Widespread frontal lobe cortical dysplasia or partial hemimegalencephaly: a continuum of the spectrum

Emilija Cvetkovska1, Igor Kuzmanovski1, Bojan Boskovski1

  • 1University Clinic of Neurology, Medical Faculty, Ss. Cyril and Methodius University, Skopje, Republic of North Macedonia.

Insights

This study describes an unusual case of focal cortical dysplasia type II (FCD type II) involving the entire frontal lobe, presenting as an intermediate condition between FCD type II and hemimegalencephaly (HME). The findings highlight the spectrum of these developmental brain disorders.

Area of Science:

  • Neuroscience
  • Neuropathology
  • Developmental Neurology

Background:

  • Focal cortical dysplasia (FCD) type II and hemimegalencephaly (HME) are recognized as a pathological continuum, primarily differentiated by lesion size.
  • Partial HME, particularly in the posterior cortex, is well-documented.

Observation:

  • A 17-year-old male presented with focal seizures since age nine, exhibiting diminished right-hand dexterity but otherwise unremarkable neurological and cognitive status.
  • The patient experienced a relapsing-remitting epilepsy course with extended remission periods.
  • MRI revealed a dysplastic left frontal lobe lesion, including the paracentral lobule, characterized by cortical thickening, polymicrogyria-like abnormal gyration, intraparenchymal calcifications, and white matter hypodensities.

Findings:

  • The presented case exhibits features intermediate to FCD type II and HME.
  • Frontal lobe lesion localization and clinical presentation (childhood onset, relapsing-remitting epilepsy, absence of hemiparesis or significant cognitive impairment) align more closely with FCD type II.
  • Shared MRI characteristics between HME and FCD type II are observed in this case.

Implications:

  • This case expands the understanding of the FCD type II/HME spectrum, particularly concerning extensive frontal lobe involvement.
  • It underscores the importance of considering the full spectrum of these conditions in epilepsy diagnosis and management.
  • Further research into the specific MRI signatures and clinical correlations across the FCD/HME continuum is warranted.

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