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Mid-gestation right basal ganglia lesion: clinical observations in two children
1Division of Pediatric Neurology, Duke University Medical Center, Durham, NC 27710, USA. delon006@mc.duke.edu
Insights
Early basal ganglia damage in children can cause severe behavioral and cognitive issues, including disinhibition and emotional dysregulation. This study highlights the lasting impact of such early-life unilateral lesions.
Area of Science:
- Neuroscience
- Developmental Pediatrics
- Pediatric Neurology
Background:
- Unilateral basal ganglia lesions in early life are uncommon.
- The resulting neurobehavioral syndromes have not been previously described.
Observation:
- Two children presented with right basal ganglia destruction, potentially due to amniocentesis.
- Clinical assessments over 10 years included MRI, PET scans, and psychometric testing.
Findings:
- Both children exhibited similar right basal ganglia destruction, leading to left hemiparesis, oculomotor dysfunction, severe disinhibition, emotional lability, and impaired nonverbal/visual-spatial skills.
- One child also had a porencephalic cyst and anomalous cortex.
Implications:
- Complete, early destruction of the right basal ganglia may hinder right hemisphere development and plasticity.
- This can result in significant behavioral issues like disinhibition, impulsivity, and emotional dyscontrol.
Objective:
To describe the neurobehavioral syndrome in two children with destruction of the right basal ganglia ostensibly from amniocentesis needle penetration at 17 weeks of gestation.
Background:
Early-life unilateral lesions of the basal ganglia are rare and the resulting syndrome not described.
Methods:
Both children had repeated clinical assessments, MRI and (18)F-fluorodeoxyglucose PET scans, and psychometric and achievement testing over 10 years.
Results:
Right basal ganglia destruction was similar and virtually coextensive in both children; optic nerve and oculomotor dysfunction were disparate. One had a right temporal pole porencephalic cyst with anomalous overlying cortex. The clinical syndrome included left hemiparesis with distal spasticity and without hypotrophy; extraocular movement disorders; severe episodic disinhibition, impulsiveness, hitting reflexively, and extreme emotional lability. Outbursts of screaming and cursing resembled "sham rage." Both had mild intellectual retardation with competent language but poor nonverbal and visual-spatial abilities, visual memory, and daily living and socialization skills.
Conclusions:
The shared behavioral and cognitive syndrome is most reasonably attributed to the right basal ganglia lesions, which were complete and coextensive in both, whereas other lesions were partial, milder, and disparate. Early destruction of the right basal ganglia may preclude normal development of right hemisphere functions without evidence of plasticity and appears associated with intense disinhibition and impulsiveness of aggressive attack activities and with general lability and dyscontrol of emotion.