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Neurology 2002;59:54-58
© 2002 American Academy of Neurology

Mid-gestation right basal ganglia lesion

Clinical observations in two children

G. Robert DeLong, MD

From the Division of Pediatric Neurology, Duke University Medical Center, Durham, NC.

Address correspondence and reprint requests to Dr. G. Robert DeLong, Division of Pediatric Neurology, Box 3936, Duke University Medical Center, Durham, NC 27710; e-mail: delon006{at}mc.duke.edu

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 18F-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.






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