A Mitochondrial Disorder in a Middle Age Iranian Patient: Report of a Rare Case

• Main Authors: Mostafa Almasi, Mohammad Reza Motamed, Masoud Mehrpour, Bahram Haghi-Ashtiani, Fahimeh Haji Akhondi, Yalda Nilipour, Seyed-Mohammad Fereshtehnejad
• Format: Article
• Language: English
• Published: Iran University of Medical Sciences 2017-07-01
• Series: Basic and Clinical Neuroscience
• Subjects: Mitochondrial disorder; MELAS Syndrome; Middle age
• Online Access: http://bcn.iums.ac.ir/browse.php?a_code=A-10-862-1&slc_lang=en&sid=1

Introduction: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) can involve multiple systems and cause stroke-like episodes and status epilepticus. Case Presentation: A 48-year-old female with history of early fatigability, migraine-type headaches, and bilateral sensory-neural hearing loss presented 3 episodes of serial seizures. On admission she was affected by Wernicke aphasia and, then, right hemiparesis. Investigations showed elevated arterial lactate and ragged red fibers on muscle biopsy. Conclusion: Though more commonly diagnosed during childhood, some cases of adult-onset MELAS syndrome are reported. This syndrome should be considered in patients with stroke-like events in adults without cerebrovascular risk factors and difficult-to-treat seizures.