Adolescent case of Alexander disease: MR imaging and MR spectroscopy

Pediatr Neurol. 1998 Jan;18(1):67-70. doi: 10.1016/s0887-8994(97)00128-8.

Abstract

A 17-year-old male was diagnosed as having Alexander disease from the clinical manifestations (psychomotor deterioration and megalencephaly), neuroradiologic findings (frontal dominant leukodystrophy), and elevation of alpha B-crystallin and heat shock protein 27 in the cerebrospinal fluid. He exhibited increased attenuation on computed tomography and T1 and T2 shortening on magnetic resonance imaging in the bilateral basal ganglia and thalamus. Some paramagnetic substances might be deposited in the basal ganglia and thalamus in the late stage of Alexander disease, at least 8 years after onset.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Astrocytes / pathology*
  • Brain Diseases / enzymology
  • Brain Diseases / pathology*
  • Crystallins / metabolism
  • Heat-Shock Proteins / metabolism
  • Humans
  • Inclusion Bodies / pathology*
  • Magnetic Resonance Imaging
  • Magnetic Resonance Spectroscopy
  • Male
  • Nerve Tissue Proteins / metabolism
  • Psychomotor Performance*

Substances

  • Crystallins
  • Heat-Shock Proteins
  • Nerve Tissue Proteins