Abstract
Background
Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with
aphasia is a rare disorder, with the associated aphasia reported as either Wernicke’s
or Broca’s. Herein, we report a patient with MELAS complicated by thalamic aphasia.
Case
A 15-year-old right-handed girl presented with headache, nausea, right homonymous
hemianopsia, and aphasia. She could repeat words said by others, but had word-finding
difficulty, paraphasia, and dysgraphia. Brain MRI revealed abnormal signals from the
left occipital lobe to the temporal lobe and left thalamus, but Wernicke’s area and
Broca’s area were not involved. Additionally, she had short stature, lactic acidosis,
bilateral sensorineural hearing loss, and a maternal family history of diabetes and
mild deafness. Based on clinical findings and the presence of a mitochondrial A3243G
mutation, she was diagnosed with MELAS. With treatment, the brain MRI lesions disappeared
and her symptoms improved. Her aphasia was classified as amnesic aphasia because she
could repeat words, despite having word-finding difficulty, paraphasia, and dysgraphia.
Based on MRI findings of a left thalamic lesion, we diagnosed her with thalamic aphasia.
Conclusion
Thalamic aphasia may be caused by MELAS. Assessment of whether repetition is preserved
is important for classifying aphasia.
Keywords
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Article Info
Publication History
Published online: May 10, 2022
Accepted:
April 24,
2022
Received in revised form:
April 20,
2022
Received:
January 8,
2022
Identification
Copyright
© 2022 The Japanese Society of Child Neurology Published by Elsevier B.V. All rights reserved.
