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Related Experiment Videos

Hemimegalencephaly--morphological and immunocytochemical study

T C Yasha1, V Santosh, S Das

  • 1Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, India.

Clinical Neuropathology
|January 1, 1997
PubMed
Summary

Hemimegalencephaly (HME) is a rare brain abnormality causing severe seizures. Postmortem analysis revealed abnormal brain development with cellular and structural anomalies in an infant with HME.

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Area of Science:

  • Neuroscience
  • Developmental Biology
  • Pathology

Background:

  • Hemimegalencephaly (HME) is a rare congenital brain malformation characterized by unilateral cerebral hemisphere enlargement.
  • It is an uncommon cause of intractable seizures in infants.

Observation:

  • A 6-month-old infant presented with uncontrolled seizures and a diagnosed mass lesion via CT scan.
  • Postmortem examination confirmed left-sided HME with significant cortical and subcortical structural abnormalities.

Findings:

  • Histological analysis revealed disrupted cortical lamination, atypical neurons with inclusions, neuronal heterotopia, and astrocytosis with calcification.
  • Aberrant neuronal migration patterns and cytoskeletal abnormalities in neurons were observed.
  • Balloon cells were predominantly astrocytic, with altered synaptophysin expression in the cortex.

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Implications:

  • This case highlights the complex cellular and developmental abnormalities in Hemimegalencephaly.
  • It offers insights into studying anomalous brain development and neuronal migration defects.
  • Understanding HME's pathology may aid in diagnosing and managing related neurological disorders.