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Thalamic aphasia associated with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes: A case report

      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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      References

        • El-Hattab A.W.
        • Adesina A.M.
        • Jones J.
        • Scaglia F.
        MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options.
        Mol Genet Metab. 2015; 116: 4-12
        • Tetsuka S.
        • Ogawa T.
        • Hashimoto R.
        • Kato H.
        Clinical features, pathogenesis, and management of stroke-like episodes due to MELAS.
        Metab Brain Dis. 2021; 36: 2181-2193
        • Mariajoseph F.P.
        • Sagar P.
        • Muthusamy S.
        • Amukotuwa S.
        • Seneviratne U.
        Seizure-induced reversible MRI abnormalities in status epilepticus: A systematic review.
        Seizure. 2021; 92: 166-173
        • De Witte L.
        • Brouns R.
        • Kavadias D.
        • Engelborghs S.
        • De Deyn P.P.
        • Mariën P.
        Cognitive, affective and behavioural disturbances following vascular thalamic lesions: a review.
        Cortex. 2011; 47: 273-319
        • Klostermann F.
        • Krugel L.
        • Wahl M.
        Learning about language and speech from Deep Brain Stimulation.
        J Neurolinguistics. 2012; 25: 63-73
        • Osawa A.
        • Maeshima S.
        Aphasia and unilateral spatial neglect due to acute thalamic hemorrhage: clinical correlations and outcomes.
        Neurol Sci. 2016; 37: 565-572
        • Fritsch M.
        • Krause T.
        • Klostermann F.
        • Villringer K.
        • Ihrke M.
        • Nolte C.H.
        “Thalamic aphasia” after stroke is associated with left anterior lesion location.
        J Neurol. 2020; 267: 106-112
        • Rangus I.
        • Fritsch M.
        • Endres M.
        • Udke B.
        • Nolte C.H.
        Frequency and phenotype of thalamic aphasia.
        J Neurol. 2022; 269: 368-376
        • Ford A.A.
        • Triplett W.
        • Sudhyadhom A.
        • Gullett J.
        • McGregor K.
        • FitzGerald D.B.
        • et al.
        Broca's area and its striatal and thalamic connections: a diffusion-MRI tractography study.
        Front Neuroanat. 2013; 7: 8
        • Bohsali A.A.
        • Triplett W.
        • Sudhyadhom A.
        • Gullett J.M.
        • McGregor K.
        • FitzGerald D.B.
        • et al.
        Broca's area – thalamic connectivity.
        Brain Lang. 2015; 141: 80-88
        • Bogousslavsky J.
        • Caplan L.R.
        Vertebrobasilar occlusive disease: review of selected aspects.
        Cerebrovasc Dis. 1993; 3: 193-205
        • Crosson B.
        The role of cortico-thalamo-cortical circuits in language: recurrent circuits revisited.
        Neuropsychol Rev. 2021; 31: 516-533
        • Le H.
        • Aphasia L.MY.
        StatPearls [Internet]: Treasure Island (FL).
        StatPearls Publishing. 2021;