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

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

Research output: Contribution to journalArticle

34 Citations (Scopus)

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

Fingerprint

Lactic Acidosis
Status Epilepticus
Psychotic Disorders
Seizures
Paranoid Behavior
Confusion
Professional Education
Premature Mortality
Delusions
Hallucinations
Medical Education
Mitochondrial DNA
Neurodegenerative Diseases
Anticonvulsants
Psychiatry
Electroencephalography
Kidney
Mutation
Mitochondrial encephalopathy

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

Cite this

MELAS with recurrent complex partial seizures, nonconvulsive status epilepticus, psychosis, and behavioral disturbances : Case analysis with literature review. / Kaufman, Kenneth R.; Zuber, Nicole; Rueda-Lara, Maria Adelaida; Tobia, Anthony.

In: Epilepsy and Behavior, Vol. 18, No. 4, 01.08.2010, p. 494-497.

Research output: Contribution to journalArticle

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