MELAS with recurrent complex partial seizures, nonconvulsive status epilepticus, psychosis, and behavioral disturbances: Case analysis with literature review

Kenneth R. Kaufman, Nicole Zuber, Maria A. Rueda-Lara, Anthony Tobia

Research output: Contribution to journalArticle

35 Scopus citations

Abstract

Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) is a progressive neurodegenerative disorder associated with polygenetic, maternally inherited, mitochondrial DNA mutations. MELAS has multisystem presentation including neurological, muscular, endocrine, auditory, visual, cardiac, psychiatric, renal, gastrointestinal and dermatological symptoms. Clinical course and prognosis are variable, often leading to cognitive decline, disability, and premature death. Both convulsive status epilepticus (CSE) and nonconvulsive status epilepticus (NCSE) are reported with MELAS. This report illustrates a case of MELAS with recurrent complex partial seizures, NCSE, confusion, aggressive behaviors, hallucinations, and paranoid delusions. Rapid video/EEG confirmation of diagnosis and aggressive antiepileptic drug intervention are required. Further education of medical professionals regarding this disorder, its appropriate management, and the significance of NCSE is indicated to avoid delay of treatment.

Original languageEnglish (US)
Pages (from-to)494-497
Number of pages4
JournalEpilepsy and Behavior
Volume18
Issue number4
DOIs
StatePublished - Aug 1 2010
Externally publishedYes

Keywords

  • Aggression
  • Coenzyme Q10
  • Complex partial seizures
  • Education
  • Levocarnitine
  • Mitochondrial encephalopathy
  • Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS)
  • Nonconvulsive status epilepticus
  • Oxidative stress
  • Paranoid delusions
  • Psychosis, hallucinations
  • Treatment
  • Valproate
  • Video/EEG monitoring
  • Vitamin E

ASJC Scopus subject areas

  • Clinical Neurology
  • Behavioral Neuroscience
  • Neurology

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