Regression of stroke-like lesions in MELAS-syndrome after seizure control.
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.
Krankenanstalt Rudolfstiftung, Institute of Radiology Urania, Vienna, Austria. fifigs1@yahoo.de
Josef
J
Finsterer
Peter
P
Barton
D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use; D002540:Cerebral Cortex / Q000473:pathology*; D003937:Diagnosis, Differential; D004569:Electroencephalography; D005260:Female; D006801:Humans; D000077213:Lamotrigine; D008140:Lorazepam / Q000627:therapeutic use; D017241:MELAS Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D012074:Remission Induction; D012640:Seizures / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D016896:Treatment Outcome; D014227:Triazines / Q000627:therapeutic use
D000927:Anticonvulsants; D014227:Triazines; D008140:Lorazepam; D000077213:Lamotrigine