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Periodic lateralized epileptiform discharges in mitochondrial encephalomyopathy
I Funakawa1, T Yasuda, A Terao
1Department of Neurology, Kawasaki Medical School, Okayama, Japan.
Abstract:
Periodic lateralized epileptiform discharges (PLEDs) were transiently recorded during the acute phase of cerebral stroke-like episodes in three patients with mitochondrial encephalomyopathy (MEM). One of the patients developed stroke-like episodes three times, always accompanied by PLEDs. These discharges were recorded from the hemisphere responsible for the stroke-like episodes or from the more severely affected hemisphere when bilateral hemispheres were affected. Although PLEDs have been rarely reported in MEM, this disease should be considered as a differential diagnosis of cerebral infarction with PLEDs.
Insights
Periodic lateralized epileptiform discharges (PLEDs) were observed in patients with mitochondrial encephalomyopathy (MEM) during stroke-like episodes. This finding suggests MEM should be considered in the differential diagnosis of cerebral infarction presenting with PLEDs.
Area of Science:
- Neurology
- Neurophysiology
- Genetics
Background:
- Mitochondrial encephalomyopathy (MEM) is a group of rare genetic disorders affecting energy production.
- Stroke-like episodes are a recognized, though infrequent, manifestation of MEM.
- Periodic lateralized epileptiform discharges (PLEDs) are typically associated with acute brain injury, such as stroke or encephalitis.
Observation:
- Three patients with MEM exhibited PLEDs during acute cerebral stroke-like episodes.
- PLEDs correlated with the affected cerebral hemisphere, either the primary site of the stroke-like episode or the more severely impacted hemisphere in bilateral cases.
- Recurrent stroke-like episodes in one patient were consistently accompanied by PLEDs.
Findings:
- This study reports a rare association between PLEDs and stroke-like episodes in MEM.
- The electrophysiological findings (PLEDs) localized to the clinically affected brain regions.
Implications:
- The presence of PLEDs during stroke-like episodes in MEM patients suggests a potential link between these phenomena.
- Clinicians should consider MEM in the differential diagnosis of patients presenting with cerebral infarction and PLEDs.
- Further research is warranted to elucidate the underlying mechanisms connecting mitochondrial dysfunction, stroke-like episodes, and epileptiform activity.