Leigh disease (subacute necrotizing encephalomyelopathy): CT and MR in five cases

C A Geyer1, K J Sartor, A J Prensky

  • 1Department of Radiology, Walter Reed Army Medical Center, Washington, D.C. 20307-5001.

Insights

Magnetic resonance (MR) imaging and CT scans aid in diagnosing Leigh disease, a rare neurological disorder. MR imaging offers superior detail in identifying affected brain regions compared to CT scans.

Area of Science:

  • Neurology
  • Radiology
  • Medical Imaging

Background:

  • Leigh disease, also known as subacute necrotizing encephalomyelopathy, is a severe neurological disorder.
  • Accurate and early diagnosis is crucial for patient management.

Purpose of the Study:

  • To evaluate the utility of Magnetic Resonance (MR) imaging and Computed Tomography (CT) in diagnosing Leigh disease.
  • To compare the effectiveness of MR imaging and CT in identifying characteristic brain lesions.

Main Methods:

  • Retrospective analysis of five patients with suspected or confirmed Leigh disease.
  • Review of MR imaging and CT scans, correlating findings with clinical and postmortem data.

Main Results:

  • Symmetrical low attenuation in the basal ganglia (putamina) on CT correlated with high signal intensity on MR imaging.
  • MR imaging identified additional affected areas, including the tectum, tegmentum, and medullary olive, which were not as clearly visualized by CT.
  • Pathological examination confirmed necrotic lesions in the identified areas.

Conclusions:

  • Both MR imaging and CT can support the diagnosis of Leigh disease.
  • MR imaging demonstrates superior sensitivity in detecting the full extent of brain involvement in Leigh disease.
  • In a relevant clinical context, MR imaging may be diagnostic for Leigh disease.