Images in Neurology |

Late-Onset Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Strokelike Episodes With Bitemporal Lesions

Yaz Y. Kisanuki, MD; Kirsten L. Gruis, MD; Teresa L. Smith, MD; Devin L. Brown, MD, MS
Arch Neurol. 2006;63(8):1200-1201. doi:10.1001/archneur.63.8.1200.
Text Size: A A A
Published online


A thin 50-year-old man with a history of diabetes and deafness and a family history of hearing dysfunction presented with new-onset headaches, psychiatric symptoms, and a seizure. He was found to have a right temporal lesion (Figure, A). Three months later, he developed aphasia, complained of headache, and had a convulsive seizure. This was followed by gradual improvement in his language and cognitive function throughout several months. A subsequent magnetic resonance image (Figure, B) showed a new lesion in the left temporal lobe. The results of magnetic resonance angiography of the head and carotid ultrasound were unrevealing. Transthoracic echocardiography revealed no thrombus or patent foramen ovale. Proteins C and S and antithrombin III levels were within the reference range. Factor V Leiden was not present and anticardiolipin antibody concentrations were not elevated. No cerebrospinal fluid pleocytosis was identified, and polymerase chain reaction was negative for herpes. The serum lactate level was within the reference range. Mitochondrial DNA analysis revealed mutation A3243G, the most common mutation in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS).2

Figures in this Article

Sign In to Access Full Content

Don't have Access?

Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more

Subscribe for full-text access to content from 1998 forward and a host of useful features

Activate your current subscription (AMA members and current subscribers)

Purchase Online Access to this article for 24 hours

First Page Preview

View Large
First page PDF preview


Place holder to copy figure label and caption

Imaging of the patient's brain. A, Brain magnetic resonance axial diffusion-weighted image shows the right temporal lesion. B, Axial fluid-attenuated inversion recovery image 3 months later demonstrates the old right temporal and the new left temporal lesions. The parieto-occipital regions, most often affected in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes, were spared.1

Graphic Jump Location




Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment


Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Topics
PubMed Articles