Subependymal Nodules

Ozlem Yildiz, Franziska Kohne
2016 Journal of Pediatric Neurology and Medicine  
We present the incidental MRI findings of a then 17-year-old German girl who presented with migraine-type headache. Contrast enhanced 2D spin-echo T1-weighted multiplanar reconstructions show multiple small nodules, isointense to gray matter, along the ependymal surfaces of the pars centralis of both lateral ventricles as indicated by arrows (images from September 12th 2015 are with courtesy of the Central Department of Radiology, Peine Hospital, Peine). These nodules are heterotopia of the
more » ... erotopia of the gray matter and consist of normal neurons and glial cells but without laminar organization ( Figure 1 ). Characteristically, they protrude into the ventricular lumen, whereby the ventricular walls get an irregular shape. Their atypical localization is due to accumulations of neurons that have had their migration to the cerebral cortex prematurely arrested [1,2]. Our patient did not have any other CNS malformations and the heterotopia is thus referred to as isolated subependymal gray matter heterotopia. Accordingly, there is an association with an X-linked (filamin 1 gene localized to Xq28) and non-X-linked (ADP-ribosylation factor guanine exchange factor 2 gene) inheritance pattern [3] . Although it is a rare condition, subependymal gray matter heterotopia is the most common identified type of heterotopia and associated with seizures in the second decade of life, affecting females more frequently than males [2] . There is no causal therapy available and management of seizures is currently the focus of treatment. Figure 1: Subependymal nodules. References 1. Raymond AA, Fish DR, Stevens JM, Sisodiya SM, Alsanjari N, et al. (1994) Subependymal heterotopia: a distinct neuronal migration disorder associated with epilepsy. J Neurol Neurosurg Psychiatry 57: 1195-1202. 2. Manganaro L, Saldari M, Bernardo S, Aliberti C, Silvestri E (2013) Bilateral subependymal heterotopia, ventriculomegaly and cerebellar asymmetry: fetal MRI findings of a rare association of brain anomalies. J Radiol Case Rep 7: 38-45. 3. Ferland RJ, Batiz LF, Neal J, Lian G, Bundock E, et al. (2009) Disruption of neural progenitors along the ventricular and subventricular zones in periventricular heterotopia. Hum Mol Genet 18: 497-516.
doi:10.4172/2472-100x.1000i101 fatcat:bjg6yjtmqvd53erea2ghg6utta