The clinical and imaging features of gray matter heterotopia: a clinical analysis on 15 patients

Neurol Sci. 2019 Mar;40(3):489-494. doi: 10.1007/s10072-018-3667-9. Epub 2018 Dec 10.

Abstract

Objective: To investigate the clinical and imaging features of gray matter heterotopia (GMH) and improve the clinicians' understanding of the disease.

Methods: A retrospective study was performed on 15 patients with GMH diagnosed at The Affiliated Hospital of Xuzhou Medical University from November 2014 to November 2016. Their clinical and imaging features are also summarized.

Results: The proportion of male and female patients was 2:1. The age of onset was 2~45 years and the average age was 19.1 years. There were 13 patients with epilepsy who also had cognitive decline (5 cases) and neurological deficit (3 cases). There were 2 patients with headache or dizziness. The imaging findings of GMH are unilateral or multiple spots in the periventricular or subependymal, subcortical, and centrum semiovale and are often accompanied by other cerebral malformations. We found that 10 patients had the subcortical type of GMH and 5 patients had the subependymal type or periventricular nodular heterotopia type. There were 8 cases of ventricular compression, 5 cases of ventriculomegaly, 5 cases of cerebral fissure malformation, 3 cases of pachygyria, 1 case of callosal agenesis, and 1 case of undeveloped septum pellucidum. All the patients were given symptomatic and supportive therapies and 3 patients were treated with antiepileptic drugs. Seizures were, however, poorly controlled.

Conclusion: GMH should also be suspected in patients with juvenile onset of seizures, cognitive decline, and neurological deficits. Magnetic resonance scans may show lesions in the white matter of the brain with signals similar to the normal gray matter.

Keywords: Epilepsy; Gray matter heterotopia; Magnetic resonance imaging; Periventricular nodular heterotopia; Subcortical heterotopia; Ventriculomegaly.

MeSH terms

  • Adolescent
  • Adult
  • Anticonvulsants / therapeutic use
  • Child, Preschool
  • Epilepsy / drug therapy
  • Epilepsy / etiology
  • Female
  • Gray Matter / diagnostic imaging*
  • Humans
  • Image Processing, Computer-Assisted
  • Magnetic Resonance Imaging*
  • Male
  • Middle Aged
  • Periventricular Nodular Heterotopia / complications
  • Periventricular Nodular Heterotopia / diagnostic imaging*
  • Periventricular Nodular Heterotopia / drug therapy
  • Periventricular Nodular Heterotopia / pathology*
  • Retrospective Studies

Substances

  • Anticonvulsants