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

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.

• Authors: Josef Finsterer, Peter Barton
• Journal: Epileptic disorders : international epilepsy journal with videotape (Epileptic Disord) Vol. 12 Issue 4 Pg. 330-4 (Dec 2010) ISSN: 1294-9361 [Print] United States
• PMID: 21059492 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anticonvulsants
• Triazines
• Lorazepam
• Lamotrigine

Topics

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