Periventricular nodular heterotopia

Case contributed by Dr Charlie Chia-Tsong Hsu



Patient Data

Age: 70
Gender: Female

Non contrast CT demonstrate nodularity of the ependymal surface of the ventricles giving it a lumpy bumpy appearance. Background of intracranial arterial calcifications and periventricular hypodensities which may represent microangiopathic changes. Posterior fossa cystic lesion of CSF attenuation and mild regional mass effect is probably either an arachnoid cyst or variant mega cisterna magna. 

Extensive subependymal nodules line the walls of the lateral ventricles. These nodules follows gray matter signal intensity on all sequences and appearance is typical of periventricular nodular heterotopia. 

Background of semi-confluent periventricular and external capsule white matter hyperintensities consistent with microangiopathy. Incidental finding of a posterior fossa arachnoid cyst with marginal regional mass effect. Partial empty sella.

Case Discussion

Periventricular nodular heterotopia is a common cortical malformation in adults. Subependymal nodules of gray matter line the lateral walls of the ventricles and follows gray matter density on CT and signal intensity on MRI. These nodules do not enhance on post contrast examination. When periventricular nodular heterotopia is recognized in female patients of reproductive age, genetic counseling is adviced as FLNA x-linked dominant mutation is fatal to affected male resulting in  perinatal/in utero demise.

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Case information

rID: 26583
Published: 29th Dec 2013
Last edited: 10th Apr 2020
Inclusion in quiz mode: Included

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