Complex partial status epilepticus in late-onset MELAS

Area of Science:

• Neurology
• Mitochondrial Medicine
• Epileptology

Background:

• Mitochondrial disorders, such as MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes), are known to cause neurological dysfunction.
• Complex partial status epilepticus (CPSE) is a prolonged seizure state characterized by altered consciousness and focal neurological signs.
• Periodic Lateralized Epileptiform Discharges (PLEDs) on electroencephalogram (EEG) can indicate focal brain injury or dysfunction.

Observation:

• A patient presented with recurrent episodes of CPSE.
• The patient exhibited a distinctive EEG pattern of PLEDs.
• Diagnostic workup revealed the patient had a mitochondrial disorder of the MELAS type.

Findings:

• This case represents a previously unreported association between MELAS and CPSE with PLEDs.
• The clinical presentation and EEG findings were consistent with CPSE in the context of MELAS.

Implications:

• The findings suggest that MELAS should be recognized as a potential underlying cause of CPSE and acute neurological deterioration.
• Clinicians should consider screening for mitochondrial disorders, including MELAS, in patients presenting with CPSE and PLEDs, especially those with unexplained neurological decline.
• This association expands the spectrum of neurological manifestations associated with MELAS syndrome.