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"Erratic" complex partial status epilepticus as a presenting feature of MELAS

Davide Corda1, Giulio Rosati, Giovanni A Deiana

  • 1Neurological Clinic, University of Sassari, Sassari, Italy.

Epilepsy & Behavior : E&B
|February 14, 2006
PubMed

Insights

Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) can present with complex partial status epilepticus (CPSE). This case highlights multifocal CPSE as a rare presenting feature of MELAS, documented by EEG and CT findings.

Area of Science:

  • Neurology
  • Mitochondrial Diseases
  • Epileptology

Background:

  • Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) is a rare genetic disorder.
  • Complex partial status epilepticus (CPSE) is an uncommon initial presentation of MELAS.

Observation:

  • A 38-year-old woman with MELAS presented with multifocal CPSE and periodic lateralized epileptiform discharges (PLEDs) during her first strokelike episode.
  • EEG revealed alternating seizure foci and PLEDs in the right hemisphere, correlating with a right temporo-parieto-occipital strokelike lesion on CT.
  • This contrasts with previously reported MELAS cases with CPSE, which showed unifocal epileptic discharges.

Findings:

  • This report details the first documented case of multifocal CPSE as the presenting feature of MELAS.
  • The findings demonstrate multifocality of neuronal hyperexcitability associated with cerebral strokelike lesions in MELAS.
  • Successful management with carbamazepine led to normalization of electroclinical status and CT findings.

Implications:

  • Highlights the diverse neurological manifestations of MELAS.
  • Suggests that multifocal neuronal hyperexcitability can occur in MELAS-related strokelike lesions.
  • Broadens the understanding of potential initial presentations of MELAS, aiding in earlier diagnosis and management.

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