Recurrent posterior reversible encephalopathy syndrome in mitochondrial disorder

Blood Press. 2009;18(3):126-9. doi: 10.1080/08037050902975106.


Objectives: Recurrent posterior reversible encephalopathy syndrome (PRES) has not been reported in association with multi-system mitochondrial disorder (MID).

Case report: In a 60-year-old HIV-negative, Caucasian female with short stature, double vision, struma, memory impairment, chronic renal failure requiring hemodialysis, seizures, intermittent atrial fibrillation, arterial hypertension, hyperlipidemia, anemia, hypacusis, tinnitus, and a daughter with multiple sclerosis, short stature and hypothyroidism; another daughter with schizophrenia, and a son who died from encephalopathy at age 3 months, an MID was suspected. At age 53 years, she experienced sudden, transient blindness for 2 days bilaterally during an episode of high blood pressure with complete recovery within 2 weeks. At age 60 years, a similar episode occurred. Four weeks later, she experienced a third PRES episode with high blood pressure, seizures, impaired consciousness and reversible blindness for 2 days. MRI at the second episode was indicative of a PRES, and MRI at the third episode additionally revealed occipital bleedings, acute embolic stroke and features indicating PRES.

Conclusions: This case indicates that MID may be associated with recurrent PRES, triggered by recurrent episodes of high blood pressure. Whether high blood pressure was a manifestation of the MID or related to other causes remains speculative. PRES does not seem to be a primary but is rather a secondary manifestation of an MID.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Brain Damage, Chronic / complications*
  • Consciousness Disorders / complications
  • Consciousness Disorders / etiology
  • Female
  • Follow-Up Studies
  • Hepatic Encephalopathy / complications
  • Humans
  • Hypertension / complications
  • Magnetic Resonance Imaging / adverse effects
  • Middle Aged
  • Mitochondrial Diseases / complications
  • Multiple Sclerosis / complications
  • Neurotoxicity Syndromes / complications
  • Recurrence
  • Seizures / complications
  • Seizures / etiology
  • Stroke / complications
  • Syndrome
  • Time Factors