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Leukoencephalopathy with bilateral anterior temporal lobe cysts

John B Bodensteiner1, John F Kerrigan, Stanley D Johnsen

  • 1Children's Health Center of St. Joseph's Hospital and The Barrow Neurological Institute, Phoenix, AZ, USA. jbodens@chw.edu

Journal of Child Neurology
|August 12, 2006
PubMed

Insights

This study describes a rare genetic neurological disorder characterized by severe intellectual disability and distinct brain MRI findings, including anterior temporal lobe cysts. The condition presents with nonprogressive motor and mental deficits, aiding diagnosis through neuroimaging.

Area of Science:

  • Neurology
  • Neuroimaging
  • Genetics

Background:

  • A distinctive neurological condition has been increasingly reported.
  • Characterized by specific magnetic resonance imaging (MRI) findings and nonprogressive clinical course.
  • 25 cases previously described, highlighting its rarity and distinctiveness.

Purpose of the Study:

  • To report four new cases of this newly recognized neurological entity.
  • To further characterize the clinical and neuroimaging features.
  • To emphasize the diagnostic utility of MRI in this condition.

Main Methods:

  • Case reporting of four new patients.
  • Detailed clinical assessment including motor and mental retardation.
  • Neuroimaging analysis, specifically magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS).

Main Results:

  • Four new patients identified with characteristic anterior temporal lobe cystic changes on MRI.
  • Clinical presentation includes severe, disabling, nonprogressive mental and motor retardation.
  • Magnetic resonance spectroscopy revealed increased myo-inositol and decreased N-acetylaspartate in cerebral white matter.

Conclusions:

  • This is a distinctive, likely genetic, neurological condition.
  • Characteristic neuroimaging and clinical features aid in diagnosis.
  • Anterior temporal lobe cystic changes on MRI are highly suggestive in the appropriate clinical context.

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