Case Report: Late-Onset Mitochondrial Disease Uncovered by Metformin Use in a Patient With Acute Verbal Auditory Agnosia
IntroductionVerbal auditory agnosia is rarely caused by mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Lactate acidosis, which is the adverse effect of metformin, has proposed links to mitochondrial dysfunction and may trigger clinical features of mitochond...
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Frontiers Media S.A.
2022-03-01
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2022.863047/full |
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| author | Wei-Hao Lin I-Hsiao Yang Hui-En Cheng Hsiu-Fen Lin Hsiu-Fen Lin |
| author_facet | Wei-Hao Lin I-Hsiao Yang Hui-En Cheng Hsiu-Fen Lin Hsiu-Fen Lin |
| author_sort | Wei-Hao Lin |
| collection | DOAJ |
| description | IntroductionVerbal auditory agnosia is rarely caused by mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Lactate acidosis, which is the adverse effect of metformin, has proposed links to mitochondrial dysfunction and may trigger clinical features of mitochondrial diseases.Case PresentationA 43-year-old right-handed man presented to our emergency department with acute onset fever and headache accompanied by impaired hearing comprehension. He could communicate well through handwritten notes but could not understand what others were saying. He had been diagnosed as having diabetes mellitus 2 months prior to this event. Vildagliptin 100 mg/day and metformin 1,700 mg/day were prescribed for glucose control. Laboratory tests revealed elevated lactate levels in serum and cerebrospinal fluid of the patient. Brain MRI disclosed bilateral temporal lesions. Acute encephalitis with temporal involved was initially diagnosed and acyclovir was given empirically. However, follow-up MRI after acyclovir treatment revealed a progression of prior lesions. Further mitochondrial genome analysis revealed a mitochondrial DNA point mutation at position 3,243 (m.3243A > G) with 25% heteroplasmy, which is compatible with MELAS. His clinical symptoms and serum lactate levels were improved after discontinuing the metformin use.ConclusionsTo our knowledge, this is the first report of a patient having late-onset MELAS syndrome that manifested as acute verbal auditory agnosia, which was identified after the patient began using metformin. Metformin is known to inhibit mitochondrial function and could trigger clinical features of MELAS syndrome. We encourage clinicians to maintain a high level of awareness that diabetes mellitus can be caused by mitochondrial disease and to exercise caution in the prescription of metformin. |
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| format | Article |
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| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-12-18T11:03:30Z |
| publishDate | 2022-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-2ff7bd7fef144ae78d4c1977702dace82022-12-21T21:10:10ZengFrontiers Media S.A.Frontiers in Neurology1664-22952022-03-011310.3389/fneur.2022.863047863047Case Report: Late-Onset Mitochondrial Disease Uncovered by Metformin Use in a Patient With Acute Verbal Auditory AgnosiaWei-Hao Lin0I-Hsiao Yang1Hui-En Cheng2Hsiu-Fen Lin3Hsiu-Fen Lin4Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Medical Imaging, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Neurology, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Neurology, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Neurology, College of Medicine, Kaohsiung Medical University, Kaohsiung, TaiwanIntroductionVerbal auditory agnosia is rarely caused by mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Lactate acidosis, which is the adverse effect of metformin, has proposed links to mitochondrial dysfunction and may trigger clinical features of mitochondrial diseases.Case PresentationA 43-year-old right-handed man presented to our emergency department with acute onset fever and headache accompanied by impaired hearing comprehension. He could communicate well through handwritten notes but could not understand what others were saying. He had been diagnosed as having diabetes mellitus 2 months prior to this event. Vildagliptin 100 mg/day and metformin 1,700 mg/day were prescribed for glucose control. Laboratory tests revealed elevated lactate levels in serum and cerebrospinal fluid of the patient. Brain MRI disclosed bilateral temporal lesions. Acute encephalitis with temporal involved was initially diagnosed and acyclovir was given empirically. However, follow-up MRI after acyclovir treatment revealed a progression of prior lesions. Further mitochondrial genome analysis revealed a mitochondrial DNA point mutation at position 3,243 (m.3243A > G) with 25% heteroplasmy, which is compatible with MELAS. His clinical symptoms and serum lactate levels were improved after discontinuing the metformin use.ConclusionsTo our knowledge, this is the first report of a patient having late-onset MELAS syndrome that manifested as acute verbal auditory agnosia, which was identified after the patient began using metformin. Metformin is known to inhibit mitochondrial function and could trigger clinical features of MELAS syndrome. We encourage clinicians to maintain a high level of awareness that diabetes mellitus can be caused by mitochondrial disease and to exercise caution in the prescription of metformin.https://www.frontiersin.org/articles/10.3389/fneur.2022.863047/fullMELASauditory agnosiapure word deafnessmetformincase report |
| spellingShingle | Wei-Hao Lin I-Hsiao Yang Hui-En Cheng Hsiu-Fen Lin Hsiu-Fen Lin Case Report: Late-Onset Mitochondrial Disease Uncovered by Metformin Use in a Patient With Acute Verbal Auditory Agnosia Frontiers in Neurology MELAS auditory agnosia pure word deafness metformin case report |
| title | Case Report: Late-Onset Mitochondrial Disease Uncovered by Metformin Use in a Patient With Acute Verbal Auditory Agnosia |
| title_full | Case Report: Late-Onset Mitochondrial Disease Uncovered by Metformin Use in a Patient With Acute Verbal Auditory Agnosia |
| title_fullStr | Case Report: Late-Onset Mitochondrial Disease Uncovered by Metformin Use in a Patient With Acute Verbal Auditory Agnosia |
| title_full_unstemmed | Case Report: Late-Onset Mitochondrial Disease Uncovered by Metformin Use in a Patient With Acute Verbal Auditory Agnosia |
| title_short | Case Report: Late-Onset Mitochondrial Disease Uncovered by Metformin Use in a Patient With Acute Verbal Auditory Agnosia |
| title_sort | case report late onset mitochondrial disease uncovered by metformin use in a patient with acute verbal auditory agnosia |
| topic | MELAS auditory agnosia pure word deafness metformin case report |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2022.863047/full |
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