Presentation
Seizure; history of seizures.
Patient Data
Hypoplasia in the upper part of the right postcentral gyrus and superior parietal lobule.
Hypoplasia of the left caudate nucleus and lentiform nucleus, with ex vacuo widened ipsilateral frontal horn.
Focal right superior parietal hypoplasia involving more than one gyrus.
Hypoplasia of the left caudate nucleus and lentiform nucleus, with ex vacuo widened ipsilateral frontal horn. The hypoplastic left putamen exhibits a high T2/FLAIR signal.
High T2/FLAIR signal foci of unknown significance: in each centrum semiovale, in the right frontal white matter, in the left postcentral gyrus white matter, and in each external capsule.
Case Discussion
Convulsed at home for <10 minutes. No tongue biting, head trauma, or temporary incontinence. Reports seizure attack one year previously and two attacks 15 years previously. Had not undergone any workup and did not receive any medication.
CT and MRI showed focal, substantial right parietal hypoplasia and left basal ganglia hypoplasia.
She was started on lamotrigine.
In this case, both cortical and basal ganglia hypoplasia were most probably a consequence of an in utero vascular insult. As the patient did not exhibit any motor deficiency, the insult must have happened at an early developmental stage, such that brain plasticity enabled the development of alternative, basal ganglia-bypassing pyramidal and extrapyramidal motor tracts.
Unilateral hypoplasia of the basal ganglia is an exceedingly rare phenomenon; we found only 2 case reports of either basal ganglia hypoplasia or aplasia without motor symptoms 1,2, both in patients who underwent imaging for seizures, plus one more case report describing motor deficits.
Co-contributor: Dr. Amin Younis
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