Dementia with impaired temporal glucose metabolism in late-onset metachromatic leukodystrophy

Dement Geriatr Cogn Disord. Mar-Apr 2001;12(2):85-8. doi: 10.1159/000051240.


An unusual case of very-late-onset metachromatic leukodystrophy (MLD) with dementia was studied. The patient was a 41-year-old male who presented with mild dementia and a single generalized tonic clonic seizure. Neuropsychological assessment demonstrated mild amnesia, visuospatial dysfunction and attention deficits with a slow psychomotor speed. MR brain imaging displayed confluent hyperintensities of periventricular and subcortical white matter. Low levels of arylsulfatase A confirmed the diagnosis. Impaired cortical glucose metabolism especially of the medial temporal and frontal cortices was observed using positron emission tomography and fluor-18-labeled fluorodesoxyglucose. The neuropsychological deficits are related to the location of deficits in glucose metabolism.

Publication types

  • Case Reports

MeSH terms

  • Brain / pathology
  • Cognition Disorders / diagnosis
  • Cognition Disorders / etiology*
  • Cognition Disorders / metabolism*
  • Fluorodeoxyglucose F18
  • Frontal Lobe / metabolism
  • Glucose / metabolism*
  • Humans
  • Leukodystrophy, Metachromatic / complications*
  • Leukodystrophy, Metachromatic / diagnosis
  • Magnetic Resonance Imaging
  • Male
  • Neuropsychological Tests
  • Occipital Lobe / metabolism
  • Psychomotor Disorders / diagnosis
  • Psychomotor Disorders / etiology
  • Radiopharmaceuticals
  • Rorschach Test
  • Severity of Illness Index
  • Temporal Lobe / metabolism*
  • Tomography, Emission-Computed


  • Radiopharmaceuticals
  • Fluorodeoxyglucose F18
  • Glucose