experiment Videos
Shaken infant syndrome: developmental neuropathology, progressive cortical dysplasia, and epilepsy
Miguel Marín-Padilla1, Joseph E Parisi, Dawna L Armstrong
1Molecular Neuroscience Program, Mayo Clinic, Guggenheim 1521A, 200 First Ave. SW, Rochester, MN 55905, USA. marinpadilla.miguel@mayo.edu
Insights
Shaken infant syndrome causes severe brain injuries that resolve, leading to encephaloclastic encephalopathies. Surviving brain regions undergo reorganization, potentially causing long-term neurological and psychological issues.
Area of Science:
- Neuropathology
- Developmental Neuroscience
- Pediatric Traumatology
Background:
- Shaken infant syndrome (SIS) is a severe form of abusive head trauma.
- Understanding the long-term neuropathological consequences of SIS is crucial for affected children.
Observation:
- Two infants surviving SIS showed initial severe injuries including contusions, hemorrhages, hypoxic/ischemic damage, and edema.
- Radiographic and postmortem studies detailed injury resolution and development of encephaloclastic encephalopathies like multicystic encephalomalacia and porencephaly within 6 months.
Findings:
- Injuries resolved, but surviving cortical regions underwent significant post-injury reorganization.
- This reorganization included progressive cortical dysplasia, cytoarchitectural disorganization, and neuronal transformation, with preserved layer 1 structures.
Implications:
- The progressive dysplastic process in surviving brain regions may alter structural and functional organization.
- This reorganization could significantly influence neurological and psychological maturation and contribute to long-term sequelae.
Abstract:
This study describes the developmental neuropathology of two infants who survived 7 and 9 years, respectively, an episode of violent shaking (shaken infant syndrome) early in their lives. The shaking injuries include cortical and subcortical contusions, hemorrhages, hypoxic/ischemic and axonal damage, and severe edema. The types, distribution, and resolution of these shaking injuries are detailed by sequential radiographic studies and by pathologic examination at postmortem. Despite their severity and extent, these injuries resolved in a relatively short period of time. By 6 months, the original injuries are repaired and the resultant encephaloclastic encephalopathies (e.g., multicystic encephalomalacia, porencephaly, generalized white matter attenuation, diffuse cortical atrophy, microgyria, ulegyria, and hydrocephalus ex vacuo) are well established. No appreciable pathologic differences are detected when radiographic findings at 6 months of age are compared to postmortem observations. On the other hand, undamaged and/or partially damaged cortical regions survive the original insult and undergo post-injury reorganization that transforms the residual cortex structural and presumably functional organization. Prominent features of this post-injury reorganization include progressive cortical dysplasia with cytoarchitectural disorganization, laminar obliteration, morphologic and functional (synaptic reorganization) transformation of some neurons, preservation of layer 1 intrinsic fibers and Cajal-Retzius cells, and the presence of large (hypertrophic) intrinsic neurons with intense neurofilament immunoreactivity. We propose that this progressive dysplastic process modifies the residual cortex structural and functional organization, influences the child's neurological and psychological maturation, and may play a significant role in the pathogenesis of ensuing neurological and/or psychological sequelae.