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Transient left temporal lobe lesion in Menkes disease may influence the generation of tonic spasms

Hiromichi Ito1, Kenji Mori, Miho Sakata

  • 1Department of Pediatrics, School of Medicine, University of Tokushima, Japan. michiito@lime.ocn.ne.jp

Brain & Development
|March 9, 2011
PubMed

Insights

This case study details a 7-month-old boy with Menkes disease and West syndrome, highlighting a transient temporal lobe lesion associated with vasogenic edema and epileptic activity. The lesion

Area of Science:

  • Pediatric Neurology
  • Neuroimaging
  • Metabolic Disorders

Background:

  • Menkes disease is a rare genetic disorder affecting copper metabolism, often presenting with neurological complications.
  • West syndrome, a severe infantile epilepsy, can be associated with various underlying conditions.

Observation:

  • A 7-month-old boy with Menkes disease presented with West syndrome and a transient left temporal lobe lesion.
  • MRI revealed specific signal intensities and diffusion characteristics, with (1)H-MRS showing decreased N-acetylaspartate and a lactate peak.
  • The lesion resolved by 8 months, with normalized MRI findings and spectroscopy.

Findings:

  • The transient temporal lobe lesion in this Menkes disease case was likely vasogenic edema.
  • Electroencephalography (EEG) indicated left hemisphere hypsarrhythmia and focal epileptic activity correlating with the lesion.
  • Resolution of the lesion led to the disappearance of tonic spasms and improved EEG findings.

Implications:

  • This case suggests a potential link between transient temporal lobe lesions (vasogenic edema) and epileptic spasms in Menkes disease.
  • Neuroimaging and spectroscopy can help characterize these transient lesions.
  • Understanding this association may aid in managing neurological manifestations of Menkes disease.

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