Band Heterotopia

Clinical Data
• X.A.
• 9/F
• Seizures
MRI Findings:
• Band of grey matter is seen located deeply and almost parallel to the
cortex with similar signal intensity; the double cortical layers are
separated by a thin white matter layer. The overlying cortex is normal
with no pacchygyria, polymicrogyria or lissencephaly. The ventricles
are normal in size for age.
Band Heterotopia
• Double cortex syndrome
• Sometimes called laminar heterotopia
• Form of diffuse grey matter heterotopia affecting almost only women.
• Refractory epilepsy is present in nearly all affected patients, with
partial complex and atypical absence epilepsy being the most
common syndromes.
Band Heterotopia
• Best thought of as being on the lissencephaly type I – subcortical
band heterotopia spectrum, as the genetic defects are in many cases
the same and result from arrested migration of neurons, such that
they form a grey matter layer within the centrum semiovale or
subcortical white matter.
• Attributed to an abnormality of the DCX gene (also called as XLIS
gene) located on the long arm of chromosome X.
• Encodes for the protein doublecortin responsible for stabilization of
microtubules, crucial in normal neuronal migration.
Band Heterotopia
• Although all modalities able to image the brain in cross section are able
to identify the abnormality, MRI is the imaging modality of choice.
• Findings:
• Characteristic 3-layer cake (continuous double cortex)
• Band of grey matter located deep to, and roughly paralleling, the cortex, with
either normal or pachygyric overlying cortex.
• The band of abnormal grey matter may be complete or incomplete and may
even be duplicated within the temporal lobes.
• The signal intensity is the same as normal cortex on all sequences.
• Reduction of NAA within the heterotopic gray on MRS as neurons therein are
dysfunctional.
Differential Diagnosis
• Lissencephaly
• Smooth brain
• Ranges from severe (agyria) to milder forms, including abnormally broad folds
(pachygyria) or heterotopic layer of gray matter embedded in the white
matter below the cortex (subcortical band heterotropia)
Grading System