Regression of stroke-like lesions in MELAS-syndrome after seizure control

Epileptic Disord. 2010 Dec;12(4):330-4. doi: 10.1684/epd.2010.0338. Epub 2010 Nov 9.

Abstract

There are some indications that seizure activity promotes the development of stroke-like episodes, or vice versa, in patients with mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome or other syndromic mitochondrial disorders. A 41-year-old Caucasian female with MELAS syndrome, presenting with short stature, microcytic anaemia, increased blood-sedimentation rate, myopathy, hyper-gammaglobulinaemia, an iron-metabolism defect, migraine-like headaches, and stroke-like episodes, developed complex partial and generalised seizures at age 32 years. Valproic acid was ineffective but after switching to lamotrigine and lorazepam, she became seizure-free for five years and stroke-like episodes did not recur. Cerebral MRI initially showed enhanced gyral thickening and a non-enhanced T2-hyperintensity over the left parieto-temporo-occipital white matter and cortex and enhanced caudate heads. After two years without seizures, the non-enhanced hyperintense parieto-temporo-occipital lesion had disappeared, being attributed to consequent seizure control. The caudate heads, however, remained hyperintense throughout the observational period. This case indicates that adequate seizure control in a patient with MELAS syndrome may prevent the recurrence of stroke-like episodes and may result in the disappearance of stroke-like lesions on MRI.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anticonvulsants / therapeutic use
  • Cerebral Cortex / pathology*
  • Diagnosis, Differential
  • Electroencephalography
  • Female
  • Humans
  • Lamotrigine
  • Lorazepam / therapeutic use
  • MELAS Syndrome / complications
  • MELAS Syndrome / diagnosis*
  • MELAS Syndrome / drug therapy*
  • MELAS Syndrome / pathology
  • MELAS Syndrome / physiopathology
  • Magnetic Resonance Imaging*
  • Remission Induction
  • Seizures / diagnosis*
  • Seizures / drug therapy*
  • Seizures / etiology
  • Seizures / pathology
  • Seizures / physiopathology
  • Treatment Outcome
  • Triazines / therapeutic use

Substances

  • Anticonvulsants
  • Triazines
  • Lorazepam
  • Lamotrigine