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Each cerebral hemisphere can be divided into three main regions. The outermost region, the cerebral cortex, is a thin layer (2 to 4 millimeters thick) made up of gray matter, consisting of neuron cell bodies, dendrites, glial cells, and blood vessels. The middle region, or white matter, is primarily composed of myelinated nerve fibers organized into three types of large tracts: association fibers, commissures, and projection fibers. Association fibers connect different areas within the same...
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The somatosensory cortex in the parietal lobes is crucial for interpreting sensory data such as touch, temperature, and proprioception. The somatosensory cortex, situated in the parietal lobes, plays a vital role in interpreting sensory information like touch, temperature, and proprioception—awareness of body position. This specialized brain region features an organized structure wherein neurons at the top primarily process sensations originating from the lower body. In contrast, those at...
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[Subcortical laminar heterotopia 'double cortex syndrome'].

A M Teplyshova1, V V Gaskin1, G V Kustov1

  • 1Moscow Research and Clinical Center for Neuropsychiatry, Moscow, Russia.

Zhurnal Nevrologii I Psikhiatrii Imeni S.S. Korsakova
|December 8, 2017
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Double cortex syndrome, a rare neuronal migration disorder, causes epilepsy and intellectual disabilities. Magnetic resonance imaging (MRI) is key for diagnosing this condition.

Keywords:
Double cortex syndromeEEGMRIepilepsysubcortical band heterotopia

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Area of Science:

  • Neurology
  • Developmental Neuroscience
  • Clinical Case Study

Background:

  • Subcortical band heterotopia, known as double cortex syndrome, is a rare congenital brain malformation.
  • It results from a disruption in neuronal migration during fetal development.
  • This condition is characterized by the presence of a heterotopic band of neurons beneath the cerebral cortex.

Observation:

  • A clinical case of a 29-year-old patient presenting with double cortex syndrome is described.
  • The patient exhibited symptoms including epilepsy, intellectual disability, and mental disorders.
  • The presentation highlights the typical neurological and cognitive impairments associated with this disorder.

Findings:

  • Magnetic resonance imaging (MRI) is the primary diagnostic tool for identifying subcortical band heterotopia.
  • MRI reveals the characteristic double cortex or subcortical band of gray matter.
  • The case underscores the correlation between the anatomical abnormality and clinical manifestations.

Implications:

  • Accurate diagnosis through MRI is crucial for managing patients with double cortex syndrome.
  • Understanding the pathophysiology aids in predicting clinical outcomes and potential comorbidities.
  • Further research into neuronal migration disorders can inform therapeutic strategies and genetic counseling.